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Tumors in children - frequency, causes. Features of tumors in children and their clinical course

The development of tumors in children has several features.

The first feature of tumors in children compared to tumors in adults is their occurrence in children on the basis of dysontogenesis - disturbances in the formation of organs and tissues during the period of intrauterine development of the embryo.

In other words, the vast majority of tumors in children arise from malformed tissues that are delayed in their development at the embryonic level, often located out of place—dystopically, i.e. Most childhood tumors are teratoid. Teratoma (translated from Greek means freak, deformity, monster) is a tumor arising on the basis of dysontogenesis at any stage of intrauterine development. The displacement of tissue buds, their loss of connection with surrounding growing and developing tissues leads to the fact that these buds are deprived of humoral and reflex influences, which normally regulate the coordinated growth and proportional development of tissues. Thanks to this switching off of regulatory influences, the displaced tissue rudiments acquire a certain autonomy. This autonomy is a prerequisite for the occurrence of true tumor growth.

In adults, teratoid tumors are relatively rare, but in children they are the main type of tumor. So, according to Willis, out of 704 cases of neoplasms in children, dysontogenetic tumors were observed in 339 cases, which is 50%.

The second feature of tumors in children is their frequency benign tumors and the relative rarity of malignant tumors. In adults, malignant tumors are much more common. In our country, benign tumors occur in children from 83.2 to 92.8% of the total number of tumors in children. Accordingly, malignant tumors in children range from 16.7 to 7.2%. There is an opinion that the rarity of malignant tumors in children is associated with the duration incubation period when exposed to cankerogens. That's why malignant diseases occur in other age periods, more often after 40-50 years. The lower frequency of contact of children with carcinogens is also important, as well as, probably, endocrine and exchange features the child's body.

The third feature of tumors in children is the predominance of sarcomas among malignant tumors and the rarity of cancer development, while in adults cancer predominates, sarcomas are less common. It has been suggested that carcinogenic substances received by the fetus in utero from the mother, circulating in the blood, primarily come into contact with tissues of mesenchymal origin - the vascular bed and stroma of organs. The importance of the child’s endocrine status is also pointed out, namely the active functioning of the thymus gland in children and the low activity of the gonads.

The fourth feature of tumors in children is the unique course of some malignant tumors in them compared to the course of malignant tumors in adults. Thus, malignant tumors of internal organs (embryonic nephroma and hamartomas) in children retain the expansive growth pattern characteristic of benign tumors for a long time and do not metastasize for a long time. Timely surgical removal of these tumors leads to a complete recovery of the child. In children, there is sometimes a transition from malignant tumors (for example, neuroblastomas) to benign tumors (ganglioneuromas), which is never observed in adults.

These features of the course of some malignant tumors in children have not yet been explained. The dysontogenetic nature of most tumors in children makes their classification difficult. On the one hand, tissues may be found in the same tumor in children of different origins, on the other hand, the embryonic nature of many tumors prevents the identification of their tissue identity. Therefore, based on isontogenetic development, all tumors in children are divided into three main types:

I. Tumors of the first type. These include teratomas. They arise from the chaotic incomplete detachment of some blastomeres. By histological structure distinguish organismoid teratmas, built not only from various tissues, but also from the rudiments various organs(they are formed, obviously, during the detachment of blastomeres during the blastula period). Organoid teratomas are built from a variety of tissues (they are apparently formed when the germ layers of the embryo are detached during the period of organogenesis). Histioid teratomas consist of a single tissue; they are obviously formed when the development of both the embryonic and early fetal periods is disrupted.)

There are mature and immature teratomas. The tissues of a mature teratoma mature synchronously with the tissues of their host. For example, in the fetus they consist of tissue elements corresponding to the age of the fetus and lose their undifferentiated character by the time the child is born. Therefore, they do not have complete autonomy and lack the ability for progressive growth. This distinguishes mature teratomas from true benign tumors. Teratoblastoma contains immature undifferentiated tissues that do not correspond to the age of the tumor carrier, are capable of progressive growth, give metastases and are true malignant tumors.

II. Tumors of the second type are those that arise from embryonic cambial tissues preserved in the central nervous system, in the sympathetic ganglia and in the adrenal glands during normal age development. Thus, in a child under the age of one year, such immature cambial anlages remain under the ependyma of the ventricles of the brain and gradually disappear by the end of the first year after birth. Immature cellular elements embryonic in nature in the form of sympathogonia with wide potential opportunities growth and differentiation, remain in the adrenal medulla until 10-11 years of age.

A feature of tumors of the second type are certain age periods within which, as a rule, these tumors are observed. These include medulloblastomas, retinoblastomas, and neuroblastomas.

III. Type 3 tumors include those tumors that arise in the adult type. In children, the vast majority of these tumors have mesenchymal histogenesis; the most common are hemoblastosis, osteogenic tumors and mesenchymal tumors of soft tissues.

Particular forms of tumors.

1. Hemangiomas - tumors from blood vessels, have autonomous, progressive growth. There are two main types observed in children: capillary and cavernous hemangiomas.

Capillary hemangioma is a hamartoma (histoid teratoma) of proliferating capillaries, has the appearance of a lobulated nodule of bluish or reddish color. Capillary angiomas are localized mainly in the skin, less often in the liver and other organs. May also ulcerate in children infancy serve as a source of sepsis. They consist of capillaries with 2-3 layers of endothelium. There is blood in the lumens of the capillaries. A feature of capillary angiomas is the infiltrative nature of their growth, which is associated with frequent relapses after their removal.

The tumor never metastasizes.

Cavernous hemangioma is more often a vascular malformation than a true tumor; it is less common in children than capillary hemangioma. It has the appearance of a blue-purple node with a spongy structure in cross-section. Localized in the liver, skin, gastrointestinal tract and other organs. In infants, congenital giant cavernous angiomas are occasionally encountered, occupying 2/3 of the liver volume; children die from bleeding. Cavernous

angiomas consist of vascular cavities separated by connective tissue septa and filled with blood. Due to thrombosis of the cavities with the organization of blood clots, complete cicatricial transformation of the angioma may occur. Blood pigments are found in the scar.

Arterial hemangioma has a branched nature, i.e. extends over a considerable distance. Consists of branching and atypically constructed arterial type vessels. It is extremely rare in children.

Hemangioendothelioma is relatively common in children. This is a capillary angioma with proliferating endothelium. The tumor grows very quickly, but does not metastasize.

Angiosarcoma is a dense, lumpy tumor infiltrating surrounding tissues, with areas of hemorrhage, necrosis and cysts with bloody contents on the section. It consists of strands of polymorphic spindle-shaped, polygonal and round cells, among which gaps and cavities are visible, reminiscent of vessels filled with plasma or blood. Between the strands of cells there is a loose network of argyrophilic fibers. The localization of these malignant tumors can be very diverse; they are more common in children aged 3-5 years.

Tumors from lymphatic vessels- lymphangiomas - are less common in children than hemangiomas. In hypertrophic lymphangioma, endothelial proliferation occurs lymphatic capillaries, formation of cavernous cavities with couplings of lymphoid cells in their walls. Congenital giant lymphangiomas of the bodies are observed, which can reach the size of a child's head. The tumor invades the organs of the neck, leads to asphyxia or becomes secondarily infected. It is impossible to remove it. The tumor consists of numerous cavities lined with endothelium, between them there is loose mesenchymal tissue poor in cells, proliferation of solid strands of endothelium and capillaries is observed, due to which infiltrating growth occurs. Lymphangiosarcomas are rare in children.

