Embryonic tumor of the yolk sac. Choriocarcinoma, embryonal carcinoma and other testicular tumors

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 embryo. During embryonic development, primordial germ cells migrate from the endoderm of the yolk sac to the genital ridge in the retroperitoneum. Here, the germ cells develop into gonads, which then descend into the scrotum, forming the testes, or into the pelvis, forming the ovaries. If during the period of this migration, for some unknown reason, a disruption of the normal migration process occurs, the germ cells can linger at any point along 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 commonly, germ cells are retained in the vagina, bladder, liver, and nasopharynx.

Germ cell tumors are an uncommon type of tumor lesion in children. They make up 3-8% of all malignant tumors of childhood and adolescence. Since these tumors can also be benign, their incidence is probably much higher. These tumors are two to three times more common among girls than boys. The mortality rate among girls is three times higher than among boys. After 14 years of age, mortality among males becomes higher, which is 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 syndrome, etc. These tumors are often combined with other malignant tumors, such as neuroblastoma and hematological malignancies. Undescended testicles pose a risk for developing 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 can be duplicated or lost. Multiple examples of germ cell tumors have been reported in siblings, twins, mothers and daughters.

Differentiation along the embryonic line gives rise to 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 germ cell lineages. Thus, teratomas may have a population of yolk sac cells or trophoblasts.

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

The factors causing malignant changes are unknown. Chronic diseases and long-term drug treatment during maternal pregnancy may be associated with an increased incidence of germ cell tumors in children.

The morphological picture of germ cell tumors is very diverse. Germinomas consist of groups of large, uniform neoplastic cells with a swollen nucleus and clear cytoplasm. Tumors of the yolk sac have a very characteristic picture: a reticular stroma, often called lacy, in which there are rosettes of cells containing a-fetoprotein in the cytoplasm. 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 the localization of the primary tumor: tumors of the yolk sac predominantly 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 (6-14 years old) tumors of the ovaries and testicles are more often diagnosed. pineal region.

Choriocarcinomas are rare but extremely malignant tumors that most often arise in the mediastinum and gonads. They can also be congenital.

Typical locations for dysgerminomas are 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 rarely found in childhood; most often, a combination of elements of embryonic cancer with other types of germ cell tumors, such as teratoma and yolk sac tumor, is recorded.

The clinical picture of germ cell tumors is extremely diverse and, first of all, is determined by the location 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 retroperitoneum, mediastinum, vagina, bladder, stomach, liver, and 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 at birth. According to 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 puberty period, when the frequency of malignant teratomas increases. Seminomas in children are extremely rare. Painless, rapidly increasing swelling of the testicle is most often noticed by the child's parents. 10% of testicular tumors are combined with hydrocele and other congenital anomalies, especially of the urinary tract. Upon examination, a dense, lumpy tumor is detected, with no signs of inflammation. An increase in alpha-fetoprotein levels 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. Upon examination, you can detect tumor masses located in the pelvis, and often in the abdominal cavity, an increase in the volume of the abdomen due to ascites. These girls often develop fever (Fig. 14-3).

Dysgerminoma is the most common ovarian germ cell tumor, which is mainly diagnosed in the second decade of life, and rarely in little girls. The disease quickly spreads to the second ovary and peritoneum. Tumors of the yolk sac are also more common in girls during puberty. Tumors are usually unilateral and large in size, therefore, rupture of the tumor capsule is a common occurrence. Clinical manifestations of malignant teratomas (teratocarcinomas, embryonal carcinomas) usually have a nonspecific picture with the presence of tumor masses in the pelvis, and menstrual irregularities may be observed. Patients in the prepubertal period may develop a state of pseudopuberty (early puberty). Benign teratomas are usually cystic, can be detected at any age, often give the clinical picture of ovarian torsion with subsequent 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 arises from the lateral or posterior walls of the vagina and has the appearance of polypoid masses, often pedunculated.

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

Mediastinum.
Mediastinal germ cell tumors in most cases are large tumors, but superior vena cava compression syndrome 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.
Germ cell tumors of the brain 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 sella turcica, where tumors are favored to be localized in girls. Germinomas form large infiltrating tumors, which are often the source of ventricular and subarachnoid cerebrospinal metastases (see the chapter “CNS Tumors”). Diabetes insipidus may precede other tumor symptoms.

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

Chest X-ray is a mandatory research method to establish a diagnosis in the case of primary mediastinal lesions, and is also indicated for identifying metastatic lesions of the lungs, which is very common.

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

CT and MRI of the brain are necessary to identify germ cell tumors of the pineal gland.

