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• Sacrococcygeal region - 42
• Mediastinum - 7
• Retroperitoneal space - 4
• Testicle - 9
• Ovary - 24
• Pineal gland area - 6
• Other areas - 6

In this article, only extracranial germ cell tumors are considered.

Histogenesis of germ cell tumors

Germ cell tumors develop from pluripotent germ cells. They originate in the endoderm of the yolk sac and normally migrate from there along the hindgut towards the urogenital crest on the posterior abdominal wall, where they become part of the developing gonads. Depending on the place of stopping on the way of migration, embryonic germ cells can give rise to tumor growth in one or another area along the midline of the body. Therefore, germ cell tumors are found in various parts of the body, they can have gonadal and extragonadal localizations.

Due to the fact that in the process of embryogenesis germ cells in the caudal part of the urogenital crest persist for a longer time than in the head, teratomas and teratoblastomas are more common in the pelvic region, sacrococcygeal region, retroperitoneal space than in the mediastinum, in the neck and intracranial region.

Germ cell tumors originate from a pluripotent germ cell and therefore may consist of derivatives of all three germ layers. As a result, they may contain tissues that are not typical for the anatomical zone in which the neoplasm occurs.

The type of developed tumor depends on the migration route and the degree of maturity of the ectopic cells.

Histological classification

Histologically, germ cell tumors are divided into germinomas and non-germinal cell tumors. The latter include teratomas, neoplasms of the yolk sac, embryonic cancer, choriocarcinoma, mixed germ cell tumors.

• Germinomas are germ cell tumors that occur in extragonadal areas (pineal region, anterior mediastinum, retroperitoneal space). A neoplasm, histologically identical to a germinoma, but developing in the testis, is called seminoma, in the ovaries - dysgerminoma.

Germinogenic tumors are divided into secreting (alpha-fetoprotein, beta-chorionic gonadotropin) and non-secreting.

• Teratomas are embryonic tumors containing tissues of all three germ layers: ectoderm, endoderm and mesoderm. They occur in the sacrococcygeal region, mediastinum, ovaries and are divided into mature teratomas (benign variant), immature teratomas (intermediate variant) and malignant tumors - teratoblastomas. According to the structure, teratomas are divided into cystic and solid.
• Neoplasms of the yolk sac (endodermal sinus) - extragonadal germ cell tumors that occur in young children in the sacrococcygeal region, in older children - in the ovaries. For localization in the testicles, two age faces are characteristic - in younger children and in adolescents. There may be foci of a yolk sac tumor in teratoblastomas. Yolk sac tumors are classified as highly malignant.
• Embryonic cancer (embryonic carcinoma) can be found both in its pure form and as a component of teratoblastoma. Localized in the testicles and ovaries. Occurs more frequently in adolescence.

How do germ cell tumors manifest?

Germinogenic tumors manifest themselves in different ways. Their symptoms depend on the location of the neoplasm.

• Sacro-lumbar region - Deformation and enlargement of this region due to neoplasm.
• Mediastinum - Respiratory disorders when the tumor reaches a large size.
• Retroperitoneal space - Symptoms characteristic of this localization.
• Testicle - Enlargement of the testicle due to a dense tuberous formation.
• Ovary - Palpable tumor of the abdominal cavity and small pelvis, with torsion of the pedicle of the tumor - pain in the abdomen.
• Area of ​​the pineal gland - Focal and cerebral symptoms.

Sacrococcygeal teratomas are usually detected at birth and diagnosed without much difficulty. The manifestation of germ cell tumors of the testicles has two peak incidence: up to 4 years (most cases) and in the period older than 14-15 years. At the same time, biology in younger childhood and adolescence is different: in the younger age group, neoplasms of the yolk sac and mature teratomas are found, while in adolescents - teratoblastoma and seminomas. In contrast to the well visualized localization in the testis, other extracranial germ cell tumors (mediastinal, abdominal, small pelvis) in children appear, as a rule, at stage III-IV of the process. The manifestation of ovarian dysgerminoma occurs in the prepubertal and pubertal periods (8-12 years). Germ cell tumors of the mediastinum are detected in early childhood and in adolescents. At the same time, at the age of 6 months to 4 years, they are represented by teratoblastomas, tumors of the yolk sac, embryonic cancer. In adolescence, the germinoma type predominates among germ cell tumors of the mediastinum.

Symptoms of a metastatic lesion depend on the location and degree of development of the metastatic process and do not have specific signs compared to other malignant neoplasms. A tumor symptom complex can develop with teratoblastoma in the case of massive decaying neoplasms.

Classification (clinical staging)

The POG/CCSG study group uses separate postoperative staging systems for testicular, ovarian, and extragonadal germ cell neoplasms.

I. Germinogenic tumors of the testis.

• Stage I - the neoplasm is limited to the testicle, removed completely as a result of a high inguinal or transscrotal orchiofuniculectomy. There are no clinical, radiological and histological signs of the spread of the neoplasm outside the organ. The content of tumor markers studied taking into account the half-life (alpha-fetoprotein - 5 days, beta-hCG - 16 hours) was not increased. In patients with normal or unknown initial values ​​of tumor markers, the retroperitoneal lymph nodes are not affected.
• Stage II - performed transscrotal orchiectomy. Microscopically, the presence of a neoplasm in the scrotum or high in the spermatic cord (less than 5 cm from its proximal end) is determined. The retroperitoneal lymph nodes are affected by a tumor (less than 2 cm in size) and / or elevated levels of tumor markers (taking into account the half-life).
• Stage III - defeat of the neoplasm of the retroperitoneal lymph nodes (sizes more than 2 cm), but there is no tumor damage to the abdominal organs and the spread of the tumor outside the abdominal cavity.

II. Germinogenic tumors of the ovaries.

• Stage I - the tumor is limited to the ovary (ovaries), lavage water from the peritoneum does not contain malignant cells. There are no clinical, radiological or histological signs of the spread of the neoplasm beyond the ovaries (the presence of peritoneal gliomatosis is not considered a basis for changing stage I to a higher one). The content of tumor markers is not increased taking into account their half-life.
• Stage II - a tumor lesion of the lymph nodes is microscopically determined (dimensions less than 2 cm), lavage water from the peritoneum does not contain malignant cells (the presence of peritoneal gliomatosis is not considered a basis for changing stage II to a higher one). The content of neoplasm markers is not increased taking into account their half-life.
• Stage III - lymph nodes are affected by a tumor (sizes more than 2 cm). After the operation, a massive tumor remained or only a biopsy was performed. Tumor lesion of adjacent organs (for example, omentum, intestines, bladder), lavage water from the peritoneum contains malignant cells. The content of neoplasm markers can be normal or elevated.
• Stage IV - distant metastases, including the liver.

III. Extragonadal germ cell tumors.

• Stage I - complete removal of the neoplasm in any of its localization, with localization in the sacrococcygeal region, the coccyx was removed, histologically resection within healthy tissues. The content of tumor markers is normal or elevated (but decreases taking into account their half-life). Regional lymph nodes are not affected.
• Stage II - microscopically determine malignant cells along the resection line, the lymph nodes are not affected, the content of tumor markers is normal or increased.
• Stage III - after the operation, a massive neoplasm remained or only a biopsy was performed. Retroperitoneal lymph nodes may or may not be affected by the tumor. The content of tumor markers is normal or elevated.
• Stage IV - distant metastases, including the liver.