Rhabdomyoma is a benign tumor from embryonic muscle cells. It is observed mainly in children. Localized in the heart, in the muscles of the limbs in the form of a node, sometimes reaching big size- up to 10-15 cm in diameter, Brown on the cut. It consists of cells resembling embryonic myoblasts, either in the form of fibers or large cells with a light nucleus and a wide light cytoplasm.

Embryonic rhabdomyosarcoma is a malignant tumor of split off embryonic rudiments of muscle tissue. Longitudinal and transverse striations are found in the cytoplasm. Consists of large cells with large, often polymorphic nuclei, reminiscent of embryonic myoblasts. It is localized ectopically in children in the pelvic organs: in the bladder, vagina, pelvic tissue, gonads, less often in the nasopharynx and other organs. Infiltrating the pelvic organs, causing obstruction urinary tract up to the development of uremia, ulcerates, bleeds, and is subject to secondary infection. Metastasizes to regional lymph nodes and hematogenously to the lungs.

Embryonic nephroma (Wilms tumor, adenosarcoma) is a malignant kidney tumor that develops from the renal anlage - metanephrogenic tissue. It grows expansively for a long time, within the capsule, squeezing and pushing aside the kidney tissue. Sometimes it reaches gigantic sizes. In section, it is pinkish-white in color, nodular in appearance, with foci of fresh and old hemorrhages. Later, the tumor grows into surrounding tissues and metastasizes hematogenously to the lungs. With rare exceptions, embryonal nephroma (nephroblastoma) occurs only in children and is the fourth most common tumor among them. It is observed in children aged 1 to 4 years.

Hepatoblastoma is a malignant liver tumor that develops from an embryonic anlage. In the form of numerous whitish-yellowish nodes, liver tissue sprouts, grows quickly, metastasizes to the lymph nodes and hematogenously to the lungs. The tumor occurs mainly in infants and young children. Fatal outcome may occur in a child before the development of metastases from hemorrhage in abdominal cavity, since the tumor is rich in blood vessels and bleeding sometimes occurs with minor trauma.

Organismoid and organoid teratomas are expansively growing tumors, often reach a large size, and consist of a variety of tissue structures derived from all three germ layers.

Teratoblastoma is a malignant tumor in which there are fields of immature, actively proliferating embryonic tissue, often of unclear histogenesis, which can be combined with elements of mature tissue. Teratoblastomas grow quickly and metastasize. Teratomas have a certain typical location for them - ovaries and testicles, sacrococcygeal region, mediastinum, retroperitoneal space, pharynx, base of the skull.

Medulloblastoma is a malignant tumor of neuroectodermal embryonic stem cells - medulloblasts. Metastasizes via the cerebrospinal fluid route within the central nervous system. Very rarely gives hematogenous metastases to the lungs.

Retinoblastoma is a malignant tumor of embryonic undifferentiated cells of the retina. The tumor grows, the surrounding tissue leads to protrusion of the eye, disfigures the face and grows into the area of ​​the base of the skull. Metastasizes to the bones, liver, less often to the lungs, as well as to the lymph nodes.

Neuroblastoma is a malignant tumor of stem cells of the sympathetic ganglia and adrenal medulla. It is more common in children under one year of age, but can occur up to age 11.

Tumors in children are observed relatively often, in 5-7% of all surgical diseases. Benign tumors predominate, their ratio to malignant ones is approximately 9:1. Malignant tumors in children are usually of mesodermal origin, while in adults they are of epithelial origin. In this regard, sarcomas are more common in children and cancer is extremely rare.

Tumors in children occur in all age groups starting from the neonatal period. In most cases, they are detected from birth or in the first months and years of life, usually before the age of 5-6 years. Tumors affect girls slightly more often than boys (in a ratio of 3:2).

Various types of tumors that are observed in adults are also observed in children. However, there are neoplasms that are characteristic only of childhood. These tumors exhibit a number of features.

In the etiology and pathogenesis of tumors in children, an important role is played by the vicious development of embryonic primordia, incorrect location cell groups of various germ layers. Thus, a significant part of vascular tumors, which are the most common and characteristic neoplasm of childhood, is a developmental malformation of the hamartoma type. A number of authors believe that the most characteristic of childhood are tumors that develop due to developmental anomalies, i.e., tumors of dysontogenetic origin. It is believed that deviation from the normal development of the kidneys can serve as a basis for the occurrence of Wilms tumor (see Wilms tumor). Thus, in children, a number of tumors are on the verge of a malformation and a true neoplasm.

A feature of childhood tumors is their development from poorly differentiated tissue. In some cases, differentiation is so poorly expressed that it is impossible to determine from which germ layer the tumor originates.

The histological picture of some immature tumors in children does not reflect malignancy, but only insufficient tissue maturity and is sometimes so peculiar that it does not even allow one to distinguish cells of connective tissue origin from epithelial ones. This makes it extremely difficult morphological diagnostics tumors in children.

The clinical course of many tumors in children also has its own characteristics. For example, a hemangioma, being benign in its histological structure, at the same time resembles a malignant tumor in its rapid infiltrating and sometimes destructive growth. Neuroblastomas have been described that have taken a benign course due to the fact that neuroblasts can mature and differentiate into mature ganglion cells. Melanoma in children is clinically benign, and after puberty it becomes malignant course. Adenosarcoma of the kidney (Wilms tumor), being encapsulated, does not manifest itself for a long time and remains “silent”. Teratomas consisting of mature tissues can metastasize. Despite the fact that some tumors in children, built from immature cells, are clinically benign, they should be considered as potentially malignant, since they can become malignant at any time.

Thus, in children, the boundaries of benignity and malignancy of tumors are less pronounced than in adults, and are often erased.

Features of tumors in children and their clinical course

Individual types and localizations of cancer represent separate and distinct nosological units, individual diseases that may have different reasons and to which they should apply various ways treatment and prevention. However, this entire group of diseases is based on a single pathological process.

The occurrence of a tumor over a long period was explained by the presence in the body special cells, cells with special potencies left over from the embryonic period. Without entering detailed analysis numerous variants of this point of view, it should only be pointed out that at present the theory of embryonic rudiments associated with the name of Conheim must be radically revised. If this theory can still explain the formal genesis of some so-called dysontogenetic tumors, then it cannot explain their causal genesis in any case. All the data of modern experimental oncology, indicating that any tumor can be caused from the growing elements of an adult organism, and not from isolated embryonic rudiments, speaks against Conheim's theory.

Virchow’s “irritation theory” played a historical role in the study of the etiology and pathogenesis of tumors. The individual facts and observations underlying this theory are still relevant today. However, modern data do not fit into the framework of the “irritation theory”. Thus, one of the main provisions of this theory was that the agent tumor-inducing, should “irritate” tissues, cause repeated damage, inflammation and regeneration in them. Meanwhile, the study of the genesis of numerous dishormonal tumors, both in humans and in experiments, convinces us that inflammation is not an obligatory stage in the development of tumors, and that tumor restructuring and tissue proliferation differ from both inflammatory and regenerative restructuring. Based on a number of experimental oncological observations and especially the study of filterable tumors and some viral agents, a hypothesis was created about viral etiology tumors. So far, however, there is no sufficient evidence of the possible role of exogenous viruses in the etiology of human tumors.

Currently, no one doubts that the causes of tumors can be varied. All researchers, with rare exceptions, share the polyetiological theory of the origin of the tumor. Doubts and disputes concern, however, significant individual aspects of the problem. For example, it is a very common idea that the basis for the occurrence of a tumor is an abrupt change in the nature of the mutation, the so-called. somatic mutation [Bauer]. It is believed that all blastomogenic agents act as mutagens. However, the gradual development of a tumor from pretumor changes, the progression of neoplasms from “dependent” to completely “autonomous” is difficult to explain by a single somatic mutation in one cell. The latest experimental data on the study of the blastomogenic effects of radiation also do not fit into the framework of the concept of somatic mutation [Blum, Bruce].