Ultrasound is a very useful examination method for quickly and easily diagnosing the primary lesion and for monitoring the effect of treatment. Ultrasound is a more convenient method, since CT often requires anesthesia to conduct 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, while neoplasms with yolk sac elements can produce AFP derivatives. 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 AFP content drops 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 before surgery and chemotherapy. After treatment (surgery and chemotherapy), in case of complete tumor removal or tumor regression after chemotherapy, their level drops, 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 cerebrospinal fluid may be useful for diagnosing patients with CNS tumors.

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

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

If there is a suspicion of a germ cell tumor in the abdominal cavity or 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 reasons, for example, in case of torsion of the cyst stalk or rupture of the tumor capsule.

If you suspect an ovarian tumor, you should not limit yourself to a classic transverse gynecological incision. Midline laparotomy is recommended. When opening the abdominal cavity, the lymph nodes of the pelvis and retroperitoneal region are examined, the surface of the liver, the subphrenic space, the greater omentum and the 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 rinsed and the resulting rinsing water subjected to cytological examination.

If an ovarian tumor is detected, the tumor should be subjected to urgent histological examination, and the ovary should be removed only after confirmation of the malignant nature of the tumor. This practice avoids removing 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 located in one ovary, removing one ovary may be sufficient. If the second ovary is affected, part of the ovary should be preserved if possible.

Recommendations for using the surgical method for ovarian damage:
1. A transverse gynecological incision should not be used.
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 rinsing waters.
5. Examination and, if necessary, biopsy:
- lymph nodes of the 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, must be removed immediately to avoid malignancy of the tumor. The operation must include complete removal of the coccyx. This reduces the likelihood of relapse of the disease. Malignant sacrococcygeal tumors must be treated initially with chemotherapy, followed by surgery to remove any residual tumor.

Surgery for a biopsy for a local tumor in the mediastinum and persistent AFP is not always justified, as it is associated with risk. Therefore, it is recommended to carry out preoperative chemotherapy and, after reducing the size of the tumor, surgical removal.

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 dysgerminoma.

Chemotherapy plays a leading role in the treatment of germ cell tumors. Many chemotherapy drugs are effective for this pathology. For a long time, polychemotherapy with three cytostatics was widely used: vincristine, actinomycin "D" and cyclophosphamide. However, recently, 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 consequences, and, first of all, reducing the risk of sterilization. The drugs most often used today for germ cell tumors are platinum (in particular, carboplatin), Vepezid and bleomycin.

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

A rather dangerous disease is a tumor of the yolk sac. Children under three years of age are at particular risk. Much less often, the neoplasm appears in adults and develops together with other germ cell type tumors. This malignant neoplasm is localized in the testicles in men and ovaries in women. It can also spread to the epididymis or spermatic cord. In addition, in women, it is clinically characterized by enlargement of the ovary in a very short period of time, which can pose a serious threat to overall health.

What is a yolk sac tumor and why is it dangerous?

A tumor of the yolk sac, or as it is also called in medicine “tumor of the endodermal sinus,” is a malignant formation, gray or yellow-gray in color. It develops from particles of the yolk sac and has a soft, elastic consistency. It is the third most common among.

Its rapid growth poses a particular danger. Untimely diagnosed pathology develops over a short period and leads to the formation of cysts and degeneration of tumor areas. The development of such events leads to the death of the patient in the absence of timely treatment.

Exact causes of development and who is at risk

Due to a failure in the process of movement and retention of embryonic cells, active growth of the tumor begins. Risk factors for such developments are:

Chemicals and their effects on the fetus in the womb.
Genetic predisposition. Often the pathology accompanies Klinefelter syndrome (an extra X chromosome in boys). Boys whose fathers suffered from this phenomenon are more susceptible to developing cancer than others.
The testicles did not descend into the scrotum. The pathology in medicine is called cryptorchidism. The temperature in the peritoneum is higher than in the cavity into which these organs should descend. This contributes to the development of oncology.
Age 20-34 years. The neoplasm can occur at different age periods, but infancy and the above-mentioned age are in a special risk zone.
HIV infection.
Congenital pathologies of the testicles, kidneys or male reproductive organ.
Race and Ethics. Statistics show that white people are disproportionately affected by this type of cancer. But there is no scientific basis for this fact yet.

The exact causes of the disease are not yet fully understood.

How to independently recognize a yolk sac tumor by its early signs?

Often such tumors are diagnosed after a complete examination of the patient. But a person is able to recognize the presence of a malignant tumor by the following signs:

torsion of the leg can create acute pain in the abdominal area, reminiscent of an exacerbation of appendicitis;
a painless volumetric roundness can be felt in the abdomen or pelvis;
Women may not have periods. In this case, pathology may develop against the background of gonadal dyskinesia. Doctors refer the patient for a karyotype study.