How are germ cell tumors recognized?

Diagnosis of the primary focus in germ cell tumors includes ultrasound, radiography. CT and/or MRI. ultrasonic doppler angioscanning. Diagnosis of possible metastases includes chest x-ray. Ultrasound of the abdominal cavity and regional zones, myelogram examination. Excretion of catecholamines and their metabolites should be investigated to exclude neoplasms of a neurogenic nature in the localization of neoplasms in the mediastinum, retroperitoneal space, presacral region.

Germinogenic tumors of the sacrococcygeal region require the identification (if any) of the presacral component of the neoplasm. This requires a rectal examination and a careful assessment of ultrasound and CT or MRI data.

Germinogenic tumors differ in that it is possible, before obtaining a histological conclusion, to assess the degree of malignancy using the Abeleva-Tatarin reaction - a study of the concentration of alpha-fetoprotein protein in the blood serum. This protein is normally synthesized by the cells of the yolk sac, liver and (in a small amount) the gastrointestinal tract of the fetus. The biological role of alpha-fetoprotein is that, penetrating the placenta into the blood of a pregnant woman, it inhibits the immunological reaction of rejection of the fetus by the mother's body. Protein alpha-fetoprotein begins to be synthesized in the early stages of intrauterine development. Its maximum content becomes at a gestational age of 12-14 years above, dropping to the level of an adult by the age of 6-12 months of postnatal life. Malignant germ cell tumors are capable of synthesizing a-fetoprotein, therefore, the study of the Abelev-Tartarinov reaction makes it possible to assess the degree of malignancy of the neoplasm. At the age of a child under 3 years of age serious condition which makes any surgical intervention undesirable, even in the amount of a biopsy, a high titer of alpha-fetoprotein can serve as a basis for starting antitumor treatment without morphological verification of the diagnosis. When determining the dynamics of the content of alpha-fetoproten in the blood serum, one should take into account the half-life of this protein and the dependence of this indicator on age.

Other oncomarkers, cancer embryonic antigen (CEA), also play an important role in the diagnosis of teratoblastoma and other germ cell tumors. Beta-human chorionic gonadotropin (beta-hCG) and placental alkaline phosphate. An increase in the latter indicator is associated with the presence of neoplasms of syncytiotrophoblasts in the tissue. The half-life of beta-hCG is 16 hours (in children under one year old - 24-36 hours).

In a lesser part of cases, teratoblastoma may occur without an increase in the content of alpha-fetoprotein and other tumor markers. On the other hand, an increase in the content of alpha-fetoprotein does not necessarily indicate the presence of a germ cell tumor. This indicator also increases with malignant neoplasms of the liver.

Mandatory and additional studies in patients with suspected germ cell tumors

Mandatory diagnostic tests

• Complete physical examination with assessment of local status
• Clinical blood test
• Clinical analysis of urine
• Biochemical blood test (electrolytes, total protein, liver tests, creatinine, urea, lactate dehydrogenase, alkaline phosphatase, phosphorus-calcium metabolism)
• Coagulogram
• Ultrasound of the affected area
• Ultrasound of the abdominal cavity and retroperitoneal space
• CT (MRI) of the affected area
• X-ray of the chest cavity in five projections (direct, two lateral, two oblique)
• Study of tumor markers
• Excretion study of catecholamines
• Bone marrow puncture from two points
• echocardiography
• Audiogram
• In children over 3 years of age and with normal and questionable alpha-fetoprotein or beta-hCG values
• The final stage is a biopsy of the neoplasm (or complete removal) to verify the cytological diagnosis. It is advisable to make prints from a biopsy for cytological examination

Additional diagnostic tests

• If metastases to the lungs are suspected - CT scan of the chest cavity
• If metastases are suspected in the brain - EchoEG and CT scan of the brain
• Ultrasound color duplex angioscanning of the affected area

How are germ cell tumors treated?

Treatment of benign germ cell tumors - surgical, malignant - combined and complex. Apply radiation therapy and course chemotherapy using platinum drugs, ifosfamide, etoposide. With dysgerminomas, the appointment of chemoradiotherapy is performed initially for unresectable neoplasms and after surgery - at II-IV postoperative stages. For other histological variants of malignant germ cell tumors (eg, yolk sac tumor, choriocarcinoma, fetal cancer), treatment for all stages consists of surgery and postoperative chemotherapy.

If a resectable neoplasm is detected, the first stage of treatment is a radical operation. If the primary tumor is unresectable, a biopsy should be limited. Radical surgery is performed after neoadjuvant chemotherapy and the tumor acquires signs of resectability against its background. In cases of detection of a neoplasm in children under 3 years of age and the undesirability of surgery even in the amount of a biopsy due to the severity of the patient's condition, a high titer of alpha-fetoprotein or B-hCG serves as a basis for refusing a diagnostic operation and starting chemotherapy without morphological confirmation of the diagnosis.

Congenital teratoid tumor of the sacrococcygeal region should be removed as early as possible. It must be borne in mind that this neoplasm can have two components: sacrococcygeal, removed from the perineal access, and presacral, removed from the laparotomic access. Thus, in such cases, surgery from a combined abdominoperineal approach is necessary. An undetected and unremoved presacral component becomes a source of recurrent growth, while in the case of an initially benign variant of the neoplasm, its malignancy with the development of a malignant recurrence is possible. Before starting the operation, in order to avoid injury to the rectum, a tube is inserted into it to control its position. It is imperative to resect the coccyx, and in case of widespread lesions, the sacrum. During the operation, the variant of the tumor (cystic, solid) should be taken into account. In the first case, opening of cystic cavities should be avoided.

Upon receipt after removal of the sacrococcygeal tumor, morphological data on the benign nature of the process, the tumor is regarded as a mature teratoma, and the treatment is completed. The picture of malignancy in histological preparations becomes the basis for the diagnosis of teratoblastoma. requiring chemoradiotherapy. In immature teratomas after surgery, patients are left under observation, chemotherapy is carried out only in the diagnosis of recurrence of the neoplasm.

Ovarian germ cell tumors, like other neoplasms of the retroperitoneal space, are removed from the laparotomy access. A salpingo-oophorectomy with a tumor is performed. With a unilateral ovarian lesion, along with its removal, a biopsy of the opposite ovary should be performed. Also, when removing an ovarian tumor, it is necessary to resect the greater omentum (the latter, due to the mechanism of contact metastasis, can be affected by metastases) and perform a biopsy of the retroperitoneal lymph nodes. The presence of ascitic fluid is an indication for its cytological examination. Bilateral tumor lesion is an indication for removal of both ovaries.

A feature of ovarian teratomas is the possibility of seeding the peritoneum with tumor cells (the so-called peritoneal gliomatosis). Gliomatosis of the peritoneum is possible in the form of a microscopic or macroscopic lesion. In cases of detection of peritoneal gliomatosis, it is advisable to prescribe postoperative chemotherapy.

