Ovarian germ cell tumors is a malignant tumor, presumably developing from primary indifferent gonadal cells. It is usually diagnosed at a young age. It often occurs against the background of hypoplasia of the genitals and general infantilism. In the early stages, it can be accompanied by pain, weakness and dysuria. In the later stages, germination of nearby organs, infection of neoplasms, hyperthermia and general intoxication are observed. The diagnosis is made taking into account complaints, general and gynecological examination data and the results of additional studies. Treatment is surgical removal followed by radiotherapy.
General information
Ovarian germ cell tumors is a malignant neoplasm, presumably developing from primary germ cells. It is the most common germinogenic tumor. It accounts for approximately 20% of ovarian malignancies that occurred before the age of 20, and 0.5-4% of the total number of ovarian tumors. In 75% of cases, it is detected at the age of 10-30 years. In 5-30% of cases, it is diagnosed in patients with signs of infantilism. In 10-15% of observations, it is two-sided. The level of malignancy can vary greatly. Lymphogenic metastasis is characteristic. Hematogenous metastases, as a rule, are detected only in the terminal stage of the disease. The treatment is carried out by specialists in the field of gynecology and oncology.
Causes
Ovarian germ cell tumors develops from primary germ cells. Normally, all germ cells at the time of birth should form primordial follicles. If the follicle formation process is disrupted, such cells remain unchanged in the organ tissue, eventually begin to proliferate uncontrollably and transform into a malignant neoplasm. Patients often suffer from general and genital infantilism. Anomalies of the female genital organs are often found. In the anamnesis, amenorrhea or later menarche may be detected.
Pathanatomy
Disease is a rounded or oval dense lumpy neoplasm. In the initial stages, it is covered with a smooth capsule, and subsequently the capsule and surrounding tissues germinate. It is more often one-sided. As a rule, disease is localized in the area of the organ gate. It is located in the Douglas space (the cavity behind the uterus, bounded by the peritoneum). The size can vary significantly. In advanced cases, the tumor completely replaces the ovarian tissue. This pathology is characterized by local aggressive growth and lymphogenic metastasis. As the tumor progresses, it can germinate the fallopian tube, uterus and other nearby organs. It usually metastasizes to the lymph nodes of the abdominal aorta and the common iliac artery. In advanced cases, distant metastasis is possible, more often to the lungs, liver and bones.
On the incision, the ovarian germ cell tumors is brownish, gray or yellowish with a pink tinge. Diffuse hemorrhages are determined. Microscopy reveals large cells with fairly clear boundaries, light nuclei and light foamy cytoplasm. The number of mitoses may vary. Giant multinucleated cells are often detected. The cells of the ovarian germ cell tumors combine into fields without a stroma or into cells located in a hyalinized or fibrous stroma. Lymphoid cell infiltrates are detected in the stroma.
Symptoms
The clinical picture is nonspecific. The first sign is often pain. Almost half of the patients have dull, dragging or aching pain. In 15% of cases, the pain is acute, resembling the clinical picture of an acute abdomen. In addition, patients with ovarian germ cell tumors may complain of dysuria, disorders of the gastrointestinal tract and menstrual cycle disorders. Weakness and increased fatigue are observed. In the later stages, infection and disintegration are possible, accompanied by hyperthermia, increased ESR and symptoms of general intoxication.
During an external examination, many patients show signs of infantilism. In some patients with ovarian germ cell tumors, symptoms of virilization are revealed. When palpating the abdomen, a tumor-like formation is determined. During the gynecological examination, a dense tumor with a diameter of 5 to 15 centimeters or more is probed, located deep in the vesico-uterine or rectal uterine recess. In the initial stages, the ovarian germ cell tumors may be mobile, as the process progresses, the mobility of the neoplasm decreases. When spreading, several nodes or a conglomerate of tissues in the pelvic region may be palpated.
Diagnostics
The diagnosis is established on the basis of complaints, general and gynecological examination data and the results of instrumental studies. The presence of a large, lumpy, dense tumor in the pelvic region in a young patient, especially in combination with signs of infantilism or masculinization, amenorrhea or late menarche is a reason to suspect ovarian germ cell tumors. Patients are referred for pelvic ultrasound with central Doppler mapping. According to the echogram data, an irregular-shaped echopositive formation with uneven contours is determined. According to the results of dopplerometry, multiple foci of vascularization are detected. The diagnosis is confirmed in the process of histological examination of the removed neoplasm.
The differential diagnosis of ovarian germ cell tumors is carried out with uterine fibromyoma and other ovarian neoplasms. Fibromyoma is usually detected in middle–aged and elderly patients, dysgerminoma – in young patients and adolescent girls. With fibromyoma, as a rule, polymenorrhea or hypermenorrhea are observed, with dysgerminoma – amenorrhea or oligomenorrhea. Differential diagnosis with other neoplasms is of little practical value, since any ovarian tumors are subject to removal.
Treatment
The treatment is operative. The scope of surgical intervention is determined taking into account the prevalence of the process, the age of the patient and her desire to have children. With small ovarian germ cell tumors in women of reproductive age planning childbirth, unilateral adnexectomy is performed. Patients of preclimacteric age and patients who do not want to have children undergo hysterectomy with appendages (pangisterectomy) and omentum removal (omentectomy).
With bilateral ovarian germ cell tumors, capsule germination and involvement of nearby organs in the process, the uterus with appendages is removed in combination with the removal of the omentum and subsequent radiation therapy. Disease has a high sensitivity to radiation. Radiotherapy is successfully used in the postoperative period, in the event of relapses and in the detection of metastases. With disseminated ovarian germ cell tumors, it is possible to use chemotherapy. Usually, patients are prescribed melphalan, cisplatin, etoposide and bleomycin.
Forecast
The forecast is relatively favorable. The five-year survival rate of patients with unilateral local ovarian germ cell tumors after removal of the appendage is about 90%. Cases of successful pregnancy and childbirth are described. Prognostically unfavorable are bilateral dysgerminomas, the spread of the tumor beyond the ovary, the presence of lymphogenic and hematogenic metastases. Opinions regarding survival in disseminated ovarian germ cell tumors vary. Some experts point out that when using combination therapy, the five-year survival rate reaches 85 percent or more. Others note that the degree of malignancy of ovarian germ cell tumors may vary, so the prognosis should be determined with great caution, especially when detecting a tumor in adolescent girls.