When analyzed, almost every patient has an increased level of AFP in the serum (a very important tumor marker).

What does an advanced clinical picture look like?

A tumor of the yolk sac poses a threat to human life. Metastases tend to grow and multiply very quickly, which often leads to the death of patients due to ignoring the disease or late diagnosis. The formation, in the absence of necessary treatment, can metastasize lymphogenously to the lymph nodes of the peritoneum, and hematogenously to other organs: liver, lungs and others. Such a development of events without the necessary treatment leads to irreversible consequences.

What is included in the diagnosis?

Most often, a neoplasm of this type is diagnosed at the age of 16-18 years. When a yolk sac tumor is detected, a number of diagnostic procedures are carried out, as with any other germ cell formation:

Anamnesis collection: examination and interview of the patient.
General analysis of urine and blood.
Ultrasound of the peritoneum.
Chest X-ray.
MRI of the affected area.
Electrocardiogram and echocardiogram.
Coagulogram and audiogram.
Biochemical blood test.
Boipsia.

If the lungs are suspected, an MRI of these organs and a separate EchoCG of the brain are performed.

A very important and indicative study is the determination of AFP in serum. Using the analysis, you can monitor the treatment process, its result, as well as identify metastases and the possibility of relapse. Doctors often use this method to determine the number of courses of chemotherapy required for a particular patient.

Non-surgical treatment and its feasibility

When a yolk sac tumor is diagnosed, surgery is immediately prescribed, since the formation is malignant and can actively spread, affecting other human organs.

Afterwards, for several years, monitoring is carried out using a chest x-ray and analysis of AFP levels. An increase in the latter indicator threatens a relapse of the pathology. In these cases, chemotherapy or radiation therapy is performed. Previously, doctors tried to treat patients with various medications, but the results did not live up to expectations. Until now, unfortunately, no miracle cure for such a malignant tumor has been invented.

What medications are used to treat yolk sac tumor?

In the past, treatment of the tumor was carried out through radiation therapy or taking an alkylating drug such as Methotrexate or Dactinomycin. But the result was not very positive: only 27% of patients managed to live at least a couple more years. Later, it was revealed that such a tumor was not sensitive to radiation, although the presence of positive dynamics at first may be confusing.

Chemotherapy, as mentioned above, is effective only after surgery. As a result of complete removal of stage 1-3 tumors, chemotherapy according to the VAC regimen for 6-9 courses in 78% of patients resulted in complete absence of signs of the disease. In the recent past, treatment was carried out with Bleomycin, Etoposide, and Cisplatin. Of the 21 people, 9 patients did not have the disease. Statistics noted by GOG experts.

Surgical treatment and its possible consequences

Surgical treatment is used at any stage of development of a yolk sac tumor. In medicine, the operation is called radical orchiectomy - complete removal of the organ down to the level of the deep inguinal ring. If there is pathology in the inguinal lymph nodes, they are also eliminated through a modified radical retroperitoneal lymphadenectomy.

After surgery, AFP levels return to normal after five days. Otherwise, incomplete removal of the formation or the presence of metastases is assumed. In most cases, surgery is successful. To consolidate the result, chemotherapy is often carried out according to an individually prescribed program for each person.

What happens if a tumor of the yolk sac is not treated and what are the consequences?

As a result of the fact that metastases radiate to the liver, kidneys and brain, it is unlikely to be able to avoid death in the absence of special treatment. Moreover, age and gender do not play any particular role. Therefore, it is very important to consult a doctor at the first signs of the disease and follow all recommendations, carry out all procedures and not refuse surgical treatment! The patient's life expectancy directly depends on this.

Prognosis and how long do such patients live?

In general, the prognosis at the first stage of pathology is favorable. When a malignant tumor is diagnosed at the first stage of its progression, treatment is successful in 95% of cases.

Children under 2 years of age have a greater chance of full recovery than people of other age categories. The reason for this is the combination of formation with other germ cell tumors in older people. With a disseminated process, the survival rate is only 50%, even if surgery and chemotherapy are performed.

Diagnosing a tumor in the early stages is treated less aggressively. As a result, there are much fewer side effects.

Malignant yolk sac tumor It is quite rare and can be successfully cured, so do not despair. Seek help from good doctors. They will do everything possible to ensure that the patient continues to live a full life for a long time. If you ignore the disease, then the outcome can be disastrous.

