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Ovarian choriocarcinoma is a rare sub type of ovarian germ cell tumor.
They account for less than 1% of ovarian tumors.
In pre-menarchal patients, the tumors manifest with iso-sexual precocity (approximately 50% of cases) and other symptoms associated with excessive hormone production.
Choriocarcinoma arising in association with the ovary can occur in a number of situations. The tumors may occur during or outside of pregnancy.
Primary ovarian choriocarcinoma (non gestational ovarian choriocarcinoma) arises presumably from a germ cell and is extremely rare. These may be mixed with other germ cell tumors like immature teratoma, dysgerminoma or rarely occur in a pure form - pure primary choriocarcinoma of the ovary.
The tumors are formed from placental trophoblastic elements. While most gestational choriocarcinomas show hematogenous spread, the non gestational choriocarcinoma tends to follow the lymphatic system.
Gestation and Non-gestational choriocarcinoma is histologically identical. The only way to distinguish an ovarian gestational choriocarcinoma (as in gestational choriocarcinoma arising from prior ectopic pregnancy within the ovary) and a non-gestation ovarian choriocarcinoma is by DNA analysis, looking for paternal elements or via history (as in prepubertal/not sexually active) - it should be noted that given how rare non-gestational choriocarcinoma is, this evidence exists in the form of case studies.
Serum β-HCG levels are often elevated
On imaging, choriocarcinomas appear as vascular solid tumors with cystic, hemorrhagic, and necrotic areas.
The staging depend on the original site of the tumor
for metastatic gestional uterine choriocarcinoma - standard choriocarcinoma staging applies.
from primary non gestational choriocarcinoma - staging ovarian cancer staging applies 6
Treatment and prognosis
As with choriocarcinoma in general, the tumors are capable of widespread metastases.
Choriocarcinoma is however extremely sensitive to chemotherapy, which is the mainstay of treatment. Since the disease is associated with a high incidence of metastasis and recurrence, the recommended management of primary extrauterine choriocarcinoma is often complete surgical resection followed by chemotherapy