Non-Gestational Ovarian Choriocarcinoma: A Rare Ovarian Cancer Subtype

Abstract

Non-Gestational Ovarian Choriocarcinoma (NGOC) is an extremely rare ovarian tumor, with an incidence of less than 0.6% of malignant ovarian germ cell tumors. Its close pathologic resemblance to Gestational Ovarian Choriocarcinoma (GOC), however, requires special attention as the treatments differ greatly. NGOC typically affects patients in late adolescence or early reproductive years. As a result, NGOCs are often misdiagnosed as ectopic pregnancies due to their common presentation of bleeding, abdominal pain, adnexal mass, and positive serum beta-HCG. On pathologic examination, the tumor is indistinguishable from GOC, and only after review of tissue for paternal genetic components can the diagnosis of NGOC be made. Imaging studies often show highly vascular lesions with further investigation with computer topography (CT) sometimes showing metastatic lesions in the lungs, pelvis, vagina, and liver. These lesions are often hemorrhagic and can lead to catastrophic bleeding. Treatment is vastly different from GOC; NGOC requires treatment with both surgical resection and chemotherapy, with Bleomycin, Etoposide, and Cisplatin (BEP) being the most used regimen. With correct diagnosis and treatment, patients can often receive fertility sparing treatment with long term survival.

Choriocarcinoma complicated with intra-abdominal and intrapleural hemorrhage in pregnancy – case report

“…It is unrelated to pregnancy and was reported to have primary sites in the ovaries, lungs, cervix, endometrium, breast, bladder, and gastrointestinal tract ( 9 ). Due to the similarities in clinical and histological features, the differentiation between these two types can only be achieved by genetic testing which identifies the presence of paternal DNA in gestational choriocarcinoma ( 12 ). In the case of our patient, it remains unclear whether choriocarcinoma derived from her previous pregnancy.…”

Primary ovarian choriocarcinoma occurring in a postmenopausal woman: A case report

“…Previous reproductive history plays an important role in distinguishing NGOC from OC. Cronin et al [ 5 ] have suggested that a diagnosis of NGOC is supported by the lack of sexual intercourse or pregnancy in the history. However, this postmenopausal patient had experienced a normal vaginal delivery and four abortions.…”

“…The level of β-hCG can help exclude the possibility of an ectopic pregnancy, in which the level is rarely higher than 100000 mIU/mL. In contrast, the β-hCG level in NGOC is commonly 10–100 times higher[ 5 ]. In our report, the postmenopausal woman presented with vaginal bleeding, an abdominal mass, and a sharply elevated serum β-hCG level at 214405.5 mIU/mL.…”

“…Compared to GOCs, NGOCs are less sensitive to chemotherapy[ 5 ] and have worse prognosis. Because of the small number of cases, there is no standard therapy currently available.…”

An unusual case of metastatic trophoblastic neoplasm presenting with diffuse cystic lung disease and pulmonary artery pseudoaneurysms in a teenager

“…5 Non-gestational trophoblastic tumors are extremely rare and unrelated to pregnancy, and DNA analysis demonstrates the absence of any paternal genes. 6 It has been hypothesized that the retained primordial germ cells may migrate abnormally during embryonic development into the different locations and develop into nongestational trophoblastic tumors. 7 Most of the GTDs are associated with gestational events and occur in the female genital tract.…”