Sclerosing Extramedullary Hematopoietic Tumor Presenting with Gross Ascites and Peritoneal Nodules: A Diagnostic Challenge on Cytology

Introduction: Sclerosing extramedullary hematopoietic tumor (SEMHT) is an uncommon lesion seen in association with chronic myeloproliferative disorders especially idiopathic myelofibrosis. We present a case of SEMHT in an elderly female, presenting with ascites.

Case presentation: A 50-year old female, apparently asymptomatic one month back, presented with abdominal distension, loss of appetite and early satiety. Ultrasound abdomen revealed altered liver architecture with ascites. Ascitic fluid analysis was suspicious of malignancy. Contrast-enhanced computed tomography (CECT) abdomen showed multiple thickened nodules in omentum and peritoneum. CA125 levels were elevated and clinical diagnosis of metastatic peritoneal nodules with ovarian primary was made. However, biopsy of these lesions was suggestive of a mesenchymal neoplasm. In view of peripheral leucoerythroblastic picture, bone marrow examination was done which showed primary myelofibrosis. Retrospective review of clinical history revealed that she underwent splenectomy four years back for unknown cause. The peritoneal nodules were finally reported as SEMHT in a case of primary myelofibrosis, status post splenectomy.

Discussion: Idiopathic myelofibrosis is characterized by leukoerythroblastic picture, marrow fibrosis, hepatosplenomegaly with evidence of extramedullary hematopoiesis (EMH) in spleen in most of the cases. In patients with splenectomy, EMH at other sites especially peritoneum and omentum should always be suspected, as in this case.

Conclusion: SEMHT, though rare should be always suspected in known cases of myeloproliferative disorders especially myelofibrosis.