Germinogenic tumors of the mediastinum

If the neoplasm is localized in the mediastinum, a thoracotomy is performed. In some cases, with variants of localization, a sternotomy is possible.

Testicular germ cell tumors

With a tumor lesion of the testicle, an orchiofuniculectomy is performed from the inguinal access with a high ligation of the spermatic cord. Removal or biopsy of the retroperitoneal lymph nodes is performed (from laparotomy access) as a second-look operation after program chemotherapy according to indications.

If the pulmonary metastases that were present before the start of treatment persist on radiographs and computed tomograms and are recognized as resectable. they need to be surgically removed.

What is the prognosis for germ cell tumors?

Malignant extracranial germ cell tumors before the use of effective chemotherapy had an extremely unfavorable prognosis. With chemotherapy, a 5-year survival rate of 60-90% has been achieved. The prognosis depends on the histological variant, age, localization and prevalence of the neoplasm, as well as on the initial level of tumor markers. With teratomas of the sacrococcygeal region, the prognosis is better in patients up to 2 months. With mediastinal teratomas, the prognosis is better in patients under 15 years of age. Favorable histological germ cell tumors (terminomas, teratomas without tumor tissue foci of unfavorable histological variants) compared to unfavorable ones (embryonic carcinoma, yolk sac tumor, choriocarcinoma) have a better prognosis. The prognosis is worse with higher levels of tumor markers before treatment compared with patients with lower levels.

Non-germinogenic tumors of the gonads

Non-germinogenic tumors of the gonads in childhood are rare, however, they are found in children. With this type of pathology, differential diagnosis with such neoplasms as germ cell tumors, as well as appropriate treatment, is necessary.

Sertolioma (sustenocytoma, androblastoma) is usually benign. It occurs at any age but is more common in infants. Clinically, sertolioma is manifested by a tumor formation of the testicle. The neoplasm consists of sustenocytes forming tubular structures.

Leydigoma (interstitial cell tumor) originates from glandulocytes. usually benign. It occurs in boys aged 4 to 9 years. As a result of hypersecretion of testosterone and some other hormones in sick boys, premature sexual development begins. Histologically, the neoplasm is indistinguishable from ectopic tissue of the adrenal cortex. In both cases, an inguinal orchiofuniculectomy is performed (as an option, an orchiectomy from the scrotal access).

Benign ovarian cyst accounts for 50% of all ovarian tumors. Cysts can be detected by accidental ultrasound. as well as laparotomy. performed for "acute abdomen" with torsion or torsion of the cyst. Such patients are required to study tumor markers before and after surgery.

Other ovarian tumors are extremely rare. Granulosa cell tumors (thecomas) are benign neoplasms of stromal origin. The tumor is manifested by premature sexual development. Cystadenocarcinoma is distinguishable from other tumors only histologically. In isolated cases, the primary manifestation of non-Hodgkin's malignant ovarian lymphoma has been described.

Gonadoblastomas are detected in patients with gonadal dysgenesis (true hermaphroditism). 80% of patients have a female phenotype with signs of virilization. The remaining 25% of patients have a male phenotype with signs of cryptorchidism, hypospadias and / or the presence of internal female genital organs (uterus, fallopian tubes or their vestiges). Histological examination reveals a combination of germ cells and elements of immature granulosa, Sertoli or Leydig cells. These neoplasms must be removed surgically along with stroke gonads due to the high risk of malignancy of the latter. To establish the true gender of the patient, a cytogenetic study of the karyotype is performed.

It is important to know!

Germ cell tumors originate from pluripotent germ cells. Violation of the differentiation of these cells leads to the emergence of embryonic carcinoma and teratoma (embryonic line of differentiation) or choriocarcinoma and tumor of the yolk sac (extraembryonic differentiation pathway).

Germ cell tumors are typical neoplasms of childhood. Their source is the primary sex cell, i.e. these tumors are malformations of the primary germ cell. During the development of the embryo, germ cells migrate to the genital ridge, and if this process is disturbed, the germ cells can be delayed at any stage of their journey, and in the future there is a chance of tumor formation.

Tumors of this type account for up to 7% of all tumors in children and adolescents. 2-4% - in children under 15 years old and about 14% in adolescents from 15 to 19 years old. The probability of falling ill in adolescent boys under 20 is slightly higher than in girls - 12 cases versus 11.1 per million. According to some reports, the pathological course of pregnancy and smoking in the mother increase the risk of germ cell tumors in the child.

Germinogenic tumors are divided into gonadal, which develop inside the gonads, and extragonadal. There are two peaks in the incidence of germ cell tumors: the first - up to 2 years of tumors of the sacrococcygeal region (74% are girls) and the second - 8-12 years for girls and 11-14 years for boys with lesions of the gonads.

The most common symptoms of the disease are an increase in the size of the affected organ and pain. There may be complaints of difficulty urinating, intestinal obstruction, the appearance of clinical signs of compression of the mediastinal organs or CNS damage.

The most common localizations of germ cell tumors:

• cross-coccygeal region;
• ovary;
• testicle;
• epiphysis;
• retroperitoneal space;
• mediastinum.

Tumors are extremely diverse in their morphological structure, clinical course and prognosis, they can be both benign and malignant.

Morphological classification of germ cell tumors:

• Dysgerminoma (seminoma);
• Teratoma mature and immature;
• Tumor of the yolk sac;
• Choriocarcinoma;
• Embryonic cancer;
• germinoma;
• Mixed germ cell tumor.

Diagnostics

If a child develops symptoms, we recommend a comprehensive diagnosis at the Oncology Research Institute. Depending on the indications, the doctor may prescribe the following tests and studies:

• laboratory tests: complete blood count, general urinalysis, biochemical blood test, AFP, coagulogram;
• instrumental studies: chest x-ray, abdominal ultrasound, ultrasound of the affected area, CT of the chest and abdomen, MRI of the affected area, osteoscintigraphy, myeloscintigraphy;
• invasive examinations: puncture, bone marrow trepanbiopsy, lumbar puncture (according to indications); tumor biopsy.

Treatment

Treatment of children with germ cell tumors is to remove the tumor and conduct chemotherapy. The sequence of surgery and chemotherapy depends on the location of the tumor. As a rule, the defeat of the gonads dictates the removal of the tumor at the first stage with chemotherapy in the postoperative period. If a CT or MRI scan shows clear infiltration into the surrounding tissue or metastases, the first therapeutic step is chemotherapy.

Most extragonadal germ cell tumors are of considerable size, and their removal is accompanied by an increased risk of opening the tumor capsule. In these cases, patients are given chemotherapy to reduce the risk of tumor recurrence. Radiation therapy is rarely used and has limited indications.

Ideally, the goals of treatment are to achieve recovery and maintain menstrual and reproductive function in patients.

Forecast

Overall survival for germ cell tumors is:

• at stage I 95%
• at stage II - 80%
• at stage III - 70%
• at IV - 55%.