So, under the influence of various exogenous and endogenous blastomogenic agents, some of which are already known, and some remain to be studied, tumors may arise, the course of development of which and the consequences are already quite well known to us. Tumors are the proliferation of qualitatively changed body cells. Therefore, it is quite natural that in their pathogenesis, along with the causative agents, implementing factors that depend on the organism as a whole play a very significant and sometimes decisive role. The body’s nutrition plays a certain role, and changes in nutrition are also associated with changes in metabolism and tissue reactivity. Heredity is important as a factor that determines the potential for growth and differentiation of cells and tissues, as well as the course and direction of metabolic processes. In the light modern ideas about the unity or close relationship of those molecular biological units that determine heredity, differentiation and cell metabolism, one should think that in changes of this kind of tissue particles lies the key to the pathogenesis of the tumor.

Features of tumors in children

Pathological anatomy. Of the childhood diseases, O. causes the highest mortality and is not very rare. According to the Surgical Children's Clinic of the 2nd MMI named after. N.I. Pirogov for 11 years (1947-1957), cancer patients accounted for 5% of all surgical patients in this clinic (M.V. Volkov). There are indications that recently there has been an increase in the number of tumors in children. Thus, according to data from American children's hospitals, 62,020 autopsies during the period from 1930 to 1934 accounted for 242 cases of tumors, and for the period from 1950 to 1954, 20,928 autopsies accounted for 425 cases of tumors [McGregor, 1958]. It is emphasized that the increase in the incidence of tumors in children is not only relative, but also absolute. According to E.P. Semenovva, compiled based on materials from children's medical institutions in Leningrad, from 1907 to 1946, tumors in children were found in no more than 1% of all autopsies, during the period from 1947 to 1951 the number of such autopsies increased to 2%, and from 1951 to 1956 g.-doz%. However, according to E.P. Semenova, the increase in the number of tumors in children over the past 10 years is only relative, because Mortality from infectious and other diseases has decreased significantly. Consequently, the question of the absolute increase in the incidence of tumors in children over the past 10 years remains unresolved. There is a slight predominance of tumors in girls.

According to Farber, malignant tumors most often occur in the first half of life and between 6 and 7 years. There are numerous descriptions of tumors in newborns and fetuses, as well as data on intrauterine metastasis. The most characteristic of childhood tumors is their occurrence on the basis of developmental anomalies (angiomas, lymphangiomas, Wilms tumor, dermoids and other teratoid tumors) or on the basis of undifferentiated embryonic elements present in the child’s body that have retained a greater ability to grow (ganglioneuromas, neuroblastomas, sympathogoniomas, fibroids from myoblasts, etc.). Thus, most childhood tumors are associated with ontogenetic disorders.

It must be borne in mind that in some cases it is difficult, and sometimes simply impossible, to distinguish a developmental anomaly from a true blastoma that has arisen due to a developmental anomaly.

Most dysontogenetic tumors of childhood are benign in nature and occur 4"/2 -5 times more often than malignant ones (E.P. Semenova). In addition to the factor of ontogeny disturbance, the occurrence of a tumor is associated with the strong growth of certain tissues in childhood: the osteochondral skeleton, lymph, apparatus (especially the digestive tract), in which, in addition, there is a rapidly emerging and pronounced proliferative reaction to the influence of various irritants. This is associated with the relative frequency of sarcomas of bone tissue and lymph nodes in childhood (M.A. Skvortsov. Sarcomas are much more common in children than cancers. Cancerous tumors are observed very rarely, mainly in older children. gastrointestinal tract and ovaries. The question of the predominance of sarcomas and the rarity of cancerous tumors in childhood in the crust remains poorly developed.

Benign tumors, which occur mainly in childhood, primarily include angioma and lymphangioma. In second place are teratoid tumors such as dermoids or epidermoids, various kinds papillomas and nevi.

Among malignant tumors, tumors of the central nervous system and eyes occupy the first place (E.P. Semenova): medulloblastomas, retinoblastomas, glioblastoma multiforme - 24%; on the second - various types of sarcoma - 23%; in the third - angioneuroblastomas, neuroblastomas, sympathogoniomas - 14%; on the fourth - embryonic O. kidneys - 10%; in fifth place - lympho- and reticulosarcoma - 9.2%; followed by lymphosarcomatosis - 7% and cancer tumors - 2.8%. The remaining 10% fall on melanomas, which are relatively rare in children, developing more often during puberty, dysgerminomas, teratoblastomas, cytoblastomas, chorionepitheliomas and seminomas.

The criterion of malignancy and benignity for childhood has a somewhat unique meaning and must be decided each time taking into account the growth characteristics of a given type of tumor. Thus, some malignant tumors of childhood that give extensive metastases can grow for a long time and reach large sizes within their capsule, for example, granulosa cell carcinomas of the ovaries and embryonal kidney tumors. Along with this, benign tumors - hemangiomas, lymphangomas, hemangio-endotelpomas - produce infiltrating growth, but, as a rule, do not metastasize.

Microscopically, the degree of cell differentiation and the number of mitoses are also not a criterion of malignancy for childhood tumors. Thus, hemangioendotheliomas consist of poorly differentiated endothelial cells with a large number of mitoses, but, as a rule, do not metastasize. Along with this, teratomas can have a completely mature organoid and even organismoid structure and metastasize widely.

Since many childhood tumors develop from embryonic rudiments, a feature of their histological structure is low differentiation cell forms, which makes morphological diagnosis extremely difficult. In some cases, differentiation is so low that it is not even possible to resolve the question of which germ layer (ectoderm or mesoderm) the tumor originates from. At the same time, some malignant tumors of childhood tend to mature in the area of ​​either the main node or metastases, thereby revealing their true origin. These are, for example, sympathogoniomas, which can “mature” to the point of ganglioneuroma. The question of the reason that causes such maturation of tumors in childhood remains open.

Cases of transmission of malignant tumors through the placenta from mother to fetus are very rare. Potter points to 4 similar observations: one of them relates to bronchogenic cancer, the other to lymphosarcoma, and the last two.

Clinical course. Various types of tumors that are observed in adults are also observed in children. However, there are tumors that are characteristic only of childhood. These O. exhibit a number of features, which makes it possible to single them out and talk about them separately. The previous opinion about the rarity of tumors in children has changed with the development of pediatric surgery and improved diagnosis. Tumors in children account for about 5% of all surgical diseases childhood; Malignant tumors are less common than benign tumors, accounting for 0.6 - 0.7% (K.A. Moskacheva, M.V. Volkov and M.M. Brzhozovsky, E.P. Semenova, etc.). A.P. Shanin, citing materials from foreign literature, points out that the mortality rate of children from tumor diseases far exceeds the mortality rate from tuberculosis, heart disease and infectious diseases. According to Pak and Eriel, in children the highest mortality from O. is observed in early age- up to 4 years, significantly decreasing in subsequent age periods of childhood.

The predominant amount falls on tumors of a vascular nature, ch. arr. hemangiomas, which is confirmed by literary data (N.V. Shvarts, N.I. Kondrashin).

Then there are bone tumors, dermoid cysts and teratomas; enough frequent tumor is a rectal polyp. Of the malignant tumors, only sarcomas were observed. According to most authors, cancer in children is extremely rare.

Tumors in children occur in all age groups, starting from the neonatal period. In the vast majority of cases, they are detected from birth or in the first months and years of life. The diagnosis is made more often before the age of 5-6 years. O. affects girls somewhat more often than boys (in a ratio of 3:2).

