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.

Highlights

  • Ovarian choriocarcinoma is an extremely rare form of ovarian cancer. It can be broadly classified into two variants, gestational ovarian choriocarcinoma (GOC) and non-gestational ovarian choriocarcinoma (NGOC)

  • NGOCs often occur in children and young adults, arising from midline structures that form during embryogenesis or primordial germ cells in the gonads after birth and demonstrate trophoblastic differentiation [7,10,11]

  • NGOCs are characterized by rapid growth and a relatively poor prognosis; overall survival of International Federation of Gynecology and Obstetrics (FIGO) stage I, II, and III disease is 100% over 3 years, with the survival rate of FIGO IV disease dropping to just 25% at 3 years

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Summary

Introduction

Ovarian choriocarcinoma is an extremely rare form of ovarian cancer. It can be broadly classified into two variants, gestational ovarian choriocarcinoma (GOC) and non-gestational ovarian choriocarcinoma (NGOC). NGOC are further subdivided into mixed, which contain other germ cell components, and pure subtypes, which contain only choriocarcinoma. The incidence of GOC is 1:369,000,000 whereas the incidence of NGOC accounts for just 0.6% of malignant ovarian germ cell tumors [1,2,3,4,5]. GOC is a form of gestational choriocarcinoma and related to a patient’s previous pregnancy history and may exist concurrently with a well-developed corpus luteum, with cure rates approaching 90% with single agent chemotherapy, typically methotrexate [6]. NGOCs often occur in children and young adults, arising from midline structures that form during embryogenesis or primordial germ cells in the gonads after birth and demonstrate trophoblastic differentiation [7,10,11]. We will explore the background, presentation, and described treatment protocols of NGOC

Background
Presentation and Diagnosis
Clinical
Laboratory
Genetic Testing
Staging
Treatment
Surgery
Fertility-Sparing Surgery
Chemotherapy
Radiation
Choriocarcinoma Syndrome
Follow Up
Recommendations
Findings
Conclusions
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