Ovarian Germ Cell Tumours | Chris O'Brien Lifehouse

General Considerations

Malignant ovarian germ cell tumours are very uncommon, comprising only 5% of ovarian cancers. Sixty percent of ovarian tumours in patients < 20 are germ cell tumours and 25% of these are malignant. They occur in young women with a median age 20 years. The younger the patient the more likely the tumour is a germ cell tumor and the more likely it is malignant.

Patients present with nonspecific symptoms of a pelvic or abdominal mass, abdominal enlargement or pain. Occasionally they may present with torsion, rupture or hemorrhage or as an unsuspected adnexal mass in pregnancy. Many are asymptomatic. Some, including embryonal and choriocarcinoma have abnormal hormonal manifestations including precocious puberty, abnormal bleeding, amenorrhea, hirsutism, virilisation and isosexual precocity. The majority are early stage at diagnosis.

The histological classification includes (i) dysgerminoma (ii) endodermal sinus tumour (iii) teratoma (iv) embryonal carcinoma (v) polyembryomas and (vi) non-gestational choriocarcinoma.

Dysgerminomas

Are the most common malignant germ cell tumour representing 1-3% of all ovarian cancers, but represent as many as 5-10% of ovarian cancers in patients younger than 20 years of age. They are the most frequent occurring malignancy associated with intersex states, dysgenetic gonads and gonadoblastoma. Up to 20% may be bilateral. They may be associated with elevated serum LDH and are responsive to both chemotherapy and radiation therapy.

Endodermal Sinus Tumours

Are the second most common malignant germ cell tumour. They are a rapidly growing tumours. Schiller-Duval bodies are present in 75% of tumours, and PAS-positive hyaline droplets consisting of AFP and alpha1-antitrypsin are always present. Most secrete AFP.

Teratomas

May be immature (malignant) or mature. In older women malignancy can occur in mature teratomas, commonly as squamous cell carcinomas. Monodermal highly specialized teratomas include (i) struma ovarii and (ii) carcinoid. Struma ovarii are teratomas containing more than 50% thyroid tissue. Rarely is this tissue biologically active. A primary ovarian carcinoid may produce the carcinoid syndrome as venous blood from the ovary drains to the IVC, bypassing the portal circulation and hepatic deactivation. The carcinoid syndrome is due to secretion of serotonin, brady kinin and histamine producing episodic cutaneous flushing, diarrhea, facial cyanosis, evidence of right sided heart failure and bronchospasm.

Immature teratomas are graded, based upon the degree of immaturity and the amount of neuroepithelium present.

Immature elements may undergo spontaneous maturation; however maturation appears to be facilitated by chemotherapy. It is not uncommon to detect nodules of mature tissue (usually glial nodules) at the time of reassessment laparotomy. If the residual nodules have been well sampled and no immature tissue is detected, no additional therapy is needed. This condition known as gliomatosis peritonei. The nodules tend to concentrate in the pelvis, omentum and sites close to the tumor.

Management

Initial surgery should consist of unilateral salpingoophorectomy, peritoneal cytology and meticulous exploration of the abdomen, including assessment of the retroperitoneal lymph nodes. If the contralateral ovary contains a cystic lesion, it should be enucleated. Biopsy of a normal appearing ovary is not indicated if the patient has a nondysgerminomatous malignancy. The role of debulking advanced ovarian germ cell tumours is not defined. As these tumour are very chemosensitive, conservative or fertility sparing surgery is indicated.

Patients with stage IA pure dysgerminomas and stage IA, grade I pure immature teratoma may be managed by surgery alone. All other patients with malignant germ cell tumour require further therapy. For completely resected, stage I disease, 4 cycles of BEP (Bleomycin, Etoposide, Cisplatin) is recommended. During chemotherapy OCP’s should be prescribed to prevent pregnancy and suppress ovarian function to reduce possible ovarian damage. The role of second look laparotomy remains controversial. After completion of therapy, all patients should be followed by serial tumor markers and examination. Most have or will resume normal menstrual function within a few months of completing chemotherapy.

Special Circumstances

Gonadoblastoma (Mixed Germ Cell and Sex Cord Tumours)

A rare tumour of the ovary, characterized by germ cells intimately mixed with indifferent sex cord elements resembling immature granulosa and Sertoli cells. It is the commonest neoplasm arising in dysgenetic gonads. Clinical presentation depends on the nature of the underlying gonadal abnormality. Eighty percent are phenotypic females who are usually virilized and 90% of all patients have a Y chromosome. Primary amenorrhea with or without signs of virilization is common in phenotypic females, as is abnormal genitalia among the phenotypic males. Serum gonadotropin levels are high due to gonadal failure. Its importance, in addition to being almost universally associated with intersex states, is its propensity for producing malignant germ cell tumors. Treatment is bilateral gonad excision as soon as they are discovered. Further treatment is determined by the histological nature of the germ cell.

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