The prognosis for patients with germ cell tumors is affected by the histological structure, the level of tumor markers, and the prevalence of the process. Unfavorable factors are late diagnosis, large tumor size, tumor rupture, chemoresistance, and relapse of the disease.

Chapter 14

Germ cell tumors develop from a population of pluripotent germ cells. The first germ cells can be found in the endoderm of the yolk sac as early as a 4-week-old embryo. During embryonic development, the original germ cells migrate from the endoderm of the yolk sac to the genital ridge in the retroperitoneum (Figure 14-1). Here, the sex glands develop from the germ cells, which then descend into the scrotum, forming the testicles, or into the small pelvis, forming the ovaries. If during the period of this migration, for some unknown reason, a violation of the normal migration process occurs, the germ cells can linger in any place of their route, where a tumor can subsequently form. Germ cells can most often be found in areas such as the retroperitoneum, mediastinum, pineal region (pineal gland), and sacrococcygeal region. Less often germ cells linger in the area of ​​the vagina, bladder, liver, nasopharynx.

Epidemiology

Germ cell tumors are an uncommon type of neoplastic lesion in children. They make up 3-8% of all malignant tumors in childhood and adolescence. Since these tumors can also be benign, their frequency is probably much higher. These tumors are two to three times more common among girls than boys. Mortality among girls is three times higher than among boys. After 14 years, mortality among males becomes higher, due to an increase in the incidence of testicular tumors in adolescent boys.

Histogenesis

Malignant germ cell tumors are very often associated with various genetic abnormalities, such as ataxia-telangiectasia, Klinefelter's syndrome, etc. These tumors are often combined with other malignant tumors, such as neuroblastoma and hemoblastoses. Undescended testicles pose a risk for the development of testicular tumors.

Patients with germ cell tumors most often have a normal karyotype, but a breakdown in chromosome I is often detected. The genome of the short arm of the first chromosome may be duplicated or lost. Multiple examples of germ cell tumors have been noted in siblings, twins, mothers and daughters.

Differentiation along the embryonic line gives the development of teratomas of varying degrees of maturity. Malignant extraembryonic differentiation leads to the development of choriocarcinomas and yolk sac tumors.

Often, germ cell tumors may contain cells of different lineages of germ cell differentiation. Thus, teratomas may have a population of yolk sac cells or trophoblasts.

The frequency of each histological type of tumor varies with age. Benign or immature teratomas are more common at birth, yolk sac tumors between 1 and 5 years of age, dysgerminomas and malignant teratomas are most common in adolescence, and seminomas are more common after 16 years of age.

Factors causing malignant changes are unknown. Chronic diseases, long-term drug treatment during pregnancy of the mother may be associated with an increase in the incidence of germ cell tumors in children.

The morphological picture of germ cell tumors is very diverse. Germinomas consist of groups of large neoplastic cells of the same type with a swollen nucleus and light cytoplasm. Tumors of the yolk sac have a very characteristic picture: a mesh stroma, often called a lacy one, in which rosettes of cells containing a-fetoprotein in the cytoplasm are located. Trophoblastic tumors produce human chorionic gonadotropin. Benign, well-differentiated teratomas often have a cystic structure and contain various tissue components, such as bone, cartilage, hair, and glandular structures.

The pathological report for germ cell tumors should include:
-localization of the tumor (organ affiliation);
- histological structure;
- state of the tumor capsule (its integrity);
-characteristics of lymphatic and vascular invasion;
-spread of the tumor to surrounding tissues;
-immunohistochemical study for AFP and HCG.

There is a correlation between the histological structure and localization of the primary tumor: tumors of the yolk sac mainly affect the sacrococcygeal region and gonads, and in children under two years of age, tumors of the coccyx and testicles are more often recorded, while in older children (6-14 years old) tumors of the ovaries and pineal region.

Choriocarcinomas are rare but extremely malignant tumors that most commonly occur in the mediastinum and gonads. They may also be congenital.

For dysgerminomas, the typical localization is the pineal region and the ovaries. Dysgerminomas account for approximately 20% of all ovarian tumors in girls and 60% of all intracranial germ cell tumors.

Embryonic carcinoma in its "pure form" is rare in childhood, most often a combination of elements of embryonic cancer with other types of germ cell tumors, such as teratoma and tumor of the yolk sac, is recorded.

Clinical picture

The clinical picture of germ cell tumors is extremely diverse and, first of all, is determined by the localization of the lesion. The most common locations are the brain (15%), ovaries (26%), coccyx (27%), testicles (18%). Much less often, these tumors are diagnosed in the retroperitoneal space, mediastinum, vagina, bladder, stomach, liver, neck (nasopharynx) (Table 14-1).

Testicle.
Primary testicular tumors are rare in childhood. Most often they occur before the age of two years and 25% of them are diagnosed already at birth. According to the histological structure, these are most often either benign teratomas or tumors of the yolk sac. The second peak in the diagnosis of testicular tumors is the pubertal period, when the frequency of malignant teratomas increases. Seminomas in children are extremely rare. Painless, rapidly increasing testicular swelling is most often noticed by the child's parents. 10% of testicular tumors are associated with hydrocele and other congenital anomalies, especially of the urinary tract. On examination, a dense, tuberous tumor is found, there are no signs of inflammation. An increase in the level of alpha-fetoprotein before surgery confirms the diagnosis of a tumor containing elements of the yolk sac. Pain in the lumbar region may be symptoms of metastatic lesions of the para-aortic lymph nodes.

Ovaries.
Ovarian tumors often present with abdominal pain. On examination, one can detect tumor masses located in the small pelvis, and often in the abdominal cavity, an increase in the volume of the abdomen due to ascites. These girls often develop a fever (Figure 14-3).

Dysgerminoma is the most common ovarian germ cell tumor, which is mainly diagnosed in the second decade of life, and rarely in young girls. The disease quickly spreads to the second ovary and peritoneum. Yolk sac tumors are also more common in puberty girls. Tumors are usually unilateral, large in size, therefore, rupture of the tumor capsule is a frequent occurrence. Clinical manifestations of malignant teratomas (teratocarcinomas, embryonic carcinomas) usually have a non-specific picture with the presence of tumor masses in the small pelvis, menstrual irregularities may be observed. Patients in the prepubertal period may develop a state of pseudopuberty (early puberty). Benign teratomas - usually cystic, can be detected at any age, often give a clinic of ovarian torsion, followed by rupture of the ovarian cyst and the development of diffuse granulomatous peritonitis.

Vagina.
These are almost always tumors of the yolk sac, all described cases occurred before the age of two years. These tumors usually present with vaginal bleeding or spotting. The tumor originates from the lateral or posterior walls of the vagina and looks like polypoid masses, often pedunculated.

Sacrococcygeal region.
This is the third most common localization of germ cell tumors. The frequency of these tumors is 1:40,000 newborns. In 75% of cases, the tumor is diagnosed before two months and almost always it is a mature benign teratoma. Clinically, in such patients, tumor formations are detected in the perineum or buttocks. These are most often very large tumors (Fig. 14-4). In some cases, neoplasms have intra-abdominal distribution and are diagnosed at an older age. In these cases, the histological picture most often has a more malignant character, often with elements of a yolk sac tumor. Progressive malignant tumors of the sacrococcygeal region often lead to dysuric phenomena, there are problems with the act of defecation and urination, neurological symptoms.

