title: Primary Leiomyosarcoma of the Pancreas-a Case Report and a Comprehensive Review.
abstract: OBJECTIVE
  Primary mesenchymal tumors of the pancreas are rare, with leiomyosarcomas the most encountered entities among the pancreatic sarcomas. With few exceptions, single case reports published over the last six decades constitute the entire scientific literature on this topic. Thus, evidence regarding clinical decision-making is scant.
  
  
  METHODS
  Based on a case report and an extensive literature search in PubMed, we discuss the clinical aspects and current management of this rare malignancy.
  
  
  RESULTS
  We identified only two papers with more than a single case presentation; these institutional patient series were limited to five and nine patients. Additionally, a few papers sought to summarize the individual case reports published in the English and/or Chinese language. The clinical presentation is rather non-specific. Moreover, modern imaging modalities are insufficiently accurate to diagnose leiomyosarcoma of the pancreas. Treatment goals include a complete resection with free margins. Proper morphologic examination using immunohistochemistry and the application of a grading system are clinically important for prognostication. The efficacy of adjuvant treatments has not been established.
  
  
  CONCLUSIONS
  Primary pancreatic leiomyosarcoma is extremely rare, and the scientific literature is primarily based on single case reports. Conclusions on management and prognosis should be drawn with caution. A multidisciplinary team consultation is warranted to discuss a thorough individual treatment plan based on the available scientific literature, despite its low evidence level.
authors.affiliation: Department of Gastrointestinal Surgery, Stavanger University Hospital, POB 8100, N-4068, Stavanger, Norway. jonarne.soreide@uib.no.|Department of Clinical Medicine, University of Bergen, Bergen, Norway. jonarne.soreide@uib.no.
authors.forename: Jon Arne
authors.identifier: http://orcid.org/0000-0001-7572-1089
authors.initials: JA
authors.lastname: Søreide
authors.affiliation: Department of Pathology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: Erling Sandøy
authors.initials: ES
authors.lastname: Undersrud
authors.affiliation: Department of Radiology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: Mohammed S S
authors.initials: MS
authors.lastname: Al-Saiddi
authors.affiliation: Department of Gastrointestinal Surgery, Stavanger University Hospital, POB 8100, N-4068, Stavanger, Norway.
authors.forename: Tore
authors.initials: T
authors.lastname: Tholfsen
authors.affiliation: Department of Gastrointestinal Surgery, Stavanger University Hospital, POB 8100, N-4068, Stavanger, Norway.|Department of Clinical Medicine, University of Bergen, Bergen, Norway.
authors.forename: Kjetil
authors.initials: K
authors.lastname: Søreide
mesh_terms: D006801:Humans; D007890:Leiomyosarcoma / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000473:pathology; D011379:Prognosis
keywords: Leiomyosarcoma; Mesenchymal tumor; Pancreas; Review