[Primary leiomyosarcoma of the pancreas]

Primary leiomyosarcoma (LMS) of the pancreas is a rare mesenchymal neoplasm (0.1% of pancreatic tumors) with only 35 cases reported in the world literature. We describe a 36th case. This tumor affects adults in the fifth decade of life, especially men. Its diagnosis is difficult and is based on immunohistochemical analyses that reveal smooth muscle cell markers. We report the case of a patient with primary LMS in the head of the pancreas. He underwent a pancreaticoduodenectomy for both diagnosis and treatment after imaging was unable to provide a precise diagnosis. The discussion covers the clinical, diagnostic, immunohistochemical, and therapeutic characteristics of this neoplasm.