Mediastinum.
Germ cell tumors of the mediastinum in most cases represent a tumor of large size, but the syndrome of compression of the superior vena cava occurs rarely. The histological picture of the tumor is predominantly of mixed origin and has a teratoid component and tumor cells characteristic of a yolk sac tumor. Brain.
Germinogenic brain tumors account for approximately 2-4% of intracranial neoplasms. In 75% of cases, they are observed in boys, with the exception of the area of ​​the Turkish saddle, where tumors are favorably localized in girls. Germinomas form large infiltrating tumors, which are often the source of ventricular and subarachnoid cerebrospinal metastases. (See the chapter "Tumors of the CNS"). Diabetes insipidus may precede other symptoms of the tumor.

Diagnostics

The initial examination reveals the location of the primary tumor, the extent of the tumor process and the presence of distant metastases.

Chest X-ray is an obligatory method of investigation, which makes it possible to establish a diagnosis in the case of a primary lesion of the mediastinum, and is also indicated for the detection of metastatic lesion of the lungs, which is very common.

Currently, CT has practically become the leading diagnostic method for any tumor localization. Germ cell tumors are no exception. CT is extremely helpful in the differential diagnosis of mediastinal lymphomas. This is the most sensitive method for detecting lung metastases, especially micrometastases. CT is indicated when ovarian lesions are detected. When the ovaries are involved, CT clearly demonstrates the lesion of the ovary itself, and also reveals the spread of the process to the surrounding tissues. For sacrococcygeal tumors, CT helps to determine the spread of the process to the soft tissues of the small pelvis, reveals damage to bone structures, although the traditional x-ray examination of the sacrum and coccyx is also very useful and more convenient for monitoring observation. X-ray examination with the introduction of a contrast agent is very often necessary to determine the position of the bladder, ureters, rectum in relation to the tumor.

CT and MRI of the brain are needed to detect a germ cell tumor of the pineal gland.

Ultrasound is a very useful imaging modality for quick and easy diagnosis of a primary lesion and for monitoring the effect of treatment. Ultrasound is a more convenient method, since CT often requires anesthesia for the study.
tumor markers.

Germ cell tumors, especially those of extraembryonic origin, produce markers that can be detected by radioimmunoassay and are commonly used in monitoring to judge response to treatment.

Tumors with a trophoblastic component can produce HCG, neoplasms with elements of the yolk sac are derivatives of AFP. The largest amount of AFP is synthesized in the early fetal period of life and the highest level of AFP is determined at 12-14 weeks of the fetal period. The content of AFP falls by birth, but its synthesis continues during the first year of life, progressively falling by 6-12 months. life. Blood levels of AFP and HCG should be determined prior to surgery and chemotherapy. After treatment (surgery and CT), in the case of complete removal of the tumor or regression of the tumor after chemotherapy, their level drops, and by half after 24-36 hours for HCG and after 6-9 days for AFP. An insufficiently rapid drop in indicators is a sign of the activity of the tumor process or the insensitivity of the tumor to the therapy. Determination of glycoproteins in the cerebrospinal fluid may be useful for the diagnosis of patients with a CNS tumor.

Staging.

Staging of germ cell tumors presents significant difficulties due to the wide variety of tumor localizations. Currently, there is no single stage classification of germ cell tumors.

It should be noted that for intracranial germ cell tumors, great importance two signs: the size of the primary tumor and the involvement of central structures. For all other localizations, the most important prognostic factor is the volume of the tumor lesion. This feature is the basis of the most commonly used stage classification at present (Table 14-2).

Treatment.

Operative method of treatment.

If a germ cell tumor is suspected in the abdominal cavity or in the small pelvis, surgery can be performed to remove the tumor or (in the case of a large tumor) to obtain morphological confirmation of the diagnosis. However, surgical intervention is often used for urgent indications, for example, in case of torsion of the cyst stem or rupture of the tumor capsule.

If you suspect an ovarian tumor, you should not be limited to the classic transverse gynecological incision. A median laparotomy is recommended. When opening the abdominal cavity, the lymph nodes of the small pelvis and retroperitoneal region are examined, the surface of the liver, subdiaphragmatic space, greater omentum and stomach are examined.

In the presence of ascites, a cytological examination of ascitic fluid is necessary. In the absence of ascites, the abdominal cavity and pelvic area should be washed and the resulting lavage should be subjected to cytological examination.

If an ovarian tumor is detected, the tumor should be subjected to urgent histological examination, removal of the ovary only after confirmation of the malignant nature of the tumor. This practice avoids the removal of unaffected organs. If there is a massive tumor lesion, non-radical operations should be avoided. In such cases, a preoperative course of chemotherapy is recommended, followed by a "second look" operation. If the tumor is localized in one ovary, removal of one ovary may be sufficient. If the second ovary is affected, if possible, part of the ovary should be preserved.

Recommendations when using the surgical method for ovarian lesions:
1. Do not use a transverse gynecological incision.
2. Median laparotomy.
3. In the presence of ascites, a cytological examination is mandatory.
4. In the absence of ascites - rinse the abdominal cavity and pelvic area; cytological examination of washing waters.
5. Examination and, if necessary, biopsy:
- lymph nodes of the small pelvis and retroperitoneal region;
- surface of the liver, subphrenic space, greater omentum, stomach.

Sacrococcygeal teratomas, most often diagnosed immediately after the birth of a child, should be removed immediately to avoid malignancy of the tumor. The operation must include the complete removal of the coccyx. This reduces the likelihood of recurrence of the disease. Malignant sacrococcygeal tumors should be treated first with chemotherapy, followed by surgery to remove the residual tumor.

Surgical intervention for the purpose of biopsy in case of a local tumor in the mediastinum and persistence of AFP is not always justified, as it is associated with risk. Therefore, it is recommended to perform preoperative chemotherapy and, after reducing the size of the tumor, surgical removal of it.

If the testicle is affected, orchiectomy and high ligation of the spermatic cord are indicated. Retroperitoneal lymphadenectomy is performed only when indicated.

Radiation therapy

Medical therapy has very limited use in the treatment of germ cell tumors. It may be effective in the treatment of ovarian dysgerminomas.

Chemotherapy

The leading role in the treatment of germ cell tumors belongs to chemotherapy. Many chemotherapy drugs are effective in this pathology. For a long time, polychemotherapy with three cytostatics was widely used: vincristine, actinomycin "D" and cyclophosphamide. However, in recent years, preference has been given to other drugs, on the one hand, new and more effective, on the other hand, having smallest number long-term consequences, and, first of all, reducing the risk of sterilization. Platinum preparations (in particular, carboplatin), vepezid and bleomycin are currently used most often for germ cell tumors.

