Yolk sac cancer is more common in older people. Germ cell tumors

Chapter 14.

Germ cell tumors develop from a population of pluripotent germ cells. The first germ cells can be found in the endoderm of the yolk sac as early as a 4-week embryo. During embryonic development, primordial germ cells migrate from the endoderm of the yolk sac to the genital ridge in the retroperitoneum (Figure 14-1). 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.

Epidemiology

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.

Histogenesis

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 location 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.

Clinical picture

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 young 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.

Diagnostics

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.

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).

Treatment.

Surgical method of treatment.

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.

Radiation therapy

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, 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

In past treatment of yolk sac tumors did not inspire optimism. Kurman and Norris reported no long-term survival in 17 patients with stage I disease who received additional RT or a single alkylating agent (dactinomycin or methotrexate). In 1979, Gallion presented a review of the literature indicating that only 27% of 96 patients with stage I disease survived 2 years. The tumor is insensitive to radiation therapy, although positive dynamics may be observed at the beginning of its implementation. Surgical treatment is considered optimal, but surgery alone is ineffective and rarely leads to a cure.

In the past there have been optimistic reports of long-term remissions in some patients receiving multicomponent chemotherapy (XT) after surgery. In their study, GOG used VAC chemotherapy (XT) to treat 24 patients with pure yolk sac tumors after complete resection and 7 after partial resection. Of the total number of patients (31), it was unsuccessful in 15, including 11 (46%) of 24 cases with complete tumor resection.

15 patients with mixed germ cell tumors containing elements of a yolk sac tumor received chemotherapy (XT) according to the VAC regimen; in 8 (53%) it was ineffective. Subsequently, GOG experts administered 6-9 courses of chemotherapy (XT) according to the VAC regimen to 48 patients with completely resected stage I-III yolk sac tumors. At a median follow-up of 4 years, 35 (73%) patients had no evidence of disease. Recently, 21 patients with similar tumors were treated with bleomycin, etoposide, and cisplatin (BEP). The first 9 patients had no signs of the disease.

The patients received 3 courses of VER-XT within 9 weeks. According to Gershenson et al., 18 (69%) of 26 patients with pure yolk sac tumors after chemotherapy (XT) with the VAC regimen were free of disease. Gallion et al. reported 17 (68%) of 25 patients with stage I disease surviving 2 years or more after treatment with the VAC regimen. Sessa et al. treated 13 patients with yolk sac tumors, 12 of whom underwent unilateral oophorectomy. All received chemotherapy (XT) according to the VBP regimen and lived for at least 20 months. up to 6 years old. Relapses were diagnosed in 3 patients, the treatment of which was completed successfully.

This experience is important because 9 patients were IIb or higher stage of the disease. Chemotherapy (XT) regimens are presented in the table below.

Schwartz et al. at stage I of the disease, the VAC regimen was used, and at stages II-IV, VBP was preferred. Of the 15 patients, 12 survived and have no signs of disease. According to the authors, after normalization of the AFP titer, at least one more course of chemotherapy (XT) is necessary. This position has now become standard in many cancer centers. One relapse was successfully treated with the BEP regimen. In 2 cases of unsuccessful VAC treatment, the VBP regimen also did not save the lives of the patients. GOG experts analyzed the results of using the VBP regimen in stages III and IV disease and in recurrent malignant germ cell tumors, in many cases with known and measurable tumor volume after surgical treatment. For yolk sac tumors, long-term survival was observed in 16 (55%) of 29 patients.

Scheme VBP produced a significant number of durable complete responses, even in patients with prior chemotherapy (XT). However, this scheme causes a large number of side effects. Although second-look laparotomy was included in this protocol, it was not performed on all patients (for various reasons). Smith et al. reported 3 cases of resistance to methotrexate, actinomycin D and cyclophosphamide (MAC), as well as to the VBP regimen; complete responses have been documented in patients following treatment with regimens containing etoposide and cisplatin. All patients had no signs of the disease for 4 years or more. According to Williams, for disseminated germ cell tumors, primarily testicular, the BEP regimen was more effective with less neuromuscular toxicity than VBP.

Williams also reported a GOG study of adjuvant postoperative (XT) BEP in 93 patients with malignant ovarian germ cell tumors: 42 had immature teratomas, 25 had yolk sac tumors, and 24 had mixed germ cell tumors. At the time of publication of the report, 91 of 93 patients had no signs of disease after 3 courses of XT according to the BEP regimen with a median follow-up of 39 months. In one patient after 22 months. after treatment, acute myelomonocytic leukemia developed, in the second one after 69 months. diagnosed with lymphoma.