The detection of tumors in children from birth or in the first years of life has allowed a number of authors to express the opinion that tumors in children are congenital in nature and are developmental defects. A huge role in the etiology and pathogenesis of tumors in children is played by the incorrect development of embryonic rudiments, the incorrect arrangement of cell groups of various germ layers during intrauterine development embryo. According to Small, congenital anomalies of tissues and organs are foci for the occurrence of childhood tumors. Such formations include dermoid and branchiogenic cysts. Malignant kidney tumors can develop as a result of kidney malformations (K.A. Moskacheva), etc.

The clinical course of many tumors in children has its own characteristics; for example, a hemangioma, being benign in its histological structure, at the same time resembles a malignant tumor in its rapid and infiltrating growth. Neuroblastomas have been described that have taken a benign course due to the fact that neuroblasts can sometimes differentiate into ganglion cells. Koop describes a favorable course with recovery of retroperitoneal neuroblastoma with metastases in a young child. Melanoma in children throughout childhood, it can clinically have a benign course, and after puberty it acquires a malignant course (Yu.F. Isakov). A kidney tumor (Wilms tumor), being encapsulated, does not show itself for a long time, remains “silent” and therefore is almost always discovered by chance (S.D. Ternovsky, N.V. Shvarts, K.A. Moskacheva, Kup et al. ).

Thus, with tumors in children, the boundaries of benignity and malignancy are less pronounced and often blurred.

Noteworthy is the paucity or complete absence of anamnestic data. Often the first sign is the discovery of the tumor itself, which did not manifest itself in any way and was discovered by the mother or doctor by chance. The child rarely complains of pain and, if there is any, he cannot always localize it. It is necessary to be attentive to such signs as the child’s lethargy, poor appetite, intestinal disturbances, fever, and anemia. Such signs general may be a manifestation of a malignant tumor. With unclear, sometimes inexplicable symptoms, one should not forget about the possibility of a tumor in the child.

The most common and characteristic tumors in children are vascular tumors. The question of the nature and origin of these tumors remains unresolved to this day, because No consensus, whether they relate to developmental defects or true neoplasms. Hemangioma, being a benign tumor, often leads to severe cosmetic and functional disorders. Intense and infiltrating growth brings it closer to a malignant neoplasm. But the peculiarity of this growth is that the hemangioma grows rapidly during the first year of the child’s life, then the growth rate weakens or growth stops altogether. They occur more often in girls than in boys - in a ratio of 2: 1 [Grob, Pak and Eriel et al.]. Diagnosis of hemangiomas is not difficult. Treatment can be surgical and conservative, the latter includes sclerosing and radiation therapy. In heavy advanced cases it is necessary to resort to combined treatment, using both surgical and conservative treatment simultaneously. Due to rapid growth Hemangiomas should be treated as soon as possible after diagnosis.

The second tumor of a vascular nature is lymphangioma. Unlike hemangiomas, lymphangiomas do not have any pattern of growth. They can give rapid growth immediately after the birth of the child or long years remain the same size, and then for no apparent reason give intensive growth. The diagnosis is made based on the cystic nature of the tumor with unclear, blurry boundaries that imperceptibly blend into the surrounding tissue. Treatment of lymphangiomas is surgical, often presenting great difficulties and danger. If lymphangioma in a young child does not increase in size, then surgery should be performed at school age. Conservative treatment methods are ineffective.

Teratoma should be considered a characteristic tumor of childhood. Teratomas of the sacrococcygeal region are more common in girls and can be very large, pushing the rectum anteriorly, squeezing it and the urethra. In this case, the acts of defecation and urination are often disrupted. According to S.D. Ternovsky, a teratoma can consist of mature tissues or embryonic tissues, in which case it is malignant. Features of malignancy (cachexia, metastases) usually appear after a year, therefore surgery should be performed no later than the first year of life (O.V. Blagoveshchenskaya), i.e. during the benign course of the tumor. Teratomas of any location are subject to surgical treatment.

Rectal polyps are of practical importance in terms of frequency in children. They represent a benign adenoma (S.D. Ternovsky, N.V. Shvarts) or the growth of the mucous membrane as a result of its irritation inflammatory process(S.D. Ternovsky). The polyp is most often located on back wall intestines, 2-3 cm higher anus, has a leg, causes minor bleeding during defecation. More frequently observed single polyps do not cause severe disorders. Multiple rectal polyps cause intestinal dysfunction, significant bleeding, colitis and proctitis due to the addition of a secondary infection.

As in adults, mediastinal tumors of neurogenic origin, dysontogenetic and vascular, occur in children; Lipomas and fibromas are observed relatively rarely. Depending on the location, these tumors may be asymptomatic and are diagnosed mainly by x-ray. When localized in the mediastinum, in some cases they cause disorders of its organs; then, regardless of age, surgical intervention should be undertaken, which should be performed in medical institutions with appropriate equipment.

Malignant tumors in children are predominantly of mesodermal origin, while in adults they are more often of epithelial origin. A typical malignant tumor of childhood is sarcoma. The localization of sarcomas is varied, but the most important in children are sarcomas of the kidneys, mesenteric and retroperitoneal lymph nodes. With any abdominal tumor in a child, first of all, one must remember about the possibility of kidney damage (S.D. Ternovsky). Wilms described in detail a kidney tumor characteristic of childhood, which is therefore called Wilms tumor . Most authors note that Wilms tumor is observed more often in children aged 1 to 5 years, but cases of these tumors have been described in newborns and even in premature stillborns, which indicates their congenital nature.

Sarcoma of mesenteric lymph nodes is more severe and quickly leads to exhaustion of the child, although complaints of pain and intestinal disorders may be absent for a long time, even when the tumor moves to the intestinal wall.

Of the benign bone tumors, the most common are juvenile exostoses, osteoblastoclastomas, and less commonly osteomas and chondromas. Malignant tumors are observed osteogenic sarcoma and Ewing's sarcoma .

One of current problems modern medicine is the study of the occurrence and development, subsequent diagnosis and treatmenttumorsthat appear in children of different ages.

Let's take a closer look attumorschildhood, the formation of which occurred at the stage of embryonic development. This process is closely related to the features that arise when the differentiation of embryonic cells stops or significantly slows down, causing normal development. The mechanisms of regulation of this process are poorly understood, but active scientific research is being conducted in this area.

Disorders of differentiation are considered to be characteristic feature tumor cells, this fact is used in diagnosis. Case in point -tumor, consisting of undifferentiated embryonic cells or teratoma. It occurs when the formation of a tissue rudiment is disrupted at any stage of embryonic development. This area loses connection with surrounding tissues, which continue to grow. And, as a result, it turns out to be deprived of influences regulating the growth and necessary development of tissues.

Usually there are several such rudiments. They acquire autonomy and remain unchanged for quite a long time after the baby is born. It is these “residues” that have not undergone differentiation that can become the basis for the onset of malignant proliferation of tumor cells, since they have a similar nature to them. In this regard, the following types of malignant tumors are distinguished:tumors:

  • Undifferentiated or adenogenictumor. It is characterized by rapid and aggressive growth, early appearance of metastases, frequent relapses and a worse prognosis for patient survival.
  • Poorly differentiated cancer. Tumor cells are distinguished by the same atypicality; they in no way resemble healthy ones.
  • Moderately differentiated cancertumor. Its malignancy is classified as moderate.
  • Highly differentiated cancer. It has low malignancy, and the cells are similar to healthy ones.

What is Williams tumor characterized by?

