home Calculators Yolk sac cancer is more common with age. Choriocarcinoma, embryonic carcinoma and other testicular tumors. Mandatory and additional studies in patients with suspected germ cell tumors

Yolk sac cancer is more common with age. Choriocarcinoma, embryonic carcinoma and other testicular tumors. Mandatory and additional studies in patients with suspected germ cell tumors

Description:

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. 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.

Germ cell tumors are an uncommon type of neoplastic lesion in children. They make up 3-8% of all children and adolescents. 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.

Symptoms:

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).

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. These girls often have a fever.

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.

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. Most often these are very large tumors. 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.
Germinogenic in most cases represent a tumor of large size, however, 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. may precede other symptoms of the tumor.

Causes of occurrence:

Malignant germ cell tumors are very often associated with various genetic abnormalities, such as -telangiectasia, Klinefelter's syndrome, etc. These tumors are often combined with other malignant tumors, such as 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. produce 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 with other types of germ cell tumors, such as teratoma and tumor of the yolk sac, is recorded.

Treatment:

For treatment appoint:

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. Medium 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.
.
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 it was widely used by three cytostatics: 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 the least number of long-term effects, 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.

Such tumors include malignant and benign formations that form from primary germ cells. During the formation and development of the human embryo, these cells move, so germ cell formations can also form outside the gonads (organs that secrete germ cells): in the brain, retroperitoneal, sacrococcygeal zone, mediastinum and other areas.

Prevalence

The frequency of occurrence of such neoplasms is affected by the age of the patient:

up to 15 years - 2-4%;
in adolescence (15-19 years) - about 14%.

For childhood, there are two periods when the incidence is at its peak:

The first - up to 2 years, girls get sick more often than boys (74%). During this period, in most cases, neoplasms are localized in the sacrococcygeal zone.
The second is slightly different for girls and boys. This peak occurs during adolescence: 11-14 years for boys and 8-12 for girls. Most tumors are found in the gonads.

In recent years, most researchers talk about an increase in the number of cases of detection of germ formations. This trend is especially pronounced in the male part of the population, with the localization of tumors in the testicles. In men, in recent years, the incidence has increased from 2 to 4.4 per 100,000 people.

A common cause of malignant germ cell tumors is various genetic abnormalities, for example, Klinefelter's syndrome or ataxia-telangiectasia, pure and mixed gonadal dysgenesis, cryptorchidism, hermaphroditism, etc.

Histological classification

Germinoma (if localized in the testicle - seminoma, in the ovary - dysgerminoma, in other anatomical zones - germinoma).
Teratoma:

mature;
immature - differs in degrees of immaturity:

first degree;
second;
third.

Embryonic carcinoma.

Neoplasm of the yolk sac.

Choriocarcinoma.

Gonadoblastoma.

Germinogenic neoplasms of mixed type.

Clinical picture

The clinical picture of this disease is characterized by diversity. And first of all, it is determined by where the tumor is located. The most common localizations:

27% - in the coccyx area;
26% - in the ovaries;
18% - in the testicles;
15% - in the brain.

In much more rare cases, such tumors are diagnosed in the mediastinum, retroperitoneal space, stomach, neck (that is, in the nasopharynx), in the bladder, liver, and vagina.

Testicle

Primary formations in the testicles (they are called testicular) are rarely diagnosed in childhood. Most often they are found in children under the age of 2 years, while 25% - already at birth.

Figure 2. - seminoma of the testis: a - macropreparation, b - MRI.

According to the histological (that is, tissue) structure, these are most often neoplasms of the yolk sac or benign teratomas.

The second peak in the occurrence of testicular tumors is puberty. During this period, the incidence of malignant teratomas increases. Seminomas are extremely rare in children.

Swelling of the testicle, which increases rapidly and does not cause pain to the child, is most often detected by parents. 10% of these neoplasms are combined with "hydrocele" (med. "hydrocele") and other congenital pathologies, especially the urinary system.

On examination, a dense neoplasm, tuberous, without signs of inflammation is seen. The diagnosis of a tumor before surgery is confirmed by an elevated level of alpha-fetoprotein (AFP).

At the same time, the neoplasm contains elements of the yolk sac.

Symptoms of metastases in the para-aortic lymph nodes are lower back pain.

ovaries

Ovarian (located in the ovaries, ovarian) tumors most often present with abdominal pain. On examination, a tumor is palpated in the pelvis, often in the abdominal cavity. Also, due to ascites (accumulation of fluid in the abdominal cavity), the abdomen is enlarged. Often, girls with this diagnosis have a fever.

