Primary pancreatic pleomorphic leiomyosarcoma.

Primary pancreatic leiomyosarcoma is a rare mesenchymal tumour that is believed to arise from the walls of the pancreatic blood vessels or the pancreatic duct. A 56-year-old female was referred with epigastric pain and abdominal mass. Preoperative computed tomography showed a large soft tissue mass in the pancreatic body and tail. Fine needle aspiration biopsy indicated a spindle cell type tumour. The patient received distal pancreatectomy with no adjuvant treatment. Histology revealed a pleomorphic spindle cell neoplasm with an immunoprofile suggestive of smooth muscle origin. The absence of other lesions in the body was consistent with the diagnosis of primary pleomorphic leiomyosarcoma. The patient was well and tumour-free 14 months after surgery. Detailed immunohistochemical analyses are necessary in the diagnosis of this highly malignant tumour. Radical resection offers the only chance of long-term survival.