title: Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature.
abstract: BACKGROUND
  First described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable.
  
  
  METHODS
  A 60-year-old woman presented with abdominal pain. Magnetic resonance imaging (MRI) and computed tomography (CT) scan detected a dishomogeneous egg-shaped 8-cm mass, arising from the pancreatic head, with duodenal compression, without dilation of the Wirsung duct. (18)F-FDG positron-emission tomography (PET)-CT showed a moderate tracer uptake, and the endoscopic ultrasound (US) showed a hypoechoic lesion, arising from the duodenal wall, suspected to be a gastrointestinal stromal tumour (GIST). CEA, CA19-9, NSE, and chromogranin A were normal. At the surgical exploration, a 10-cm mass, adherent to the anterior aspect of the pancreatic head, was found. The lesion was easily separable from the duodenal wall and was totally excised. The frozen intraoperative examination showed a mesenchymal tumour, with spindle-shaped cells, suggesting that a GIST diagnosis was likely. Postoperative course was uneventful. Histology and immunohistochemistry demonstrated a well-differentiated leiomyosarcoma, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC)), with positive smooth muscle actin, desmin, and caldesmon but negative CD117 (c-kit) and S-100. The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.
  
  
  CONCLUSIONS
  In the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported. If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.
authors.affiliation: Pancreatic and Digestive Endocrine Surgical Unit-Department of Surgery, Gastroenterology and Oncology, University of Padua, Via Giustiniani, 2-35128, Padua, Italy. ac.milanetto@email.it.
authors.forename: Anna Caterina
authors.initials: AC
authors.lastname: Milanetto
authors.affiliation: Pancreatic and Digestive Endocrine Surgical Unit-Department of Surgery, Gastroenterology and Oncology, University of Padua, Via Giustiniani, 2-35128, Padua, Italy. valbonalico@hotmail.it.
authors.forename: Valbona
authors.initials: V
authors.lastname: Liço
authors.affiliation: Pathology-Department of Medicine, University of Padua, Via Gabelli, 61-35128, Padua, Italy. stella.blandamura@unipd.it.
authors.forename: Stella
authors.initials: S
authors.lastname: Blandamura
authors.affiliation: Pancreatic and Digestive Endocrine Surgical Unit-Department of Surgery, Gastroenterology and Oncology, University of Padua, Via Giustiniani, 2-35128, Padua, Italy. claudio.pasquali@unipd.it.
authors.forename: Claudio
authors.initials: C
authors.lastname: Pasquali
keywords: Leiomyosarcoma; Mesenchymal tumour; Pancreas; Pancreatic tumour; Sarcoma