Since the spectrum of germ cell tumors is extremely diverse, it is impossible to offer a single treatment regimen. Each localization and histological variant of the tumor requires its own approach to treatment and a reasonable combination of surgical, radiation and chemotherapy methods.

The most common germ cell tumor in boys under 5 years of age.

Choriocarcinoma of the testis (chorioepithelioma) - a malignant tumor of the testicles from germ cells with extra-embryonic differentiation, in structure resembles a tumor arising from the tissue of the placenta of a pregnant woman. It consists of mononuclear cells with a clear cytoplasm (reminiscent of cytotrophoblast cells) and giant cells (reminiscent of the structures of syncytiotrophoblast).

Macroscopically a small, painless seal with foci of necrosis and hemorrhage on the incision. Large choriocarcinomas are less common.

Microscopically syncytiotrophoblast is represented by irregularly shaped giant cells with highly vacuolated cytoplasm. The cytotrophoblast is formed by polygonal cells with round hyperchromic nuclei and a small amount of cytoplasm. The tumor is extremely invasive, sprouts blood vessels, resulting in foci of hemorrhage. In some cases, hemorrhagic necrosis is so pronounced that it is quite difficult to identify living tumor cells, and testicular choriocarcinoma is replaced by scar tissue. Testicular choricarcinoma, consisting only of cytotrophoblast and syncytiotrophoblast, is rare, more often the tumor is found as a component of mixed germ cell tumors.

Mixed germ cell tumors.

Almost half of testicular germ cell tumors are composed of more than one type of transformed germ cells and are classified as mixed germ cell tumors. There are over a dozen possible combinations of different types of tumor cells.

The most common are the following: 1) teratoma and embryonic cancer (teratocarcinoma); 2) teratoma, fetal cancer and seminoma; 3) embryonic cancer and seminoma. These combinations may include
and components of a yolk sac tumor. Teratocarcinoma in 20% (more often than embryonic cancer) is detected after the development of metastases.

In some cases, with a painless testicular tumor, the diagnosis of epididymitis or orchitis is erroneously diagnosed. Sometimes the first symptoms of the disease are due to metastases. Possible ureteral obstruction(manifestation of lesions of the para-aortic lymph nodes). It is also possible to observe stomach ache or pulmonary symptoms caused by many metastatic nodes.

Tumor markers. The presence in the blood of the characteristic products of tumor germ cells helps in the diagnosis, treatment and prognosis of the disease. The content of tumor markers in the blood decreases after orchiectomy (testicular resection) and again increases with the re-growth of the tumor.

Metastasis. Tumor tissue from transformed germ cells grows into the appendage and metastasizes to regional lymph nodes and lungs. Choriocarcinoma, unlike other germ cell tumors, immediately disseminates hematogenously to the lungs. In order of decreasing frequency, metastases are found in the retroperitoneal lymph nodes, lungs, liver, and mediastinal lymph nodes. Distant metastases are usually detected in the first 2 years after diagnosis and surgical treatment. Metastases of nonseminoma germ cell tumors treated with chemotherapy after orchiectomy are represented by the components of teratoma.

Tumors from stromal cells and seminiferous tubules.

Primary tumor growth from Sertoli cells, Leydig cells and granulosa cells accounts for 5% of all testicular tumors. There are tumors from one type of cells or mixed - from Sertoli cells and Leydig cells.

Tumor from Leidig's cells.

A rare neoplasm (about 2% of all testicular tumors) that develops from interstitial Leydig cells. The disease is detected in boys older than 4 years and in men from 30 to 60 years. Functionally active cells synthesize androgens and/or estrogens, the level of which in the blood can be increased. The activity of tumor cells in boys in the prepubertal period leads to premature physical and sexual development. In men, in some cases, on the contrary, feminization and gynecomastia are found.

TUMORS OF THE GENITAL ORGANS IN CHILDREN.

Malignant tumors of the genital organs account for 3% to 4% of the number of malignant neoplasms in childhood.

Embryogenesis of the human genitourinary system is very complex. The development of the urinary and reproductive systems proceeds inextricably and jointly by dividing the embryonic urogenital crest into medial (genital) and lateral (mesonephric) parts. Primordial germ cells form from the endoderm of the yolk sac at 4–6 weeks of fetal development and begin to migrate into the developing embryo, namely the urogenital crest. In the process of its development, the genital organs are increasingly isolated from the urinary system and are displaced into the small pelvis. Deviation from the normal course of this complex process causes the frequency of malformations (undescended testis, incomplete duplication of organs - kidneys, ureters, uterus and vagina, etc.) and tumors of the urogenital area (tumors of the ovaries, testicles, vagina). It should also be remembered that the gonad contains elements of all three germ layers and thus has the initial rudiments for the potential development of any malignant tumor.

Malignant tumors of the genital organs of girls affect mainly the ovaries (86%), then in terms of the frequency of lesions in second place are tumors of the vagina and cervix (10%), damage to the body of the uterus (3%). Very rarely, the vulva and external opening of the urethra are affected by rhabdomyosarcoma.

Malignant tumors of the genital organs in girls occur at any age from the neonatal period to 15 years, however, there are certain patterns in the structure of incidence depending on age: up to 5 years, rhabdomyosarcoma of the vagina and cervix is ​​more often noted, and at an older age and especially in during puberty, the tumor affects the ovaries.

The international classification of ovarian tumors is called histological, but at the same time it is consistent with the clinical and biological characteristics of tumors and is applicable in clinical practice (WHO, 1973). Here is an abridged version of it:

I. Epithelial tumors.

II. Sex cord stromal tumors:

A. Granulosoma-stromal cell tumors,

B. Androblastomas: tumors from Sertoli and Leydig cells,

B. Unclassified sex cord stromal tumors.

III. Lipid cell tumors.

IV. germ cell tumors.

V. Gonadoblastoma.

VI. Tumors of soft tissues, non-specific to the testis.

VII. unclassified tumors.

VIII. Secondary (metastatic) tumors.

Of all the morphological types, the most common are ovarian germ cell tumors (up to 80%) and sex cord stromal tumors (up to 13%). Epithelial tumors or true ovarian cancers are not typical for childhood and account for 7%. This is the most important difference in the incidence structure of children from adults, where ovarian cancer predominates.

germ cell tumors- Neoplasms typical of childhood make up to 3% of all malignant tumors in children. These tumors are extremely diverse in their morphological structure, clinical course and prognosis.

Germinogenic tumors are 2 times more common in girls. There are 2 peaks in the incidence of germ cell tumors in childhood: in children under 2 years of age with a decrease by 6 years and at the age of 13-14 years. The peak incidence of germ cell tumors in adolescents at the age of 13-14 is mainly due to damage to the ovaries and testicles.

The most common germ cell tumors are found in the testicles, ovaries, and sacrococcygeal region. Damage to the retroperitoneal space, mediastinum and vagina is not excluded.