The most commonly diagnosed germ cell tumor is dysgerminoma. Mostly it is found in the second decade of life. It is rare in little girls. Such a disease spreads relatively quickly, affecting the peritoneum and the second ovary. Usually neoplasms are unilateral, have large sizes. In this regard, a frequent occurrence is the rupture of the neoplasm capsule.

Figure 3. - ovarian teratoma a - ultrasound, b - MRI.

Malignant teratomas usually present with a non-specific clinical presentation associated with the presence of a tumor:

increased volume of the abdomen;
pain;
violation of the menstrual cycle (not always observed).

sacrococcygeal zone

According to the frequency of detection, this is the third zone of location of germ cell tumors. In 75% of cases, it is diagnosed up to 2 months, almost always it is a benign, mature teratoma. Neoplasms in such patients are detected in the perineum or buttocks. Most often, tumors are characterized by large sizes. Sometimes neoplasms are detected at an older age and are common inside the peritoneum. In such cases, according to the results of histology, a malignant nature is revealed, often with elements of a neoplasm of the yolk sac.

Tumors in the sacrococcygeal zone often lead to difficulties with the act of defecation and urination (dysuric disorders).

Figure 4. Sacrococcygeal teratoma in a child.

Figure 5. Sacrococcygeal teratoma in a 2-year-old child (MRI).

Mediastinum

In the mediastinum, in most cases, there are large tumors, but the syndrome of compression of the superior vena cava is rarely diagnosed.

Figure 6. - CT scan of the chest - mediastinal seminoma.

The histological picture of the neoplasm is mainly of mixed origin, has a teratoid component and cells that are characteristic of a yolk sac neoplasm.

Brain

Germinogenic neoplasms in this area are approximately 2-4% of all intracranial (intracranial) ones. Pathology in 75% of cases is diagnosed in boys, with the exception of the Turkish saddle, where neoplasms are mainly localized in girls.

Germinomas form large infiltrating tumors that are often sources of subarachnoid (between the pia mater and arachnoid) and ventricular (ventricular) metastases.

Figure 7. - Germinoma of the brain.

Vagina

Almost always, these are neoplasms of the yolk sac. All cases described in medicine were found at the age of 2 years. These tumors usually present with spotting and bleeding from the vagina.

The tumor has the appearance of polypoid masses, comes from the back and side walls of the vagina.

Germinogenic neoplasms secrete AFP, as well as human chorionic gonadotropin (CG). The ability to secrete these substances is expressed in different ways, depending on the morphology of the tumor.

Normally, AFP is secreted by the cells of the yolk sac and liver of the embryo and is its important serum protein. As the fetus progresses to later stages of development, AFP production switches to albumin production. Therefore, in newborns, AFP is determined in the blood serum in very high concentrations, gradually decreasing and reaching normal values for an adult by the 1st year. AFP has a half-life of 4-5 days.

CG is normally produced by placental syncytiotrophoblasts. Germinogenic neoplasms produce it by trophoblastic structures, as well as giant cells of syncytiotrophoblast. Intact serum CG has a half-life of 24-36 hours.

A highly specific method for determining hCG and AFP in children significantly improves the quality of diagnosis, since its efficiency approaches 100%. Tumor markers are determined at the stage of differential diagnosis, after surgery and before each course of chemotherapy. A full examination is impossible without the definition of markers in dynamics.

Another marker of any germ cell tumors is lactate dehydrogenase (LDH). However, the activity of this indicator is less specific than the levels of hCG and AFP. LDH activity in the late stages of development of non-seminoma neoplasms is increased in 50-60% of patients, in the late stages of development of seminoma - in 80%.

Diagnostics

The diagnostic process includes several stages:

Treatment

Children undergo surgery to remove the tumor and chemotherapy. The sequence of actions depends on the location and prevalence of the process. Usually, if the gonads are affected, then the tumor must first be removed, and chemotherapy should be carried out after the operation. If CT / MRI showed a clear infiltration (germination) into the surrounding tissues or the presence of metastases in the lymph nodes, lungs, liver and other organs, then primary chemotherapy is first performed after determining tumor markers and instrumental imaging.

Indications for chemotherapy and the number of courses are determined by how common the process is, what is the level of tumor markers in the blood, and the nature of the operation performed.