Dimopoulos reported similar findings to the Hellenic Cooperative Oncology Group. 40 patients with tumors that did not include dysgerminomas received treatment according to the BEP or VBP regimen. With an average follow-up of 39 months. in 5 patients the disease progressed and they died, but of these only 1 patient received VER.

In Japan Fujita observed 41 cases of pure and mixed yolk sac tumors over a long observation period (1965-1992); 21 patients underwent unilateral oophorectomy. More radical surgical interventions did not improve survival. Survival rates did not differ between VAC and VBP. All patients with stage 1 disease who received VAC or PBV after surgery survived with no signs of relapse.

Definition serum AFP- a valuable diagnostic method for yolk sac tumors, it can be considered as an ideal tumor marker. AFP allows you to monitor treatment results, detect metastases and relapses. As noted earlier, many researchers use AFP values as a criterion for determining the number of courses of chemotherapy (CT) required for a particular patient. In many cases, only 3 or 4 cycles of chemotherapy (XT) were needed to achieve remission with long-term survival.

After organ-saving operations and chemotherapy(XT) a significant number of successful pregnancies occurred. However, Curtin reported 2 patients with normal AFP levels but a positive second-look laparotomy, although at present such cases should be considered an exception. According to publications, relapses in retroperitoneal lymph nodes can occur in the absence of intraperitoneal metastases.

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.

T u m o r i s c l e t o c l e d i g a .

A rare neoplasm (about 2% of all testicular tumors) developing from interstitial Leydig cells. The disease is detected in boys over 4 years of age and in men from 30 to 60 years of age. 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.

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

Tumors of this type account for up to 7% of all tumors in children and adolescents. 2-4% - in children under 15 years of age and about 14% in adolescents from 15 to 19 years of age. Teenage boys under 20 are slightly more likely to get the disease than girls - 12 cases versus 11.1 per million. According to some data, the pathological course of pregnancy and maternal smoking increase the risk of germ cell tumors in the child.

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

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

The most common locations of germ cell tumors:

sacrococcygeal region;
ovary;
testicle;
pineal gland;
retroperitoneal space;
mediastinum.

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

Morphological classification of germ cell tumors:

Dysgerminoma (seminoma);
Teratoma mature and immature;
Yolk sac tumor;
Choriocarcinoma;
Embryonic cancer;
Germinoma;
Mixed germ cell tumor.

Diagnostics

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

laboratory tests: general blood test, general urinalysis, biochemical blood test, AFP, coagulogram;
instrumental studies: chest radiography, abdominal ultrasound, ultrasound of the affected area, CT of the chest and abdominal cavity, MRI of the affected area, osteoscintigraphy, myeloscintigraphy;
invasive examinations: puncture, bone marrow trephine biopsy, lumbar puncture (if indicated); tumor biopsy.

Treatment

Treatment for children with germ cell tumors involves removing the tumor and administering chemotherapy. The order of surgery and chemotherapy depends on the location of the tumor. As a rule, damage to the gonads dictates removal of the tumor at the first stage with chemotherapy in the postoperative period. If a CT or MRI scan reveals clear infiltration into the surrounding tissue or metastases, the first therapeutic step will be chemotherapy.

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

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

Forecast

The overall survival rate for germ cell tumors is:

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

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

Yolk sac tumor(embryonic carcinoma of the infantile type; tumor of the endodermal sinus) is rare, mainly in children under 3 years of age, but also occurs in adults, usually in combination with other germ cell tumors. It is found in the testes, ovaries and extragonadal locations. Clinically characterized by rapidly progressive testicular enlargement.

Macroscopically testicle enlarged, the tumor is soft, whitish or yellowish in color with hemorrhages, areas of mucoidization and sometimes the formation of cysts. May spread to the epididymis and spermatic cord.

Microscopically tumor consists of primitive epithelial cells with indistinct boundaries of cubic, prismatic or flattened shape, reminiscent of endothelium. The cytoplasm is light, eosinophilic, often vacuolated, containing varying amounts of glycogen, mucus and lipids. Intra- and extracellular PAS-positive hyaline bodies are found. The nuclei are small, round or slightly elongated, often vacuolated. The cells grow in solid fields and form cords in the form of anastomosing glandular structures of the polyvesicular type. Polyvesicular structures are considered more mature, characterizing differentiation into a primitive gut. There are papillae formed by a thin fibrovascular stroma, covered with two rows of cells - structures reminiscent of a developing yolk sac (Schiller-Duval bodies).