Questions of morphological classification and histogenesis of germ cell tumors are closely interrelated. In the process of accumulating knowledge, classifications are constantly supplemented and changed. The following morphological classification of germ cell tumors of gonadal and extragonadal localization has been proposed (WHO, 1985):

I. Tumors of one histological type:

1. Germinoma (seminoma, dysgerminoma) classic.

2. Spermatocytic seminoma (only in the testis).

3. Embryonic cancer.

4. Tumor of the yolk sac (endodermal sinus).

5. Polyembryoma.

6. Choriocarcinoma.

7. Teratoma:

A. Mature,

B. Immature,

C. With malignant transformation (only in the ovary),

D. With a one-sided orientation of differentiation (ovarian struma, carcinoid).

II. Tumors of more than one histological type in various combinations.

It was noted that most often in children there are mature and immature teratomas, then tumors of the yolk sac and germ cell tumors of a complex structure. When comparing the morphological structure of the tumor and its localization, some regularities were noted. In the ovaries, teratomas, dysgerminomas and germ cell tumors of a complex structure are most common. With the localization of a tumor lesion in the testicle, the yolk sac tumor is in the first place, then teratomas, germ cell tumors of a complex structure, etc. A yolk sac tumor is more common in the vagina.

In the clinical picture of ovarian neoplasms, the leading symptoms are abdominal pain, an increase in the size of the abdomen and the presence of "seal" in the abdominal cavity. Some patients may have signs of precocious puberty or no signs of sexual development. Often, patients with ovarian tumors are hospitalized in surgical hospitals with a picture of an "acute abdomen", which is caused by torsion of the tumor stem or its rupture. Only when the process is disseminated, symptoms of intoxication appear: lethargy, pallor of the skin, loss of appetite, weight loss, etc.

Diagnosis and differential diagnosis of ovarian neoplasms include a carefully collected history, general clinical examination, palpation of the formation, examination of the perrectum, palpation of the abdominal organs with muscle relaxants, X-ray examination of the chest organs, excretory urography, ultrasound examination of the affected area. In unclear cases, to clarify the localization of the lesion or the prevalence of the process, computed tomography, angiography, irrigoscopy, cystoscopy, etc. are shown. During the examination, it is necessary to pay attention to the areas of regional metastasis, lungs, liver, bones.

If a germ cell tumor of any localization is suspected, a test for alpha-fetoprotein (AFP) is necessary. AFP is a specific component of embryonic and fetal serum alpha-globulin. After birth, there is a rapid decrease in AFP titer. A persistent and intense resumption of AFP production is characteristic of germ cell tumors. In addition, choriocarcinoma is characterized by the determination of the titer of chorionic hormone (CH). Carrying out these reactions allows you to clarify not only the diagnosis, but also to monitor the effectiveness of the treatment, since the levels of AFP and CG in serum correlate with the volume of tumor masses.

The data of a comprehensive examination make it possible to establish the stage of the tumor process:

T1 - the lesion is limited to the ovaries

T1a - one ovary, the capsule is intact,

T1b - both ovaries, the capsule is intact,

T1c Capsular rupture, surface tumor, malignant cells in ascitic fluid or abdominal lavage.

T2 - spread to the pelvis

T2a - uterus, tubes,

T2b - other tissues of the pelvis,

T2c - malignant cells in ascitic fluid or flushing from the abdominal cavity.

T3 - intraperitoneal metastases outside the pelvis and / or metastases in regional lymph nodes

T3a - microscopically detectable intraperitoneal metastases,

T3b- macroscopically detectable intraperitoneal metastases up to 2 cm,

T3c - defined intraperitoneal metastases up to 2 cm and / or metastases in regional lymph nodes.

T4 - distant metastases (excluding intraperitoneal)

Note: Metastases in the liver capsule are classified as T3/stage 3, metastases in the liver parenchyma are classified as M1/stage 4. Positive cytological findings in the pleural fluid are classified as M1/stage 4.

The prognosis in patients with ovarian tumor is determined by the possibility of radical removal of the tumor. As a rule, with ovarian tumors it is possible to perform surgery at the first stage of treatment. In case of ovarian tumors, surgical treatment consists in the removal of the uterine appendages on the side of the lesion and resection of the greater omentum, since it has been established on a large clinical material that the lesion of a malignant ovarian tumor is one-sided.

It should be emphasized that in case of true ovarian cancer, which is very rare in children, an operation is necessary in the amount of amputation or extirpation of the uterus with appendages on both sides and resections of the greater omentum, so the role of an urgent histological examination of the removed tumor is very high to decide on the extent of surgical intervention. .

Then chemotherapy is required. For the treatment of germ cell tumors of the ovaries, we most often use the VAB-6 scheme in a slightly modified version:

vinblastine 4 mg/m2 IV on day 1, cyclophosphamide 600 mg/m2 IV on day 1, dactinomycin 1 mg/m2 IV drip for 1 day, bleomycin 20 mg/m2 on days 1, 2, 3, cisplatin 100 mg/m2 intravenously drip for 4 days.

Intervals between courses are 3-4 weeks. Conducted 6 courses of the specified chemotherapy. In the treatment of dysgerminoma with a good clinical effect, the following chemotherapy regimen is used:

vincristine 0.05 mg/kg iv on days 1, 8, 15, cyclophosphamide 20 mg/kg iv on days 1,8, 15, prospidin 10 mg/kg IM every other day until DM = 2500-3000 mg.

Courses are held with an interval of 4 weeks, the number of courses is 6.

A good effect in the treatment of ovarian tumors was obtained with the use of chemotherapy drugs such as vepezid, adriamycin, etc. When prescribing chemotherapy for rare ovarian tumors, it is necessary to individually select a chemotherapy regimen and change it in a timely manner (in the absence of an effect from the treatment).

Radiation therapy for ovarian tumors is practically not used, with the exception of the treatment of ovarian dysgerminoma. In cases of non-radical surgery or treatment of metastases, radiation therapy should be performed on the lesion in SOD 30-45 Gy. Dysgerminoma is highly sensitive to radiation treatment, which makes it possible to obtain good treatment results even with a widespread tumor process.

The results of treatment are completely determined by the timeliness of the start of treatment and the radicalness of the surgical intervention.

Tumors of the vagina and cervix are considered together because, as a rule, one histological type of tumor is determined in children - rhabdomyosarcoma, which has the ability of multicentric growth. When the genitourinary tract is affected, embryonic rhabdomyosarcoma, the botryoid variant, is diagnosed.

Most often, rhabdomyosarcoma of the vagina and cervix occurs in girls under 3 years of age. At first, the tumor has the appearance of a polyp, which can only be detected with vaginoscopy. With further tumor growth due to trauma or insufficient blood supply and tumor decay, bloody or purulent-bloody discharge from the vagina appears. Often, as the tumor masses grow, they fall out of the vagina. There may be manifestations of cystitis and urination disorders due to compression of the tumor of the bladder, urethra, or infiltration of the bladder wall. The tumor is characterized by the ability to recur, it metastasizes at a later date, as a rule, against the background of an already existing recurrence of the disease.

Diagnosis of a tumor lesion of the vagina and cervix is ​​not difficult, it is enough to perform a rectal examination, vaginoscopy with a tumor biopsy. After a biopsy, there is no significant bloody discharge. At the time of the initial examination, due to the large size of the tumor, it is not always possible to establish the localization of the lesion, it is specified in the course of treatment after the reduction in the size of the formation.