Associated with violations embryonic development and the occurrence of cancertumorsWilliams or neuroblastoma. It is diagnosed mainly in infants or newborns, and in three- to four-year-old children. IN in rare cases thistumoroccurs in patients older than 10 years. Neuroblastoma accounts for about 7% of cases of childhood cancer pathologies.TumorWilms' disease usually affects one kidney. This disease was first identified by the German surgeon Max Wilms, after whom it was named.tumor. At an early stage, the symptoms of neuroblastoma may not be noticeable. But later they appear as follows:

  • the presence of blood in the urine;
  • a round and dense formation palpable on the abdomen
  • pain either in the kidney area or in the abdomen;
  • increased blood pressure;
  • sweating, body temperature can reach 38-40 degrees;
  • swelling in the legs.

Various forms are knowntumorsWilms. It can only be located within the kidney and not affect neighboring tissues, then it can be relatively easily removed surgically. In casetumorneuroblastoma also spreads to neighboring tissues; the success of the operation depends on both the presence of metastases and their number. In some casestumorWilms disease can cause complications in lymph nodes distant from the kidneys and in other organs. Probability of birthbabysuffering from this typetumors, is associated with the age of the mother; if a woman is over 35 years old, then the risk increases.

Common types of cancer tumors in children

The overall incidence of malignant tumors in childhood is significantly lower than in adults. But this does not reduce the mortal threat to the lives of young patients who do get sick. Approximately one third of cancer cases in children are blood diseases (leukemia or leukemia). Besides genetic factors, the development of childhood malignant neoplasms can be provoked by such environmental influences as:

  • excess ultraviolet radiation due to solar radiation;
  • radon exposure;
  • smoking in the presence of the baby or attempts to smoke by the teenager himself;
  • carcinogenic substances that may be contained in air, water or products;
  • unhealthy diet, predominance of smoked and fried foods, lack of vitamins and fiber;
  • long-term use of certain types of drugs - barbiturates, diuretics, androgens, immunosuppressants;
  • some viral infections (infection of the baby with herpes, hepatitis B, Epstein-Barr viruses).

Most often, young patients are diagnosed with leukemia, the essence of which is the defeat of a malignanttumorblood cells or bone marrow(34% of the total number of childhood cancer pathologies). In second place (27%) –tumors, affecting the brain and other parts of the nervous system. In most cases thistumorappear in the cerebellum and brain stem. When cancer affects the lymphatic system, it is called lymphoma. The share of such well-known types of this disease as Hodgkin's lymphoma and lymphoma. non-Hodgkin's, accounts for 4% of cases of malignant childhoodtumors.

The occurrence of malignant neoplasms in the soft tissues of the neck, groin, abdomen, pelvis, and extremities signals rhabdomyosarcoma (3% in the general picture of childhood oncopathologies). Eye cancer ortumorretinoblastoma occurs with the same frequency (3%), usually at an early age (up to 2 years). Bone cancer affects children most often in the form of osteosarcoma and Ewing's sarcoma. Osteosarcoma is common in adolescents and usually develops where bone tissue is growing most actively. As for Ewing's sarcoma, thistumorappears on the bones of the legs, pelvis, ribs, shoulder blades.

Statistical information provided by the American Cancer Society made it possible to determine with a high degree of probability the values ​​of 5-year survival rates for children with cancer. Five years are accepted by doctors as the optimal period for monitoring the health of a small patient after treatment for malignanttumors. After their expiration, the risk of further relapses is significantly reduced, so in the majority of cases we can talk about recovery. So, the survival rate of children observed from 2002 to 2008 was characterized by the following percentage indicators during treatment:

  • leukemia - 84%;
  • brain cancer (and nervous system) - 71%;
  • Wilms tumors - 89%;
  • Hodgkin's lymphoma - 96%;
  • non-Hodgkin's lymphoma - 86%;
  • rhabdomyosarcomas - 68%;
  • neuroblastoma - 75%;
  • bone cancer or osteosarcoma - 71%.

These are aggregate indicators and therefore cannot serve as the only source medical forecasts. In each specific clinical case factors such as size and location influence survivaltumors, sensitivity and resistance of the body, the age of the patient, as well as the type of treatment.

How to recognize cancer tumor in childhood

Symptoms of malignanttumorsin childhood they may resemble common diseases and injuries (hematomas, lumps). But in order to avoid tragic developments whentumorhas already been diagnosed late stage and cannot be treated, parents should take a number of preventive measures. It is recommended to ensure regular medical examinations of the baby and independently monitor whether they are manifested in his appearance or behavior. warning signs. Among the symptoms that signal the need for a thorough medical examination of children for the appearance of malignanttumors:

  • the appearance of unusual swelling or lumps;
  • pale face, unexplained weakness;
  • frequent occurrence of hematomas;
  • feeling constant pain in a specific area of ​​the body;
  • sudden lameness;
  • the appearance of a feverish state;
  • frequent headaches, especially if they are accompanied by vomiting;
  • the baby began to see worse for no obvious reason;
  • phenomenon of rapid weight loss.

Most symptoms may indicate an infection or injury, but a doctor's examination never hurts. If there have been previously reported cases of cancer in the family, then it is better to insure yourself as much as possible and keep the child under constant medical supervision.

Aspects of treatment of malignant tumors childhood

Treatment of cancer in children requires a special approach, which should be based on scrupulous medical supervision.It can only be successful if the early diagnosis. A number of objective difficulties can lead to the occurrence of medical errors in this area. They are due to the fact that quite oftentumorsin children they can be located in hard-to-reach areas, such as the retroperitoneum, mediastinum, and cranial cavity. Symptoms of malignanttumorsin children are nonspecific, long time can occur under the guise of other diseases.

In some cases, oncological diseases in childhood do not have characteristic clinical manifestations at all for a long time. This may also be a factor in late diagnosis. According to Russian statistics, approximately a quarter of young patients are diagnosed with cancer (tumor) at a very early stage. Additional difficulties in diagnosing childhood cancer are caused by the fact that babies cannot clearly explain what they are feeling, and in the case of infants, when compiling an anamnesis, doctors have to rely only on the accuracy of their parents’ observations.

Choosing a treatment method for malignanttumorsin children depends mainly on the stage at which the disease is located. The treatment program may include chemotherapy, surgery, radiation therapy, and other methods. Various combinations of these treatments are often used. Cancer in children due to rapid growthtumors(which is associated with the overall growth of the child’s entire body), responds well to chemotherapy. Children recover better than adults after receiving high doses chemotherapy.

If more intensive treatment options are used, this increases the chances of success, but the risk of developing side effects short-term and long-term action. For example, inas an element complex treatment quite often (in 70% of children suffering from malignanttumors), radiation therapy is used. But when using it, subsequent complications sometimes arise.That's why oncologists accept everything possible measures in order to achieve an acceptable compromise between the intensity of treatment for childhood cancer and possible negative post-treatment effects. Among them are:

    • problems with lung function (may be caused by specific chemotherapy drugs or radiation therapy);
    • occurrence of delays in physical development and in the growth of the child (when treatment negatively affected the functioning of the musculoskeletal system);
    • deviations that have arisen in the field of sexual development and possible consequences for girls, creating problems with conceiving and giving birth to a child in adulthood (infertility);
    • in the field of mental development of the child, problems may arise associated with deterioration in learning;
  • the emergence of an increased risk of developing a new cancer pathology.

