UNVEILING THE UNEXPECTED

A RARE PARAOVARIAN PARAGANGLIOMA MASQUERADING AS AN ADNEXAL MASS

Abstract

INTRODUCTION/BACKGROUND
Paragangliomas are rare neuroendocrine tumors that arise from extra-adrenal paraganglionic tissue, typically associated with the autonomic nervous system. While they are most commonly found in the adrenal medulla (as pheochromocytomas) or along the sympathetic and parasympathetic chains, their occurrence in the paraovarian region is extremely rare.

CASE
We present the case of a 42-year-old female with a history of left external iliac and common femoral vein thrombosis, who presented with progressive abdominal distension over the past six months. She was normotensive and exhibited no constitutional symptoms, features of catecholamine excess, or compressive symptoms. Blood investigations revealed an elevated CA125 level while other tumor markers were within the normal range. A Computed Tomography (CT) scan of the thorax, abdomen, and pelvis revealed a large intra-abdominal cystic mass measuring 15.2 × 20.8 × 24.5 cm, likely originating from the left ovary with significant mass effect.

The patient underwent an extrafascial hysterectomy with bilateral salpingo-oophorectomy (EHBSO), left pelvic lymph node dissection (PLND), omentectomy, appendicectomy, and adhesiolysis. Histopathological examination of the left ovarian tumor and fallopian tube favored a diagnosis of extra-adrenal paraganglioma as the immunohistochemical staining was positive for S-100, synaptophysin, and chromogranin. Retrospectively, the tumor was likely a non-functioning paraganglioma, as the patient underwent surgery without complications.

CONCLUSION
Paraovarian paraganglioma is an exceptionally rare entity that presents significant diagnostic challenges due to its atypical location and non-specific clinical features. This case highlights the importance of considering paraganglioma in the differential diagnosis of adnexal masses as the perioperative management may differ.