TY - JOUR
T1 - Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature
AU - Zizzo, Maurizio
AU - Ugoletti, Lara
AU - Tumiati, David
AU - Castro Ruiz, Carolina
AU - Bonacini, Stefano
AU - Panebianco, Michele
AU - Sereni, Giuliana
AU - Manenti, Antonio
AU - Lococo, Filippo
AU - Carlinfante, Gabriele
AU - Pedrazzoli, Claudio
PY - 2018
Y1 - 2018
N2 - Background: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, with distinctive morphology and expression of myo-melanocytic markers. Current scientific literature reported just 24 cases of pancreatic PEComas. With our systematic review, we aimed at improving our understanding of the disease, focusing on the knowledge gained on epidemiology, etiology, clinical presentation, diagnosis, treatment and prognosis. Methods: Based on the PRISMA guidelines, a systematic research was carried out on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and EBSCO using the search terms: (“perivascular epithelioid cell tumor” OR ″PEComa”) and (“pancreas “OR″ pancreatic”). Results: The 4th-6th decades of life and female sex (86.9%) turned out as the most affected. Pancreatic head was the most involved site (50%), with a single lesion in almost all cases. The diagnosis was only obtained after histopathological examination (70.8%). The clinical presentation was non-specific, abdominal pain being the main symptom (60.9%). At immunohistochemistry, PEComa showed benign epithelioid predominance and a strong positivity for HMB-45, Melan-A, and α-SMA. Surgical resection was performed in almost all cases, while for one patient the multidisciplinary group chose just endoscopic and imaging follow-up, based on the benign nature of the lesion. Conclusions: The biological characteristics of pancreatic PEComa remain an enigma. Its prognosis seems to depend on whether atypical (“worrisome”) histological features are available or not. Surgery turned out as the most appropriate treatment, without reaching any agreement on surgery timing. Further studies on larger population are needed to better understand the biological features of pancreatic PEComa, in order to set up guidelines in the diagnosis, treatment and follow-up.
AB - Background: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, with distinctive morphology and expression of myo-melanocytic markers. Current scientific literature reported just 24 cases of pancreatic PEComas. With our systematic review, we aimed at improving our understanding of the disease, focusing on the knowledge gained on epidemiology, etiology, clinical presentation, diagnosis, treatment and prognosis. Methods: Based on the PRISMA guidelines, a systematic research was carried out on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and EBSCO using the search terms: (“perivascular epithelioid cell tumor” OR ″PEComa”) and (“pancreas “OR″ pancreatic”). Results: The 4th-6th decades of life and female sex (86.9%) turned out as the most affected. Pancreatic head was the most involved site (50%), with a single lesion in almost all cases. The diagnosis was only obtained after histopathological examination (70.8%). The clinical presentation was non-specific, abdominal pain being the main symptom (60.9%). At immunohistochemistry, PEComa showed benign epithelioid predominance and a strong positivity for HMB-45, Melan-A, and α-SMA. Surgical resection was performed in almost all cases, while for one patient the multidisciplinary group chose just endoscopic and imaging follow-up, based on the benign nature of the lesion. Conclusions: The biological characteristics of pancreatic PEComa remain an enigma. Its prognosis seems to depend on whether atypical (“worrisome”) histological features are available or not. Surgery turned out as the most appropriate treatment, without reaching any agreement on surgery timing. Further studies on larger population are needed to better understand the biological features of pancreatic PEComa, in order to set up guidelines in the diagnosis, treatment and follow-up.
KW - Neoplasm
KW - PEComa
KW - Pancreas
KW - Perivascular epithelioid cell
KW - Surgery
KW - Neoplasm
KW - PEComa
KW - Pancreas
KW - Perivascular epithelioid cell
KW - Surgery
UR - http://hdl.handle.net/10807/151948
U2 - 10.1016/j.pan.2018.02.007
DO - 10.1016/j.pan.2018.02.007
M3 - Article
SN - 1424-3903
VL - 18
SP - 238
EP - 245
JO - Pancreatology
JF - Pancreatology
ER -