Plots available mesh structure, in which it is difficult to distinguish cytoplasmic vacuoles from vessels anastomosing among themselves. The sharply edematous stroma may contain bizarrely arranged strands of tumor cells. In the stroma there are sometimes cells resembling smooth muscle elements and areas of primitive mesenchyme, which, however, does not provide grounds for the diagnosis of teratoma.
In patients with yolk sac tumor elevated fetoprotein is always determined.

Prognosis in children up to 2 years of age is more favorable than in other age groups (where there is usually a combination of a yolk sac tumor with other germ cell tumors).

Polyembryoma tumor, consisting mainly of embryonic bodies. Embryoid bodies consist of a cylindrical disc and cavity, surrounded by loose mesenchyme, in which tubular structures resembling endoderm and elements of syncytiotrophoblast can be found. The disc consists of one or several layers of large undifferentiated epithelial-like cells; the cavity is lined with flattened epithelial cells and resembles the amniotic cavity. Embryoid bodies resemble a two-week-old embryo. More often, various variants of embryoid bodies are found in the form of nests or layers of cells, partially lying in the cavity, with or without an organoid structure. Polnembryomas in their pure form are extremely rare. Embryoid bodies are usually found in embryonic cancers and teratomas. The prognosis is unfavorable.

Choriocarcinoma(chorionethelioma) is an extremely malignant tumor of the testicles, consisting of cells identical to cyto- and syncytitrophoblast. Often the first clinical symptoms are caused by metastatic damage to the lungs (hemoptysis), brain, and liver. It is found in its “pure” form very rarely, mainly in people aged 20-30 years. Macroscopically, the tumor is often small in size and dark red in color. Microscopically, the only reliable criterion for diagnosis is the close connection of cyto- and syncytitrophoblastic elements. The tumor contains structures resembling villi and consisting of a cytotrophoblast surrounded by a syncytiotrophoblast.

Having one of these components, even with a high level of human chorionic gonadotropin, is not enough to make a diagnosis. Elements of syncytiotrophoblast are found in seminomas, embryonal cancer, teratoma, but only their combination with nitotrophoblast makes it possible to judge choriocarcinoma. Typically, choriocarcinoma is combined with other germ cell tumors (embryonic cancer, teratomas, etc.). Chorionic gonadotropin in the blood serum and urine in these patients is usually high. The prognosis is unfavorable.

Teratoma tumor, usually consisting of several types of tissues that are derivatives of all three germ layers: endoderm, mesoderm, ectoderm. In cases where the tumor consists of derivatives of one germinal tissue (skin, brain), it is regarded as a teratoma. If differentiated tissue (cartilage, glands) is combined with seminoma or embryonal carcinoma, this tissue should be considered as elements of a teratoma.
Teratoma occurs in children and adult men under 30 years of age.

Macroscopically testicle may be of normal size or more often significantly enlarged. The tumor is dense with a lumpy surface, grayish-white in cross section with areas of cartilage or bone (or without them), with cysts of various sizes filled with brownish, gelatinous or mucinous contents.

Mature teratoma consists of well differentiated tissues (cartilage, smooth muscle, brain, etc.). Often these tissues are located in the form of organoid structures, resembling the gastrointestinal tract, respiratory tube, salivary or pancreas, etc. In a simpler form, teratoma contains cysts lined with squamous, respiratory or intestinal epithelium. The cyst wall is formed by mature connective tissue. If the wall of cysts lined with mature epithelium is formed by myxomatous tissue such as primitive mesenchyme, or if the teratoma contains areas of primitive mesenchyme, it should be classified as immature.

Diagnosis of mature teratoma can be diagnosed only after a thorough examination of the entire tumor to exclude immature components and elements of other germ cell tumors. For children, the prognosis is favorable; in adults, despite the visible maturity of the tissues, it is impossible to predict the clinical course of the tumor, since cases of metastasis are known.

All of the above tumors in recent years they have been grouped into the group of “non-seminomas”.
Dermoid cysts, similar to those found in the ovary, are extremely rare in the testicle. They must be distinguished from the group of mature teratomas. It is necessary to distinguish between epidermal cysts, the wall of which is lined with stratified squamous epithelium, but does not contain skin appendages. If epidermal cysts are adjacent to scar or cartilage, they should be classified as teratoma.

Immature teratoma consists of tissues with incomplete differentiation. It can be represented by immature tissues, derivatives of all germ layers. In addition, it may have an organoid structure with the formation of abortive organs, most often these are the neural tube, structures of the gastrointestinal tract and respiratory tract. Along with this, there are elements of mature tissues. In some cases, patients with immature teratoma have a positive reaction to fetoprotein. It should be noted that immature teratoma is rare in children. The prognosis is unfavorable. ,