title: Desmoid tumor of the pancreas: a case report.
abstract: BACKGROUND
  Desmoid tumor is a rare, benign, usually asymptomatic fibromatous lesion. The etiology is unknown and the diagnosis is based on histopathological examination. The treatment is complete resection of the tumor. Pancreatic desmoid tumor is extremely rare. In the literature there have been only 11 cases described, most of them as solid or solid-cystic masses. We report the case of a patient with an isolated cystic pancreatic desmoid tumor that is, to the best of our knowledge, the second reported case.
  
  
  METHODS
  A 13-year old Caucasian boy presented with recurrent pain of two months' duration in the left hypochondrium of his abdomen. An ultrasound examination and computed tomography scan revealed the presence of a cystic mass located in his splenic hilum, tightly adjacent to the pancreatic tail. A splenic cyst was suspected. Operative findings showed a 10 x 10 cm cystic mass tightly connected to the pancreatic tail and left colonic flexure, adherent to the spleen, splenic vein and artery. Distal splenopancreatectomy with en bloc resection of the left colonic flexure was performed. Histological analysis confirmed that the resection was complete. The mass had infiltrated the pancreatic parenchyma. All tumor cells were positive for anti-beta-catenin staining characteristic for desmoid tumor. No abnormalities in the spleen and colon were found.
  
  
  CONCLUSIONS
  Isolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, especially because of uncharacteristic symptoms and radiological findings, as in our patient. This case report should be of interest not only to surgeons, as the treatment of choice is radical resection, but also gastroenterologists, considering it is in close relation with familial adenomatous polyposis, and oncologists as the reason for differentiation with other pancreatic tumors.
authors.affiliation: Department of Pediatric Surgery, Medical University of Warsaw, Marszałkowska 24, Warsaw, 00-576, Poland. zslowikmoczydlowska@gmail.com.
authors.forename: Żaneta
authors.initials: Ż
authors.lastname: Słowik-Moczydłowska
authors.affiliation: Department of Pediatric Surgery, Medical University of Warsaw, Marszałkowska 24, Warsaw, 00-576, Poland. mikrochirurg@wp.pl.
authors.forename: Robert
authors.initials: R
authors.lastname: Rogulski
authors.affiliation: Department of Pediatric Surgery, Medical University of Warsaw, Marszałkowska 24, Warsaw, 00-576, Poland. chirurgia@litewska.edu.pl.
authors.forename: Anna
authors.initials: A
authors.lastname: Piotrowska
authors.affiliation: Department of Pathology, Medical University of Warsaw, Marszałkowska 24, Warsaw, 00-576, Poland. chirurgia@litewska.edu.pl.
authors.forename: Jadwiga
authors.initials: J
authors.lastname: Małdyk
authors.affiliation: Department of Pathology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, Warsaw, 04-730, Poland. zaklad.patologii@ipczd.pl.
authors.forename: Przemysław
authors.initials: P
authors.lastname: Kluge
authors.affiliation: Department of Pediatric Surgery, Medical University of Warsaw, Marszałkowska 24, Warsaw, 00-576, Poland. andrzej.kaminski@litewska.edu.pl.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kamiński
mesh_terms: D000293:Adolescent; D003937:Diagnosis, Differential; D018222:Desmoid Tumors / Q000175:diagnosis*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010179:Pancreas / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed