Pancreatic PEComa, a not so uncommon neoplasm? Systematic review and therapeutic update

Pancreatic PEComas are extremely rare neoplasms with malignant potential, which mostly affect middle-aged women and are characterized by presenting melanocytic and myogenic markers in immunohistochemical analysis. There are no symptoms or pathognomonic imaging tests, so the diagnosis is established...

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Published inGastroenterología y hepatología Vol. 47; no. 1; p. 93
Main Authors Nogueira Sixto, Manuel, Carracedo Iglesias, Roberto, Estévez Fernández, Sergio, Rodríguez Pereira, Carlos, Sánchez Santos, Raquel
Format Journal Article
LanguageEnglish
Published Spain 01.01.2024
Subjects
Endoscopic Ultrasound-Guided Fine Needle Aspiration
Female
Humans
Middle Aged
Perivascular Epithelioid Cell Neoplasms - diagnosis
Perivascular Epithelioid Cell Neoplasms - surgery
Neoplasm
Perivascular epithelioid cell tumour
PEComa
Surgery
Cirugía
Neoplasia
Pancreas
Páncreas
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Summary:Pancreatic PEComas are extremely rare neoplasms with malignant potential, which mostly affect middle-aged women and are characterized by presenting melanocytic and myogenic markers in immunohistochemical analysis. There are no symptoms or pathognomonic imaging tests, so the diagnosis is established with the analysis of the surgical specimen or the FNA obtained with preoperative endoscopic ultrasound. The mean treatment consists on radical excision, adapting the intervention to the location of the tumor. To date, 34 cases have been described; however, more than 80% of them have been reported in the last decade, which suggests that it is a more frequent pathology than expected. A new case of pancreatic PEComa is reported and a systematic review of the literature is carried out according to the PRISMA guidelines with the aim of divulge this pathology, deepening its knowledge and updating its management.
ISSN:0210-5705
DOI:10.1016/j.gastrohep.2023.05.009