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

Research output: Contribution to journalReview article

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)238-245
Number of pages8
JournalPancreatology
Volume18
Issue number3
DOIs
Publication statusPublished - Apr 1 2018

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Perivascular Epithelioid Cell Neoplasms
MART-1 Antigen
Guidelines
Literature
PubMed
MEDLINE
Abdominal Pain
Pancreas
Epidemiology
Therapeutics
Immunohistochemistry

Keywords

  • Neoplasm
  • Pancreas
  • PEComa
  • Perivascular epithelioid cell
  • Surgery

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Endocrinology

Cite this

Primary pancreatic perivascular epithelioid cell tumor (PEComa) : A surgical enigma. A systematic review of the literature. / Zizzo, Maurizio; Ugoletti, Lara; Tumiati, David; Castro Ruiz, Carolina; Bonacini, Stefano; Panebianco, Michele; Sereni, Giuliana; Manenti, Antonio; Lococo, Filippo; Carlinfante, Gabriele; Pedrazzoli, Claudio.

In: Pancreatology, Vol. 18, No. 3, 01.04.2018, p. 238-245.

Research output: Contribution to journalReview article

Zizzo, Maurizio ; Ugoletti, Lara ; Tumiati, David ; Castro Ruiz, Carolina ; Bonacini, Stefano ; Panebianco, Michele ; Sereni, Giuliana ; Manenti, Antonio ; Lococo, Filippo ; Carlinfante, Gabriele ; Pedrazzoli, Claudio. / Primary pancreatic perivascular epithelioid cell tumor (PEComa) : A surgical enigma. A systematic review of the literature. In: Pancreatology. 2018 ; Vol. 18, No. 3. pp. 238-245.
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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.",
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T2 - 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

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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.

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KW - Pancreas

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KW - Perivascular epithelioid cell

KW - Surgery

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