Classification diagnostic studies

Carrying out diagnostics in the event of the appearance of malignanttumorsin a child, the doctor answers the following questions: where is thetumor, what morphological structure it differs from, what stage of development it is at, whether it has metastases. Such a thorough study suggests the use of radiation methods. The list describing modern clinical, diagnostic and laboratory methods for diagnosing malignanttumors in children, includes:

  • Collection of clinical and anamnestic data. It also includes the study of hereditary pathologies in the family and among other relatives of the child (so-called proband studies).
  • Data obtained by medical imaging include the results of RTK or CT, ultrasound and MRI. It also takes into account the results of using a wide range of radiographic methods and radioisotope studies.
  • Data that was obtained through a variety of laboratory tests: biochemical, histological and cytological. Also included in a number of laboratory studies are the use of optical, laser and electron microscopy, immunofluorescence and immunochemical analysis.As one of modern methods in-depth diagnosis of cancer is becoming increasingly popularpathomorphology of tumors in children. It is a microscopic examination of samples of body tissue taken during operations and biopsies.
  • Results of molecular biological studies of DNA and RNA. Their types include the usean experimental method of molecular biology that involves a significant increase in small concentrations of DNA fragments in samples biological material(PCR), carrying outcytogenetic analysis. And also the use of a method, the essence of which isidentifying a specific DNA sequence in a tissue sample, the so-calledSouthern blot analysis.

An undeniable advantage of the ultrasound method is the absence of negative biological effects on the child’s body. When using devices that operate in real time, internal organs can be easily visualized in a two-dimensional image. This allows us to assess their anatomical and functional status, determine the nature and prevalence of the tumor process.

Traditional X-ray diagnostics continues to be important in diagnosing children suffering from many types of oncological diseases. But computer and magnetic resonance imaging (CT and MRI) are still more informative. Using CT, you can study any part of the body wheretumor, with much greater resolution than conventional radiography. Computed tomography reveals a pattern of micrometastases in the lungs that are not visible using conventional radiography.

Therefore, the use of CT is necessary when implementing primary diagnosis in children sufferingtumorsthat give metastases in the lungs. The only negative is limited use computed tomography for studyingtumorsin infants. As for magnetic resonance imaging, it can be called such a highly sensitive method that its capabilities surpass both ultrasound and CT.

MRI is simply irreplaceable when examining children withtumorspostcranial fossa or spine. With the radionuclide diagnostic method, possible dysfunctions of organs and systems and metabolism are studied. Also, using this method, you can obtain a fairly clear anatomical and topographic image of internal tissues and organs.

Finally, we present statistical data that convincingly demonstrates the need for early diagnosis in childrentumorsmalignant in nature. With the zero stage of cancer, the child’s recovery is 100% guaranteed, with the first stage – 90-95%. Treatment of the second stagetumorsgives a chance of recovery in 70-80% of cases of the disease, in the third stage of developmenttumorsOnly 30% of children can be saved from death. The most severe threat occurs at the fourth stage; children with this diagnosis are cured only in 10% of cases.

The effect of age on tumor growth is manifested in some features of tumors in children compared to those in adults.

The first feature tumors in children is frequent occurrence them from embryonic tissues as a result of disturbances in the formation of organs and tissues during the period of intrauterine development of the embryo. Therefore, the vast majority of tumors in children develop from tissues that were delayed in their development at the embryonic level, often located in the wrong place - dystopically. Tumors from embryonic tissues are called dysontogenetic, or

teratoid. Teratoma (from Greek teratos

- freak) - a tumor of embryonic undifferentiated germ cells that occurs when their migration is disrupted during the morphogenesis of the gonads of the embryo. Disruption of the morphogenesis of the tissue rudiment at any stage of embryo development, the loss of its connection with the surrounding growing tissues lead to the fact that this rudiment is deprived of humoral and reflex influences that normally regulate the coordinated growth and proportional development of tissues. As a result of this switching off of regulatory influences, the displaced tissue rudiment acquires a certain autonomy. The latter is probably a prerequisite for the occurrence of true tumor growth.

In adults, teratoid dysontogenetic tumors are rare; in children, they are the main type of tumor. tumors in children is the frequency of benign tumors and the relative rarity of malignant tumors, while in adults malignant tumors are more common.

The most common benign tumors in children are skin tumors - angiomas and nevi.

The third feature tumors in children are the predominance of sarcomas among malignant tumors and the rarity of cancer, while in adults the opposite ratio is observed.

Lymphosarcomas and osteosarcomas predominate among sarcomas in childhood. Cancer occurs mainly in organs that are not in contact with the external environment, endocrine glands - thyroid gland, adrenal glands, gonads. Stomach cancer is observed as an exception in children aged 10-11 years. It has been suggested that carcinogenic substances received by the fetus transplacentally from the mother, circulating in the blood, co-

They contact primarily the tissues of the internal environment of mesenchymal origin - the vascular bed and stroma of organs, as well as endocrine glands, the brain, and internal organs.

The fourth feature tumors in children is the uniqueness of the course of some malignant tumors in them compared to those in adults. Thus, malignant tumors of internal organs (embryonic nephroma and hepatoma) in children for a long time retain the expansive growth pattern characteristic of benign tumors and do not metastasize for a long time. Along with this, some benign tumors in children exhibit infiltrating growth, such as angiomas. In children, there is an amazing phenomenon of the transition of malignant tumors (for example, neuroblastomas) to benign ones (ganglioneuromas), which is not observed in adults; this phenomenon is called tumor reversion. Malignant tumors occur mainly in children from the neonatal period to 6 years of age. More often they occur at the age of 3-5 years, which indicates the importance of intrauterine carcinogenic influences, probably coming from the mother.

Classification. The dysontogenetic nature of most tumors in children makes it difficult to create their classification. On the one hand, in children, tissues of different histogenesis can be found in the same tumor; on the other hand, the embryonic nature of many tumors prevents the identification of their tissue identity. Therefore, it is not always possible to classify tumors in children according to histogenetic principles, as tumors in adults are classified. Based on the principles of ontogenetic development, all tumors in children can be divided into three main types.

TO type I tumors relate teratomas, dysontogenetic, teratoid, dysontogenetic, tumors. The occurrence of teratomas is currently explained by a violation of the migration of germinal germ cells during the formation of the caudal end of the urogenital ridge. The separation of part of the undifferentiated germ cells from the caudal end of this ridge and their persistence lead to the development of teratomas.

Based on the histological structure, they are distinguished histioid, organoid And organismoid teratomas(cm. Tumors). There are also teratomas that develop from elements of the embryoblast - embryonal teratomas and developing from trophoblast elements - extraembryonic teratomas.

There are mature embryonic and extraembryonic teratomas and immature - teratoblastoma.

The tissues of a mature teratoma mature synchronously with the tissues of their host. For example, in a fetus they consist of tissue elements corresponding to the age of the fetus, and lose their undifferentiated character by the time of birth. Therefore, they do not have complete autonomy and lack the ability for progressive growth. This distinguishes mature teratomas from true benign tumors. Teratoblastoma contains immature undifferentiated tissues, often

extraembryonic, capable of progressive growth, metastasizes and is a true malignant tumor.

Histioid teratomas are otherwise called hamartomas; in the malignant version - hamartoblastomas. Hamartoma (from Greek. hamarta- error) - a tumor from embryonic tissue, delayed in its differentiation compared to the tissues of the tumor carrier, developing from excessively disproportionately developed tissue complexes, for example, from excessively developed blood vessels. Hamartomas in children include angiomas, nevi, embryonal tumors of internal organs, embryonal tumors of mesodermal and mesenchymal histogenesis (benign and malignant). The group of hamartomas also includes tumors that develop on the basis of run through- remnants of organs of the embryonic period that have not undergone timely involution, for example tumors from the remnants of the dorsal chord, gill arches, pituitary tract, etc.

