First pancreatic perivascular epithelioid cell tumor (PEComa) treated by mTOR inhibitor.
Adolescent
Humans
Magnetic Resonance Imaging
Male
Pancreas
/ pathology
Pancreatic Neoplasms
/ diagnostic imaging
Perivascular Epithelioid Cell Neoplasms
/ diagnostic imaging
Signal Transduction
/ drug effects
Sirolimus
/ therapeutic use
TOR Serine-Threonine Kinases
/ antagonists & inhibitors
Tomography, X-Ray Computed
Treatment Outcome
Mesenchymal tumor
Pancreatic cancer
Treatment
Journal
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
ISSN: 1424-3911
Titre abrégé: Pancreatology
Pays: Switzerland
ID NLM: 100966936
Informations de publication
Date de publication:
Jun 2019
Jun 2019
Historique:
received:
11
03
2019
revised:
22
04
2019
accepted:
17
05
2019
pubmed:
28
5
2019
medline:
31
12
2019
entrez:
28
5
2019
Statut:
ppublish
Résumé
Perivascular epithelioid cell tumor, an extremely rare mesenchymal tumor, could be ubiquitous but rarely arises from pancreas. Surgery is considered the most appropriate treatment. Nevertheless, activation of mTOR pathway seems to be a common pathogenic event in PEComas paving the way to chemotherapy by mTOR inhibitor. A 17 year-old man presented a hypervascular tumor of 55 mm, located in the head of pancreas without bile duct or pancreatic duct compression. Histopathology showed epithelioid cells with clear or focally granular eosinophilic cytoplasm with melanocytic (HMB-45, Melan-A) and myoid markers which confirmed diagnosis of PEComa. Given the absence of worrisome feature, we ruled out surgery and decided to initiate treatment with Sirolimus, an mTOR inhibitor. After 3.5 years, we showed a significant reduction in size of the tumor. This first case of pancreatic PEComa treated by mTOR inhibitor without surgery suggests a good efficiency of this therapy.
Sections du résumé
BACKGROUND
BACKGROUND
Perivascular epithelioid cell tumor, an extremely rare mesenchymal tumor, could be ubiquitous but rarely arises from pancreas. Surgery is considered the most appropriate treatment. Nevertheless, activation of mTOR pathway seems to be a common pathogenic event in PEComas paving the way to chemotherapy by mTOR inhibitor.
METHOD
METHODS
A 17 year-old man presented a hypervascular tumor of 55 mm, located in the head of pancreas without bile duct or pancreatic duct compression.
RESULTS
RESULTS
Histopathology showed epithelioid cells with clear or focally granular eosinophilic cytoplasm with melanocytic (HMB-45, Melan-A) and myoid markers which confirmed diagnosis of PEComa. Given the absence of worrisome feature, we ruled out surgery and decided to initiate treatment with Sirolimus, an mTOR inhibitor. After 3.5 years, we showed a significant reduction in size of the tumor.
CONCLUSION
CONCLUSIONS
This first case of pancreatic PEComa treated by mTOR inhibitor without surgery suggests a good efficiency of this therapy.
Identifiants
pubmed: 31130397
pii: S1424-3903(19)30562-9
doi: 10.1016/j.pan.2019.05.459
pii:
doi:
Substances chimiques
MTOR protein, human
EC 2.7.1.1
TOR Serine-Threonine Kinases
EC 2.7.11.1
Sirolimus
W36ZG6FT64
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
566-568Informations de copyright
Copyright © 2019 IAP and EPC. Published by Elsevier B.V. All rights reserved.