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

Int J Surg Case Rep 2015 28;14:77-9. Epub 2015 Jul 28.

Tepecik Education and Research Hospital, Department of General Surgery, İzmir, Turkey.

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 8×7×8cm 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.

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http://dx.doi.org/10.1016/j.ijscr.2015.07.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4573608PMC
September 2015
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