Publication: Cystic gastrointestinal stromal tumors
of the pancreas simulating cystoadenocarcinoma.
Report of three cases and short review of the literature
Authors
Ambrosio, Maria Raffaella ; Rocca, B.J. ; Mastrogiulio, M.G. ; Pesci, A. ; De Martino, A. ; Mazzei, M.A. ; Volterrani, L. ; Arcuri, F. ; Cintorino, M. ; Tripodi, S.A.
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Publisher
F. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histología
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DOI
https://doi.org/10.14670/HH-29.1583
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info:eu-repo/semantics/article
Description
Abstract
Gastrointestinal stromal tumors (GISTs)
represent a distinct subset of mesenchymal tumours of
the gastrointestinal tract. They are more common in the
stomach and small intestine, and are characterized by the
proliferation of spindle or epithelioid cells and by the
expression of CD117. Extra-gastrointestinal stromal
tumors are rare and only 13 cases of pancreatic GISTs
have been reported in the literature, only 1 of which
presented as a cystic lesion. Mutational analysis of KIT
and Platelet derived growth factor receptor-α genes was
performed only in two out of the 13 cases.
We report 3 cases of cystic GISTs of the pancreas,
radiologically mimicking a cystoadenocarcinoma.
Routine histopathology and molecular characterization
of the tumours have been performed. In two of them,
molecular analysis showed unusual genetic alterations
(the internal repeat of codon 502 and 503 in exon 9 of
the KIT gene and the KIT exon 9 single nucleotide
substitution c.1427G>T).
Pancreatic GIST should be included in the
differential diagnosis of both cystic and solid masses of
the pancreas. The diagnosis should be accomplished by a
combination of radiology, histology, immunohistochemistry
and molecular biology. The evaluation of
CD117 expression and the sequence analysis of KIT and
Platelet derived growth factor receptor-α gene is
mandatory for therapy.
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