Classification of tumors of the cervix, vagina is applicable only to cancer. It takes into account the depth of tumor invasion. Rhabdomyosarcoma - a tumor growing from under the mucous layer, as a rule, has the appearance of a tumor cluster, may have several isolated tumor nodes. More acceptable in this case is the International Classification of Soft Tissue Sarcomas (in children).

T1 - the tumor is limited to the organ, removal is possible:

T1a -< 5 см, Т1б - >5 cm

T2 - spread to neighboring organs / tissues, removal is possible:

T2a - < 5 см, T2b - > 5 cm.

T3, T4 are not determined, however, partial removal is possible, residual tumor is determined microscopically or residual tumor is determined macroscopically. Regional lymph nodes for the upper two-thirds of the vagina are the pelvic lymph nodes, for the lower third - the inguinal lymph nodes on both sides.

After histological verification of the diagnosis, special therapy begins with chemotherapy. In the process of treatment, the sensitivity of the tumor to chemotherapy is determined and the localization of the lesion is specified. Chemotherapy is carried out according to the scheme:

vincristine 2 mg/m2 IV on days 1, 8, 15, cyclophosphamide 200 mg/m2 IV on days 1,8, 15, dactinomycin 200 mg/m IV on days 2, 5, 9, 12, 16.

After 1-2 courses of chemotherapy and removal of the remaining tumor masses from the vagina, the affected area can be clearly defined.

If the vagina is damaged, it is impossible to perform a radical surgical intervention, so treatment is of great importance in this case, namely, intracavitary radiation therapy, which allows you to deliver significant doses (up to SOD 60 Gy). Only at such a dose is it possible to obtain a therapeutic effect in rhabdo-myosarcoma. In the future, special therapy should be continued in the form of chemotherapy.

In case of damage to the cervix, a radical surgical intervention is possible in the amount of extirpation of the uterus with the upper third of the vagina and fallopian tubes. After the operation, as well as with vaginal rhabdomyosarcoma, it is necessary to continue special therapy in the form of intracavitary irradiation of the vaginal stump and courses of chemotherapy. Number of chemotherapy courses 6-8.

If there is no effect from ongoing chemotherapy, it is necessary to include adriamycin in the regimen or change the regimen. Most often in such cases, the effect is obtained from the use of platinum with Vepezid.

Germinogenic tumors in the vagina are more often represented by a tumor of the yolk sac. A characteristic feature of these tumors is bleeding, more pronounced than with vaginal rhabdomyosarcoma. It should be noted the absence of significant bleeding, obviously, this is due to the still insufficiently developed genital organs and their blood supply.

It must be emphasized that in children with vaginal tumors, the question is about saving the life of the child. Unfortunately, the quality of life cannot be guaranteed in this category of sick children. Further scientific research is required to resolve this issue.

Testicular tumors- relatively rare tumors in boys and account for up to 1% of solid malignant tumors. Most often, children under 3 years of age are affected.

When studying the follow-up of sick children, a significant increase in the risk of testicular tumors in case of tuberculosis in the mother during pregnancy was established. The relative risk of testicular tumors was noted in boys whose mothers had epilepsy or had a history of stillbirths. Mothers of boys with testicular tumors suffered more often from severe toxicosis. Predisposing factors also include various congenital anomalies and malformations (hypoplasia or testicular atrophy, cryptorchidism, testicular ectopia). Trauma also plays a role, and possibly a family history as well.

It should be emphasized that malignant tumors predominate among testicular tumors: yolk sac tumor and embryonic cancer (up to 44%), embryonic rhabdomyosarcoma (15%), immature teratoma (up to 12%), mature teratoma (up to 10%), then more rare tumors - malignant tumors of the sex cord stroma, seminoma, leidigoma, neurofibroma, leiomyosarcoma. Seminomas, unlike adults, are rare in children.

With a tumor of the testicle, as a rule, the leading symptom is the presence of a dense, painless formation and an increase in the size of the testicle. Rarely, a tumor is an operative finding during surgery for hydrocele. General symptoms of intoxication appear only with the dissemination of the tumor process.

Diagnosis of testicular tumors consists in the usual examination - palpation, if in doubt, an aspiration biopsy is indicated, which, after a cytological examination of the punctate, makes it possible to establish the malignancy of the process in 85% of cases. Metastasis occurs in the retroperitoneal lymph nodes. To establish the prevalence of the process, it is necessary to conduct x-ray of the lungs, excretory urography, ultrasound of the scrotum, inguinal region on the side of the lesion, small pelvis, retroperitoneal space, liver; if necessary, computed tomography. As a diagnostic factor, as well as to monitor treatment, the determination of the AFP titer is shown.

Clinical international classification makes it possible to characterize the primary tumor:

T1 - the tumor is limited to the body of the testicle,

T2 - tumor extends to the white of the testicle or epididymis,

TK - the tumor spreads to the spermatic cord,

T4 - The tumor has spread to the scrotum.

However, it is convenient to determine the tactics of treatment according to the classification (Royal Marsden Hospital):

Stage I - no signs of metastasis, the primary tumor does not affect the spermatic cord and / or scrotum,

Stage II - there are metastases in the retroperitoneal lymph nodes,

Stage III - lymph nodes above the diaphragm are involved in the process,

Stage IV - there are non-lymphogenic metastases in the lungs, liver, brain, bones.

The prognostic value in testicular tumors is the stage of the disease and the morphological structure of the tumor, and in the case of a widespread process, the number and size of the affected lymph nodes and / or metastases to the lungs.

In the treatment of testicular tumors, a surgical method, radiation therapy and chemotherapy are used in terms of combined or complex treatment.

Surgical treatment of the primary focus consists in orchiofuniculectomy with ligation of the spermatic cord at the level of the internal opening of the inguinal canal. Bilateral lymphadenectomy of the retroperitoneal lymph nodes in children does not improve the results of treatment and therefore is not performed.

In the localized stage after removal of testicular rhabdomyosarcoma, prophylactic chemotherapy is indicated:

vincristine 0.05 mg/kg IV on days 1.8, 15, etc. weekly for 1.5 years (single dose not more than 2 mg),

cyclophosphamide 10-15 mg/kg IV or IM 1, 2, 3, 4, 5 days every 6 weeks,

dactinomycin 10-15 mcg/kg IV on days 1, 2, 3, 4, 5 every 12 weeks.

The duration of the indicated course of chemotherapy is up to 1.5 years. This chemotherapy can be enhanced with adriamycin. Testicular germ cell tumors are treated with the same drugs as ovarian tumors.

If retroperitoneal lymph nodes are affected, irradiation of the pelvic and para-aortic lymph nodes together with chemotherapy can cause a long-term remission. When lung metastases are affected, it is possible to obtain some success from the use of chemotherapy and total irradiation of the lungs in SOD 15 Gy and additional local irradiation in SOD 30 Gy.

The prognosis is much more favorable in children under 1 year of age, who are more likely to be diagnosed with localized forms of testicular tumors.