Retroperitoneal paraganglioma presenting with pancytopenia: A rare case with rare manifestation

Abstract

INTRODUCTION: Paragangliomas are tumors that arise from extraadrenal chromaffin cells and most of them are asymptomatic presenting with painless mass. Retroperitoneal paragangliomas are mostly benign with good prognosis; however, they can present with abdominal pain, palpable mass, or hypertensive episodes. Surgical resection is still the main treatment and necessary for histological assessment.

CASE REPORT: A 41 year old female patient presented with 6 months of loss of appetite, weight loss, weakness and breathlessness on exertion.. The patient's initial blood examination showed marked anemia, reduced leukocyte count with neutropenia and lymphopenia and a marked reduction in the platelet count. The patient was admitted for evaluation of her pancytopenia. Magnetic resonance imaging revealed a 8 x 7 x 8 cm sized mass closed to the pancreatic tail invading splenic hilum. A large mass was identified retroperitoneally, closed to the tail of pancreas with a splenic hilum invasion. Total mass resection and splenectomy was performed.

DISCUSSION: Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as for recurrent or metastatic neoplasms. Reactive thrombocytosis is a common cause of thrombocytosis. Splenectomy was found to be one of the main causes of extreme reactive thrombocytosis. Reactive thrombocytosis is a predictable finding after splenectomy and management of the thrombocytosis and prevention of complications should be initiated. (C) 2015 The Authors. Published by Elsevier Ltd.