Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature.

Maurizio Zizzo, Lara Ugoletti, David Tumiati, Carolina Castro Ruiz, Stefano Bonacini, Michele Panebianco, Giuliana Sereni, Antonio Manenti, Filippo Lococo, Gabriele Carlinfante, Claudio Pedrazzoli

Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] 2018;18(3):238-245

PMID: 29478828

Full Text on Elsevier Science View on PubMed

Abstract

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.

Address: Department of Oncology and Advanced Technologies, Surgical Oncology Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy; Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy. Electronic address: [email protected].; Department of Oncology and Advanced Technologies, Surgical Oncology Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy.; Department of Oncology and Advanced Technologies, Oncology Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy.; Department of Oncology and Advanced Technologies, Gastroenterology - Digestive Endoscopy Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy.; Department of General Surgery, Azienda Ospedaliero-Universitaria - Policlinico, Del Pozzo Street 71, 41124 Modena, Italy.; Department of Oncology and Advanced Technologies, Thoracic Surgery Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy.; Department of Oncology and Advanced Technologies, Pathology Unit, IRCCS Arcispedale Santa Maria Nuova, Risorgimento Avenue 80, 42123 Reggio Emilia, Italy.

DOI: 10.1016/j.pan.2018.02.007