Abstract

Irappa Madabhavi*, Apurva Patel, Mukesh Choudhary and Asha Anand

Ovarian ependymomas are extremely rare tumors of the ovary with gliomatous differentiation toward ependymal cells that usually arises in the central nervous system. De novo thrombosis of the Internal Jugular Vein is also an extremely rare entity in patients with ovarian malignancy without any previous history of chemotherapy. We present the case of a 67 year old postmenopausal woman who presented to us a swelling in the right side of the neck for 2 months followed by intermittent subacute pelvic pain, lower abdominal distention and weight loss for 1 month. Her coagulation profile, blood chemistry, lipid profile, AFP, Beta-HCG and LDH were within normal limits. Neck Doppler ultrasonography revealed thrombus in the right internal jugular vein without any atherosclerotic plaque or calcification. CT scan of the thorax also showed right sided IJV thrombosis without any evidence of lung metastasis, mediastinal lymphadenopathy or any mass lesions compressing over IJV. CT scan of abdomen showed bilateral ovarian masses. Later patient was subjected to bilateral salpingo-oophorectomy for suspected ovarian cancer. Microscopic examination revealed a highly cellular tumor composed of small cells with hyper chromatic, round-tooval nuclei and scanty cytoplasm with perivascular pseudo rosettes and ependymal rosettes. Diagnosis was confirmed by immunophenotype showing strong positivity to glial fibrillary acidic protein(GFAP). Postoperative adjuvant chemotherapy (including cisplatin, bleomycin and etoposide) was started along with anticoagulants. After 3 cycles of chemotherapy, CT abdomen was done which didn’t show any abnormal feature. Neck Doppler revealed no thrombus in right internal jugular vein. Total 6 courses of chemotherapy were completed. Patient is under surveillance for 3 months. Ovarian cancer is usually epithelial origin in most of the geriatric patients. Thus the diagnosis of ovarian ependymoma is an unusual occurrence. Further IJVT was the first symptom that developed in this patient. 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. An extensive review of literature, did not reveal many cases of ovarian ependymoma. 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”.