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Pure Ovarian Non-gestational Choriocarcinoma in a 10-Year Female

  • Paul Paramita,
  • Gupta Shivam

摘要

Introduction

Ovarian choriocarcinoma can be either gestational or non-gestational. Unlike gestational ovarian choriocarcinoma which is considered to be a metastatic disease, non-gestational ovarian choriocarcinoma is considered to be of germ-cell origin and usually found admixed as a component in other germ-cell tumors without any paternal genetic influence. Non-gestational choriocarcinomas are exceedingly uncommon.

Case

A 10-year female presented with sudden-onset abdominal pain and hematuria. CECT showed a relatively well-defined solid-cystic mass-lesion measuring \(\sim \) 8.8 × 7.4 × 6.6 cm in right adnexa with extensive peripheral vascularity. A clinical diagnosis of torsion ovary was considered initially as the reports of radiology and serology were not available and an upfront right oophorectomy with omentectomy was performed. Serum markers were investigated. Beta-HCG was elevated (1222.5 IU/L), while AFP was normal. Histopathology revealed a tumor comprised of admixture of mononucleate-cytotrophoblast, intermediate-trophoblast and multinucleate-syncytiotrophoblastic cells with large areas of hemorrhage and necrosis. No other germ-cell elements were present. Further radiological work up after the initial histopathological diagnosis also revealed metastatic nodules in the lung. The patient was treated with a total six cycles of PEB (Bleomycin etoposide cisplatin). Resection of residual tumor post-3 cycles of chemotherapy did not show any viable malignancy; however, the beta-HCG was still elevated, though showing a declining trend. Also, the lung nodules persisted. Addition of 3 more cycles of chemotherapy normalized the beta-HCG levels with significant decrease in size of the lung nodules. The patient is alive post 6-month follow-up with decrease in size of metastatic lesion and no new complaints.

Conclusion

Though an aggressive and rare disease, a knowledge of this entity along with timely surgical and chemotherapeutic intervention helps in increasing the disease-free survival.