TY - JOUR
T1 - Asymptomatic pancreatic perivascular epithelial cell tumor (PEComa) in a male patient
T2 - Report and literature review
AU - Zemet, Roni
AU - Mazeh, Haggi
AU - Neuman, Tzahi
AU - Freund, Herbert R.
AU - Eid, Ahmed
PY - 2011/1
Y1 - 2011/1
N2 - Context Perivascular epithelial cell tumors (PEComas) are a family of rare mesenchymal neoplasms which share cellular, immunohistochemical and ultrastructural characteristics but are found in different visceral and soft tissue sites. PEComas of the pancreas are extremely rare neoplasms. Case report We describe a 49-year-old male who was incidentally diagnosed with a pancreatic mass. Endoscopic ultrasound-guided biopsy suggested a PEComa. An uneventful pylorus-preserving pancreaticoduodenectomy was thus performed. The tumor was a solid well-circumscribed mass in the pancreatic head with dilatation of the main pancreatic duct. Histopathology revealed a well-circumscribed and vascularized neoplasm, measuring 32x27x30 mm, composed of epithelioid smooth muscle cells with clear cytoplasm rich in glycogen. The tumor exhibited immunoreactivity to alphasmooth muscle actin and to melanoma-associated antigen HMB-45. Conclusions Although rare, pancreatic PEComas should be included in the differential diagnosis of a pancreatic mass. Currently, the paucity of cases published makes it impossible to predict the behavior and prognosis of these tumors or to advocate an optimal therapy.
AB - Context Perivascular epithelial cell tumors (PEComas) are a family of rare mesenchymal neoplasms which share cellular, immunohistochemical and ultrastructural characteristics but are found in different visceral and soft tissue sites. PEComas of the pancreas are extremely rare neoplasms. Case report We describe a 49-year-old male who was incidentally diagnosed with a pancreatic mass. Endoscopic ultrasound-guided biopsy suggested a PEComa. An uneventful pylorus-preserving pancreaticoduodenectomy was thus performed. The tumor was a solid well-circumscribed mass in the pancreatic head with dilatation of the main pancreatic duct. Histopathology revealed a well-circumscribed and vascularized neoplasm, measuring 32x27x30 mm, composed of epithelioid smooth muscle cells with clear cytoplasm rich in glycogen. The tumor exhibited immunoreactivity to alphasmooth muscle actin and to melanoma-associated antigen HMB-45. Conclusions Although rare, pancreatic PEComas should be included in the differential diagnosis of a pancreatic mass. Currently, the paucity of cases published makes it impossible to predict the behavior and prognosis of these tumors or to advocate an optimal therapy.
KW - Endosonography
KW - Pancreas
KW - Pancreaticoduodenectomy
KW - Perivascular Epithelioid Cell Neoplasms
UR - http://www.scopus.com/inward/record.url?scp=78651491677&partnerID=8YFLogxK
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C2 - 21206104
AN - SCOPUS:78651491677
SN - 1590-8577
VL - 12
SP - 55
EP - 58
JO - Journal of the Pancreas
JF - Journal of the Pancreas
IS - 1
ER -