TO type II tumors include those that arise from embryonic cambial tissues, preserved in the central nervous system, sympathetic ganglia and adrenal glands during normal development. Thus, in a child under the age of 1 year, such immature cambial anlages remain under the ependyma of the ventricles of the brain and gradually disappear by the end of the first year after birth. Immature cellular elements of an embryonic nature in the form of sympathogonia with wide potential for growth and differentiation remain in the adrenal medulla for up to 10-11 years. A feature of type II tumors are certain age periods within which, as a rule, these tumors are observed. However, it is not possible to accurately distinguish them from hamartomas, and the identification of this type of tumor is somewhat arbitrary. Type II tumors include medulloblastoma, retinoblastoma, neuroblastoma.

TO type III tumors relate tumors that arise like adult tumors. In children, the vast majority of these tumors have mesenchymal histogenesis; the most common are hemoblastosis, osteogenic tumors and mesenchymal tumors of soft tissues. Epithelial benign tumors - papillomas and polyps - occur relatively often in children. Epithelial malignant tumors (cancer) are rare in children; cancer of the endocrine glands and genital organs occurs mainly.

Dysontogenetic tumors

Hamartomas and hamartoblastomas of vascular origin

Hemangioma- a tumor of blood vessels, has an autonomous, progressive growth, in contrast to hemangioectasias, local dilation of blood vessels, in which there is no progressive growth. There are two main types observed in children: capillary and cavernous hemangiomas.

Capillary, dysontogenetic, hypertrophic, hemangioma(cm. Tumors)- hamartoma of proliferating capillaries, has the appearance of a lobular nodule of bluish or reddish color. This is the most common benign tumor in children. However, sometimes, especially in infants, it begins to grow rapidly. Rarely occur giant congenital partly capillary, partly - cavernous hemangiomas(Fig. 326), accompanied by thrombocytopenia, massive bleeding with a fatal outcome (Kasabach-Merritt syndrome). Capillary angiomas are localized mainly in the skin, less often in the liver and other organs, and are characterized by a high proliferative ability. They can ulcerate, and then in infants they serve as a source of sepsis. A feature of capillary angiomas is the infiltrative nature of growth, which is associated with frequent relapses after their removal. The tumor never metastasizes.

Cavernous hemangioma- rather a malformation of blood vessels than a true tumor, it is less common in children than capillary. Localized in the liver, skin, gastrointestinal tract and other organs. In infants, congenital giant cavernous angiomas are occasionally observed, occupying up to 2/3 of the liver volume (Fig. 327). Such patients die from bleeding. Hemangiomas can undergo scarring - spontaneous regression.

Relatively rare in children angiosarcoma- a true malignant tumor of blood vessels.

Lymphangioma It is less common in children than hemangioma. In hypertrophic lymphangioma, the endothelium of the lymphatic capillaries proliferates, and cavernous cavities with clutches of lymphoid cells are formed in their walls. The development of cavernous lymphangioma may be accompanied by macrocheilia And macroglossia. Congenital giant lymphangiomas of the neck are observed (hydroma cysticum colli congenitum), which can reach the size of a child's head. The tumor grows into the organs of the neck, leading to asphyxia or becoming secondarily infected. It cannot be removed. The tumor consists of numerous cavities lined with endothelium, between them there is loose mesenchymal tissue poor in cells, proliferation of solid strands of endothelium and capillaries is observed, due to which infiltrating growth occurs. Rarely occurs in children lymphangiosarcoma.

Hamartomas and hamartoblastomas of striated muscle tissue

Rhabdomyoma- benign tumor of embryonic muscle cells. Refers to rare tumors, observed mainly in children. It is localized in the heart and muscles of the limbs in the form of a node, its diameter sometimes reaches 10-15 cm in a brownish section. Multiple congenital cardiac rhabdomyomas should be differentiated from glycogenosis (see. Dystrophy).

Rhabdomyoblastoma, dysontogenetic, embryonal rhabdomyosarcoma,- a malignant tumor from split off embryonic rudiments of muscle tissue (see. Tumors). It consists of large cells with large, often polymorphic nuclei, reminiscent of embryonic myoblasts. Longitudinal and transverse striations are found in the cytoplasm (Fig. 328). Localized in children ectopically in the pelvic organs - urinary

Rice. 328. Embryonic rhabdomyosarcoma

in the bladder, vagina, pelvic tissue, in the gonads, less often in the nasopharynx and other organs. In the area of ​​the bladder and vagina it has the appearance of whitish polypous, grape-shaped growths covered with mucosal epithelium, hence the name of the tumor of this localization - botryoid(from botryoides- grape-shaped) polyp.

This rare malignant tumor occurs primarily in children. Infiltrating the pelvic organs, it causes obstruction of the urinary tract, up to the development of uremia, ulcerates, bleeds, and is subject to secondary infection. Metastasizes to regional lymph nodes and hematogenously to the lungs.

Hamartoblastomas of internal organs

Nephroblastoma, dysontogenetic, embryonal nephroma (Wilms tumor, adenosarcoma),- a malignant kidney tumor that develops from a pluripotent renal anlage - metanephrogenic tissue (Fig. 329). It grows expansively for a long time, within the capsule, squeezing and pushing aside the kidney tissue. Sometimes it reaches gigantic sizes. On the section it is pinkish-white in color, nodular in appearance, with areas of fresh and old hemorrhages. Later, the tumor grows into surrounding tissues and metastasizes to the lungs. It consists of solid fields or strands of cells with an oval or round nucleus, in the center of which tubes are formed, resembling renal tubules or glomeruli. Between the solid fields and tubes there is loose, soft-fibrous tissue with elongated cells. It may contain various derivatives of mesoderm - striated and smooth muscle fibers, fatty tissue, vessels, cartilage, in rare cases - derivatives of the ectoderm in the form of nervous tissue.

With rare exceptions, nephroblastoma occurs in children (mainly from 1 year to 4 years) and is the fourth most common tumor among them.

Hepatoblastoma, dysontogenetic, embryonal hepatoma,- a malignant liver tumor that develops from an embryonic pluripotent anlage. Liver tissue sprouts in the form of numerous whitish-yellow nodes, grows quickly, meta-

Rice. 329. Nephroblastoma (preparation by V.N. Sukhova)

Rice. 330. Hepatoblastoma

stasis in the lymph nodes and hematogenously - in the lungs (Fig. 330). It can produce bile both in the main node and in metastases. It consists of solid fields of embryonic liver cells, among which there are tubes and cysts resembling bile ducts (Fig. 331). The degree of differentiation of the liver tumor tissue varies. Between the solid fields there is loose mesenchymal tissue, sometimes containing many vessels of the sinusoidal type, up to the formation of cavernous cavities. Some tumors may contain derivatives of mesenchyme and mesoderm: adipose tissue, cartilage, bone and sometimes even embryonic striated muscle. Hepatoblastoma was discovered

Varies mainly in children

infancy and early childhood.

Fatal outcome sometimes occurs in a child before the development of metastases

from hemorrhage into the abdominal cavity, since the tumor is rich in blood vessels,

and bleeding sometimes occurs with minor trauma.

Teratomas and teratoblastomas

Organismoid teratomas And organoid- expansively growing tumors, often reaching a large size, consist of a variety of tissue structures - derivatives of all three germ layers with the presence or absence of elements of extraembryonic tissues (trophoblast elements).

Teratomas have a specific, typical localization: ovaries and testicles, sacrococcygeal region, mediastinum, retroperitoneal space, pharynx, base of the skull. In adults, teratomas of the gonads and mediastinum are most common. The most common location of teratomas in children is the sacrococcygeal region.

