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

Epidemiology

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

Histogenesis

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

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

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

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

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

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

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

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

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

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

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

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

Clinical picture

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

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

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

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

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

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

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

Diagnostics

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

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

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

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

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

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

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

Staging.

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

It should be noted that 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).

Treatment.

Operative method of treatment.

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

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

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

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

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

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

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

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

Radiation therapy

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

Chemotherapy

The leading role in the treatment of germ cell tumors belongs to chemotherapy. Many chemotherapy drugs are effective in this pathology. For a long time, polychemotherapy with three cytostatics was widely used: vincristine, actinomycin "D" and cyclophosphamide. However, in recent years, preference has been given to other drugs, on the one hand, new and more effective, on the other hand, having 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.

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

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

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

The patients received 3 courses VER-XT within 9 weeks. According to Gershenson et al., 18 (69%) of 26 patients with clear yolk sac tumors after VAC chemotherapy (XT) showed no signs of disease. Gallion et al. reported 17 (68%) of 25 patients with stage I disease who survived 2 years or more after VAC treatment. 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 20 months. up to 6 years old. 3 patients were diagnosed with relapses, the treatment of which was completed successfully.

This experience is important because 9 patients were IIb or higher stage of the disease. Chemotherapy regimens (XT) 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 showed no signs of illness. According to the authors, after the normalization of the AFP titer, at least one more course of chemotherapy (XT) is necessary. Now this position has become the standard in many cancer centers. One relapse was successfully treated with the PEP regimen. In 2 cases of unsuccessful treatment of VAC, the VBP regimen also did not save the lives of patients. GOG experts analyzed the results of the VBP regimen in stage III and IV disease and in recurrent malignant germ cell tumors, in many cases with a 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 gave a significant number of persistent complete responses, even in patients after previous 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 in 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 treated with regimens containing etoposide and cisplatin. All patients had no signs of the disease for 4 years or more. According to Williams, in disseminated germ cell tumors, primarily testicular, the BEP regimen was more effective with less neuromuscular toxicity than VBP.

Williams also reported on the 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 were disease-free after 3 courses of XT on the BEP regimen with a median follow-up of 39 months. One patient after 22 months after treatment, acute myelomonocytic leukemia developed, the second after 69 months. diagnosed with lymphoma.

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

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

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

After organ-preserving operations and chemotherapy(XT) had a significant number of successful pregnancies. However, Curtin reported 2 patients with normal AFP levels but a positive second-look laparotomy, although these cases should now be considered exceptions. According to publications, relapses in the retroperitoneal lymph nodes can also occur in the absence of intraperitoneal metastases.

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

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

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

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

Mixed germ cell tumors.

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

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

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

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

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

Tumors from stromal cells and seminiferous tubules.

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

Tumor from Leidig's cells.

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

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

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

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

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

The most common localizations of germ cell tumors:

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

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

Morphological classification of germ cell tumors:

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

Diagnostics

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

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

Treatment

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

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

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

Forecast

Overall survival for germ cell tumors is:

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

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

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

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

Microscopically tumor consists of primitive epithelial cells with indistinct borders of a cubic, prismatic or flattened shape, resembling an endothelium. The cytoplasm is light, eosinophilic, often vacuolated, contains varying amounts of glycogen, mucus, and lipids. There are intra- and extracellular PAS-positive hyaline bodies. The nuclei are small, rounded or slightly elongated, often vacuolated. Cells grow in solid fields, form strands 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 resembling a developing yolk sac (Schiller-Duval bodies).

Plots available mesh structure in which it is difficult to distinguish between cytoplasmic vacuoles and anastomosing vessels. In a sharply edematous stroma, bizarrely located strands of tumor cells may be located. In the stroma, cells resembling smooth muscle elements and areas of primitive mesenchyme are sometimes found, which, however, does not give grounds for the diagnosis of teratoma.
In patients with yolk sac tumor always determine the increased fetoprotein.

Prognosis in children up to 2 years 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 disc and a cylindrical cavity surrounded by loose mesenchyme, which may contain tubular structures resembling the endoderm and elements of syncytiotrophoblast. The disk consists of one or more 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. Pure embryomas are extremely rare. Typically, embryoid bodies are found in embryonic cancers and teratomas. The prognosis is unfavorable.

Choriocarcinoma(Chorioneithelioma) is an extremely malignant tumor of the testicles, consisting of cells identical to cyto- and syncytitrophoblast. Often the first clinical symptoms are due to metastatic lesions of the lungs (hemoptysis), brain, liver. It occurs in a "pure" form very rarely, mainly in people aged 20-30 years. Macroscopically, the tumor is often small, dark red in color. Microscopically, the only reliable criterion for the diagnosis is the close relationship of cyto- and syncytitrophoblastic elements. In the tumor there are structures resembling villi and consisting of cytotrophoblast surrounded by syncytiotrophoblast.

Having one of these components, even with a high content of human chorionic gonadotropin, is not enough to establish a diagnosis. Elements of syncytiotrophoblast are found in seminomas, embryonic cancer, teratoma, but only their combination with nitotrophoblast makes it possible to judge choriocarcinoma. Usually, choriocarcinoma is combined with other germ cell tumors (embryonic cancer, teratomas, etc.). The chorionic gonadotropin in the blood serum and urine of 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 embryonic cancer, this tissue should be considered as elements of a teratoma.
Teratoma occurs in children and adult men under 30 years of age.

Macroscopic testicle may be of normal size or more often greatly enlarged. The tumor is dense with a bumpy surface, grayish-white in 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 muscles, 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 the cysts lined with mature epithelium is formed by myxomatous tissue of the primitive mesenchymal type, or if there are areas of primitive mesenchyme in the teratoma, it should qualify as immature.

Diagnosis of mature teratoma can be set 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 apparent 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 are united in the group of "nonseminomas".
Dermoid cysts, similar to those found in the ovary, are extremely rare in the testis. They must be distinguished from the group of mature teratomas. Epidermal cysts should be distinguished, the wall of which is lined with stratified squamous epithelium, but does not contain skin appendages. If epidermal cysts are adjacent to a scar or cartilage, they should be classified as a teratoma.

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