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

Testicular choriocarcinoma (chorionepithelioma) - a malignant tumor of the testicles from germ cells with extra-embryonic differentiation, the structure resembles a tumor arising from the placenta tissue of a pregnant woman. Consists of mononuclear cells with clear cytoplasm (resemble cytotrophoblast cells) and giant cells (resemble syncytiotrophoblast structures).

Macroscopically small painless compaction with foci of necrosis and hemorrhages on the incision. Larger choriocarcinomas are less common.

Microscopically syncytiotrophoblast is represented by giant cells of irregular shape with highly vacuolated cytoplasm. The cytotrophoblast is formed by polygonal cells with round hyperchromic nuclei and a small volume of cytoplasm. The tumor is extremely invasive, grows blood vessels, resulting in areas of hemorrhage. In some cases, hemorrhagic necrosis is so severe that it can be 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 consist of more than one type of transformed germ cells and are classified as mixed germ cell tumors. There are more than a dozen possible combinations of different types of tumor cells.

The most common are the following: 1) teratoma and embryonal cancer (teratocarcinoma); 2) teratoma, embryonal carcinoma and seminoma; 3) embryonal cancer and seminoma. Such combinations may contain
and yolk sac tumor components. Teratocarcinoma is detected in 20% of cases (more often than embryonal cancer) after the development of metastases.

In some cases, a painless testicular tumor is mistakenly diagnosed as epididymitis or orchitis. Sometimes the first symptoms of the disease are caused by metastases. Possible ureteral obstruction(manifestation of lesions of the para-aortic lymph nodes). You can also watch stomach ache or pulmonary symptoms caused by multiple metastatic nodes.

Tumor markers. The presence of characteristic products of tumor germ cells in the blood helps in the diagnosis, treatment and prognosis of the disease. The content of tumor markers in the blood decreases after orchiectomy (resection of the testicle) and increases again with 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 into 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 teratoma components.

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 cell or mixed - from Sertoli cells and Leydig cells.

Tumor from Leidig's cells.

A rare neoplasm (about 2% of all testicular tumors) developing 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 may be increased. The activity of tumor cells in boys during the prepubertal period leads to premature physical and sexual development. In some cases, in men, on the contrary, feminization and gynecomastia are detected.

Description:

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

Symptoms:

Ovaries.
Ovarian tumors often present with abdominal pain. Upon examination, you can detect tumor masses located in the pelvis, and often in the abdominal cavity, increasing the volume of the abdomen due to. These girls often develop fever.

Sacrococcygeal region.
This is the third most common location of germ cell tumors. The incidence of these tumors is 1:40,000 newborns. In 75% of cases, the tumor is diagnosed before two months and is almost always a mature benign teratoma. Clinically, such patients have tumor formations in the perineum or buttocks. Most often these are very large tumors. In some cases, neoplasms have intra-abdominal spread and are diagnosed at an older age. In these cases, the histological picture is most often more malignant, often with elements of a yolk sac tumor. Progressive malignant tumors of the sacrococcygeal region often lead to dysuric symptoms, problems with bowel movements and urination, and neurological symptoms.

Mediastinum.
Germ cell tumors in most cases represent a large tumor, but compression syndrome 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.
Germ cell tumors of the brain 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 sella turcica, where tumors are favored to be localized in girls. Germinomas form large infiltrating tumors, which are often the source of ventricular and subarachnoid cerebrospinal metastases. may precede other tumor symptoms.

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 developing testicular tumors.

Often, germ cell tumors may contain cells of different germ cell lineages. 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, uniform neoplastic cells with a swollen nucleus and clear cytoplasm. Tumors of the yolk sac have a very characteristic picture: a reticular stroma, often called lacy, in which there are rosettes of cells containing a-fetoprotein in the cytoplasm. 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 often arise in the mediastinum and gonads. They can also be congenital.

Typical locations for dysgerminomas are 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 the following is prescribed:

If you suspect an ovarian tumor, you should not limit yourself to a classic transverse gynecological incision. Median is recommended. When opening the abdominal cavity, the lymph nodes of the pelvis and retroperitoneal region are examined, the surface of the liver, the subphrenic space, the greater omentum and the 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 dysgerminoma.

Chemotherapy.
Chemotherapy plays a leading role in the treatment of germ cell tumors. Many chemotherapy drugs are effective for this pathology. For a long time, it was widely used by three cytostatics: vincristine, actinomycin "D" and cyclophosphamide. However, recently, 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 consequences, and, first of all, reducing the risk of sterilization. The drugs most often used today for germ cell tumors are platinum (in particular, carboplatin), Vepezid and bleomycin.