Primary Pancreatic Sarcoma

Primary pancreatic sarcomas are extremely rare entities, accounting for less than 0.1% of all pancreatic neoplasms1. They originate from the mesenchymal tissue of pancreatic support, and the leiomyosarcoma subtype is the most frequently reported2. This neoplasia usually presents poor prognosis due to late diagnosis and accelerated growth relative to other pancreatic neoplasia1,3. The pancreatic head is the most commonly involved site, followed by the tail and body, and it occurs more frequently in younger individuals3. Clinically, patients may present with weight loss, palpable abdominal mass, epigastric pain, nausea and vomiting, similar to other pancreatic diseases, thus being unspecific to sarcomas1-3. On CT scanning, the findings include a bulky, heterogeneous mass and, with peripheral enhancement after contrast injection, pseudocystic masses are also described. CSV is an exclusive marker of sarcoma regardless of its tissue origin4. The prognosis is influenced by the patient’s age, tumor size, the presence of tumor necrosis, and vascular invasion. The curative treatment is surgical – extensive surgical resection should be advocated, even when morphologic results show a low-grade lesion2. The tumor is likely to metastasize to the liver but not to regional lymph nodes. The role of chemot