Paraneoplastic Internal Jugular Vein Thrombosis Leading to Diagnosis of Bilateral Ovarian Ependymoma

Abstract

Ovarian ependymomas are extremely rare tumors of the ovary. We present a case of a 67-year-old lady presented to us with swelling in the right side of neck for 2 months followed by pelvic pain, lower abdominal distention, and weight loss for 1 month. Her coagulation profile, blood chemistry, lipid profile, and tumor markers were within normal limits. Neck Doppler ultrasonography revealed thrombus in the right internal jugular vein and CT scan of the abdomen showed bilateral ovarian masses. Patient was subjected to debulking surgery for suspected ovarian cancer and microscopy revealed a highly cellular tumor composed of small cells with hyperchromatic, round-to-oval nuclei with scanty cytoplasm, and perivascular pseudorosettes. Diagnosis was confirmed by immunophenotype showing strong positivity to glial fibrillary acidic protein, estrogen receptors, and progesterone receptors. Patient was successfully managed with anticoagulants, adjuvant chemotherapy with BEP regimen, and letrozole. After ruling out other common conditions for thrombosis in this age group, this seems to be a paraneoplastic presentation of ovarian malignancy that preceded the diagnosis of ependymoma by 2 months. To the best of our knowledge this is the first case report in the world literature as “paraneoplastic internal jugular vein thrombosis leading to diagnosis of bilateral ovarian ependymoma.”

Primary pure ependymoma: a rare entity of the ovary

“…In previous reports most patients with early stage received operation only [3,5,8] while patients with advanced stage administrated with adjuvan radiation and/or chemotherapy after cytoreductive surgery [1,3,6,7]. Patients with early stage have longer overall survival with less recurrence rates, so stage seems to be the most important prognostic parameter of survival.…”

“…Ependymomas are an infrequent glial tumors that usually originate from the central nervous system, but they can occasionally occur in various extra-axial regions [1,2]. Pure ovarian ependymoma, classified as differentiated glioma, was first described by Kleinman et al in 1984 [3] and only a few cases have been reported since then.…”

Internal jugular vein thrombosis presenting as paraneoplastic syndrome in benign cystic teratoma of ovary: a case report

“…Appropriate recognition of the syndrome is important because VTE often requires careful medical surveillance and management. 9 Internal jugular vein thrombosis is a rare vascular disease that can be overlooked or misdiagnosed. 10 It most commonly occurs in persons with intravenous drug abuse or patients with prolonged central venous catheterization due to iatrogenic trauma.…”