Published online Dec 14, 2013. doi: 10.3748/wjg.v19.i46.8793
Revised: October 9, 2013
Accepted: October 17, 2013
Published online: December 14, 2013
Desmoid tumors (DTs) are nonmetastatic, locally aggressive neoplasms with a high rate of postoperative recurrence. Pancreatic DTs are especially rare; only a few cases have been reported to date. This paper describes a case of a sporadic cystic DT of the pancreas managed successfully with central pancreatectomy, with no signs of recurrence 40 mo after surgery. According to the literature, this is the first reported case in China of a pancreatic DT presenting as a solid cystic lesion, as well as the first pancreatic DT managed with central pancreatectomy and pancreaticogastrostomy. We report the case for its rarity and emphasize disease management by concerted application of clinical, pathological, radiological and immunohistochemical analyses.
Core tip: Desmoid tumors (DTs) are rare, representing approximately 0.03% of all tumors and 3% of soft tissues tumors. They are nonmetastatic and locally aggressive, with a high local recurrence rate. The pancreas is an extremely rare location for DTs. Moreover, pancreatic desmoids resembling solid cystic tumors are the rarest form of DTs. We report a 17-year-old patient presenting with a sporadic cystic DT of the pancreas, and subsequent disease management with central pancreatectomy. We report the case for its rarity and emphasize disease management by concerted application of clinical, pathological, radiological and immunohistochemical analyses. Associated English-language literature is also reviewed and summarized.