The standard chemotherapy drugs are etoposide, bleomycin, and cisplatin. In the presence of extensive lung disease, respiratory failure of any degree, in order to prevent bleomycin toxicity in the lungs, other treatment regimens may be carried out as an alternative.

It is necessary to say separately that when conducting systemic drug therapy to a patient, it is necessary to strictly observe the time frame - the next cycle is carried out on the 22nd day.

When conducting chemotherapy, it is necessary to regularly evaluate its effectiveness. To do this, every 2 cycles and after its completion, a CT scan of those areas that were initially affected by tumors is performed. Also, before each cycle, the level of tumor markers in the blood is checked. If during therapy or after its completion, the level of tumor markers increases or its decrease slows down, then this indicates that the tumor process is active and a second line of chemotherapy should be performed.

Radiation therapy is indicated more often when the process is localized in the brain. It allows you to accurately, locally act on tumors.

Forecast

Percentage of overall survival in germ cell neoplasms:

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

The prognostic factors are:

the level of tumor markers;
histological structure of the tumor;
prevalence of the process.

Unfavorable factors are the large size of the neoplasm, late diagnosis, tumor rupture, resistance to chemotherapy (resistance to drug treatment), and relapse of the disease.

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 such chemotherapy drugs 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.

- a group of neoplasias that develop from the primary germ cells of the gonads. They can occur both in the testicles or ovaries, and extragonadally. Manifestations depend on localization. With superficial neoplasms, visible deformation is observed, with nodes in the ovary, pain, dysuria and menstrual irregularities are noted. With germ cell tumors of the mediastinum, shortness of breath occurs, with intracranial lesions, focal and cerebral symptoms are detected. The diagnosis is made taking into account the symptoms, x-ray data, ultrasound, CT, MRI and other methods. Treatment - surgery, chemotherapy, radiotherapy.

General information

Germ cell tumors are a group of benign and malignant neoplasias arising from primary germ cells that are the precursors of the testicles and ovaries. Due to the migration of such cells during embryogenesis, germ cell tumors can develop outside the gonads: in the mediastinum, sacrococcygeal region, brain, retroperitoneal space, and other anatomical areas. Primary extragonadal neoplasms account for 5% of the total number of germ cell tumors.

The ratio between the number of extra- and intragonadal neoplasia changes with age. In young children, lesions of the sacrococcygeal zone predominate; as they grow older, the frequency of neoplasms in the testicles and ovaries increases. Germ cell tumors of all localizations account for 3% of the total number of oncological diseases in children, germ cell tumors of the ovaries - 2-3% of all malignant ovarian neoplasia in women, germ cell lesions of the testicle - 95% of the total number of testicular tumors in men. Treatment is carried out by specialists in the field of oncology, gynecology, urology and other fields of medicine.

Causes of germ cell tumors

Germinogenic tumors arise from germinal germ cells, which are formed in the yolk sac at the initial stages of embryogenesis, and then migrate through the body of the embryo to the urogenital scallop. During migration, some of these cells can linger in various anatomical zones, which subsequently leads to the formation of germ cell tumors of extragonadal localization. Normally, germ cells turn into mature cells of the testicles and ovaries, however, under certain conditions, such cells can remain in their embryonic state and, under the influence of negative external and internal factors, give rise to neoplasms of the gonads.

It has been established that germ cell tumors are often diagnosed in patients with various genetic abnormalities, such as Klinefelter's syndrome. A hereditary predisposition is revealed, which may or may not be combined with chromosomal disorders. A characteristic feature of germ cell tumors is the isochromosome resulting from the doubling of the short arm and the loss of the long arm on chromosome 12, however, other chromosomal abnormalities may also be detected. There is a frequent combination of germ cell tumors with other oncological lesions, including leukemia, lymphomas and neuroblastomas. The likelihood of testicular germ cell neoplasia is increased in cryptorchidism.

The histological type of germ cell tumors depends on age. In newborns, benign teratomas are more often diagnosed, neoplasia of the yolk sac is detected in young children, malignant teratomas and dysgerminomas are found in adolescents, seminomas in adults, etc. The factors contributing to the activation of growth and malignant transformation of germinal germ cells have not yet been elucidated. It is assumed that the impetus for the development of germ cell tumors in children may be chronic diseases of the mother or the mother taking certain medications.

Classification of germ cell tumors

There are several classifications of germ cell neoplasia, based on the morphological characteristics of the neoplasm, location and course of the disease. According to the WHO classification, the following morphological types of germ cell tumors are distinguished:

Germinoma (dysgerminoma, seminoma)
Embryonic cancer
Yolk sac neoplasia
spermatocyte seminoma
Polyembryoma
Teratoma, including mature, immature, with a certain direction of tissue differentiation (carcinoid, ovarian struma), malignant.
Mixed germ cell tumor, which is a combination of several histological variants of neoplasia.

The source of germinomas is primary germ cells, the source of other neoplasias is the elements of the environment of such cells.

Based on localization, gonadal and extragonadal germ cell tumors are distinguished. Extragonadal neoplasias are divided into extracranial and intracranial. In addition, there are malignant and benign germ cell neoplasia, as well as primary and recurrent neoplasms.

Symptoms of germ cell tumors

Features of the course of the disease are determined by the localization, size and degree of malignancy of neoplasia. Typical symptoms of ovarian germ cell tumors are abdominal pain of varying intensity in combination with menstrual irregularities. In children, the last sign is absent, which leads to a lack of alertness in relation to damage to the internal genital organs in the initial stages of the disease. With the progression of germ cell tumors, the listed symptoms are accompanied by an increase in the abdomen and urination disorders. On palpation in the initial stages, a rounded, moderately mobile node with clear contours is determined. Subsequently, the node increases in size, there is an increase and deformation of the abdomen. In the later stages, ascites and dysfunctions of various organs due to distant metastasis are detected.

Germinogenic testicular tumors are manifested by an increase in the corresponding half of the scrotum, a feeling of heaviness and fullness. Soreness or hypersensitivity of the affected area is noted by about 25% of patients. On palpation, a tumor-like formation or a uniform increase in the testicle will be determined. In 5-10% of patients with germ cell tumors, a hydrocele is detected, in 10-14% - gynecomastia. With lymphogenous and distant metastasis, an increase in inguinal lymph nodes, neurological disorders, pain in the bones, in the back and in the abdomen are possible.

Germinogenic tumors of the mediastinum, as a rule, are localized behind the sternum. Benign neoplasms (teratomas) are characterized by slow growth, malignant ones (teratoblastomas and other neoplasias) - aggressive spread and rapid germination of nearby organs. The most common manifestations of a germ cell tumor are shortness of breath, cough, and chest pain. With compression of the superior vena cava, noise in the head, headache, tinnitus, disturbances of consciousness, drowsiness and visual disturbances occur. Seizures are possible. In malignant germ cell tumors, hyperthermia, fever, weight loss and dysfunction of various organs due to germination or distant metastasis are observed.

Retroperitoneal germ cell tumors are asymptomatic for a long time. May present with dyspepsia, abdominal pain, dysuria, shortness of breath, edema, and varicose veins of the lower extremities. With malignant lesions in the later stages, symptoms of cancer intoxication are revealed. Germinogenic tumors of the sacrococcygeal zone are usually diagnosed in young children and proceed benignly. With large neoplasia, pain and weakness in the lower extremities, defecation disorders and dysuria are observed. Bleeding and necrosis are possible. Intracranial germ cell tumors are more often localized in the area of the epiphysis, sometimes in the area of the hypothalamus or pituitary gland. Manifested by headache, nausea, vomiting and disorders of the movements of the eyeballs.

Diagnosis and treatment of germ cell tumors

The diagnosis is established taking into account complaints, the results of a physical examination and data from additional studies. Depending on the location of the neoplasia, a rectal examination or vaginal examination may be required. Patients are prescribed ultrasound, CT and MRI of the affected area. Assess the content of alpha-fetoprotein in the blood serum. In malignant germ cell tumors to exclude lymphogenous and distant metastases, however, the effectiveness of this method in germ cell tumors is still difficult to assess due to the insufficient number of observations.

The prognosis for benign neoplasia is usually favorable. Malignant germ cell tumors were previously considered as prognostically unfavorable, but the use of combination therapy has increased the five-year survival rate for this pathology to 60-90%. Survival is affected by the type and extent of the germ cell tumor, the radical nature of the surgical intervention, and the presence or absence of metastases.

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.

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.

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 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.

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

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.

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.

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 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 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 two features are of great importance for intracranial germ cell tumors: 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 stage classification most commonly used at present (Table 14-2).

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.

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

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

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 the least number of long-term effects, 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.