Sacrococcygeal teratoma, As a rule, it is detected from birth and is also found in fetuses, more often in girls. Most teratomas of this localization are benign, organismoid. They sometimes reach a very large size and then complicate the course of labor. They can fill the pelvic cavity without damaging the pelvic bones. The tumor consists of organ-like structures and various tissues, such as intestinal loops, liver tissue, rudiments of limbs, etc. (Fig. 332). Less common when, along with mature tissues, the growth of carcinoma-like solid or papillary structures predominates. Papillary growths are derivatives extraembryonic tissues (trophoblast).

From ovarian teratomas are more common in children compared to adults malignant teratoblastomas, than benign dermoid cysts. They have character multilocular cysts, consisting

Rice. 332. Sacrococcygeal teratoma in a girl:

a - multilayered squamous epithelium; b - fatty tissue; c - glandular tissue

from mature tissues, between which there are fields of solid growths of an undifferentiated embryonic and extraembryonic nature. Such teratoblastomas can metastasize to the lungs. In children, cases of mature teratomas are observed, consisting of differentiated tissues, which, when surgically removed, however, cause seeding in the peritoneum.

Testicular teratomas are more common in children under 2 years of age and are often detected from birth. Testicular teratomas as opposed to ovarian teratomas in children are more often benign. In adults, on the contrary, they are more often malignant. Malignant teratomas have been described in boys aged 15-16 years: histologically, they often consist of derivatives epithelial tissue- flat keratinizing epithelium, mucous glands, undifferentiated epithelial tissue.

Retroperitoneal and mesenteric teratomas in most cases, they appear from birth or at the age of 2-3 years. They reach a large size and are more common in girls. Located closer to the diaphragm than to the pelvic region, they are usually benign, very rarely malignant.

Large polycystic and solid teratomas with the presence of immature tissue are always malignant.

Teratomas of the throat (congenital polyps of the throat)- polypoid formations found in fetuses and newborns. They are located in the area of ​​the upper dome of the pharynx, from where they grow in the form of polypoid growths, one part of which is covered with mucous membrane, the other with skin. They sometimes reach a significant size and then make childbirth difficult. As a rule, they consist of mature tissues and vestigial organs. Sometimes there are highly differentiated forms corresponding to an inferior second twin, reaching the size of a child's head; the tumor attaches to the jaws or pharynx (the so-called epignatus). Malignant teratomas of this area are rare.

Intracranial teratomas in half of the cases they are malignant and contain embryonic growing tissue. In rare cases, extrapineal teratomas are chorionepitheliomas; they give hematogenous metastases to the lungs and are located in the base of the skull. Described in fetuses and newborns. In boys, they are often localized near the pineal gland and may be accompanied by endocrine disorders in the form of premature puberty.

Teratoblastoma is a malignant analogue of teratoma; it always contains fields of immature actively proliferating embryonic or, more often, extraembryonic tissue, which can be combined with elements of mature tissues. Teratoblastomas grow quickly and metastasize. In metastases, as in the primary node, a combination of mature and immature tissues is found. Less common are teratoblastomas, consisting only of immature, usually extraembryonic tissues.

Tumors from cambial embryonic tissues

Medulloblastoma- a malignant tumor of neuroectodermal embryonic stem cells - medulloblasts. Consists of oval or round cells with scanty, almost indistinguishable cytoplasm. Cells fold into sockets (form ring-shaped structures), in the center of which cellular processes are found. Education is typical rhythmic structures, having the form of cell rows or columns (Fig. 333). It is believed that during ontogenesis, medulloblasts differentiate into neuroblasts and spongioblasts. The tumor occurs mainly in children and is localized in the cerebellum in the midline - along the line of closure of the medullary tube. It has a soft consistency, grayish-pink color, and grows into the brain tissue and pia mater. Metastasizes along the cerebrospinal fluid pathways within the central nervous system. Very rarely gives hematogenous metastases to the lungs.

Retinoblastoma- a malignant tumor of embryonic undifferentiated cells of the retina. Some believe that medulloblasts are also the source of development. Tumor masses are grayish-yellow in color, brain-shaped, soft in consistency. The tumor consists of round and oval cells, forms sockets, prone to necrosis, often has the appearance of couplings located around the vessels. Foci of necrosis easily undergo calcification. Retinoblastomas are more common in children under the age of 2 years, are bilateral, sometimes occur spontaneously, and sometimes are hereditary (inherited in a dominant manner). Isolated cases of spontaneous recovery have been described (reversions). The tumor grows into surrounding tissues, leads to protrusion of the eye, disfigures the face, and grows into the base of the skull. Metastasizes to the bones, liver, and less commonly to the lungs and lymph nodes.

Neuroblastoma- a malignant tumor of stem cells of the sympathetic ganglia and adrenal medulla. Recently, a point of view has emerged that neuroblastomas are histogenetically classified as apudomam, because they highlight catecholamines. Localized in the area of ​​the adrenal glands, sympathetic nodes neck or chest area

Rice. 333. Medulloblastoma in a girl

3 years 2 months

sti, retroperitoneal space. May occur multicentrically - in both adrenal glands, adrenal gland and ganglia chest cavity etc. It looks like a node in a thin capsule that destroys the adrenal gland, on a section it is pinkish-white in color, with numerous necrosis and hemorrhages. Histologically, the tumor consists of round lymphocyte-like cells with a hyperchromic nucleus and barely visible cytoplasm - sympathogonia, hence the old name for this undifferentiated type of tumor - sympathogonoma. Cells form sockets (Fig. 334), in the center of which, when impregnated with silver, nerve processes are revealed. The tumor contains extensive areas of necrosis with karyorrhexis and hemorrhage. In more differentiated tumors - sympathoblastomas the cells are larger, their cytoplasm is wider, the nuclei are lighter, giant cells are found, the neurofibrillary network is more clearly expressed. An even more differentiated type of tumor - ganglioneuroblastoma, characterized by the presence of atypical ganglion cells. Cases of transition of undifferentiated neuroblastoma to mature benign ganglioneuroma And ganlioneurofibromas(Fig. 335), both spontaneously and under the influence of treatment.

Neuroblastoma grows quickly and metastasizes widely. There are two types of metastases: 1) to regional lymph nodes and liver; 2) in the bones of the skeleton - ribs, spine, pelvic bones and skull. Metastases to the skin are sometimes observed. The clinical course in children may be accompanied by increased blood pressure, sweating due to the secretion of catecholamines by tumor cells, which can be

found in blood and urine. Neuroblastoma most often occurs in children under 1 year of age, but can occur up to 11 years of age. Cases of neuroblastoma in fetuses and newborns have been described; in adults it is described as casuistry.

Tumors in children that develop like tumors in adults

The predominant tumors in children are central nervous system tumors- astrocytomas and tumors of hematopoietic tissue(leukemia, malignant lymphoma). Among benign soft tissue tumors, it should be noted juvenile angiofibroma of the nasopharynx. Occurs between the ages of 8-18 years. It has a dense consistency and the appearance of polypous growths, covered with a mucous membrane, is localized in the nasopharynx, grows very quickly, fills the nasal cavity, grows into the bones of the facial skeleton and the base of the skull. It often ulcerates, bleeds, and becomes infected. Microscopically, it is a fibroma with the presence of juicy fibroblasts and a large number of thin-walled vessels (Fig. 336). It is difficult to radically remove the tumor; after surgery it often recurs. Sometimes after puberty it undergoes reversions.

Although the structure of the tumor is benign and no metastases are observed, the clinical course and prognosis are unfavorable. Common tumors in children are bone tumors: benign - And osteomas chondromas, osteoblastoclastomas and malignant - osteosarcomas, Ewing's sarcomas. Benign chondromas, or so-called cartilaginous exostoses,