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A ganglion‐rich gastrointestinal stromal tumor: A case report
Author(s) -
Iemura Yoshiki,
Katsushima Hiroki,
Kataoka Tatsuki R.,
Sakurai Takaki,
Hirota Seiichi,
Shimada Toshihide
Publication year - 2019
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/pin.12802
Subject(s) - interstitial cell of cajal , pathology , gist , stromal cell , neural crest , biology , stromal tumor , progenitor cell , mutation , stem cell , ganglion , immunohistochemistry , cancer research , medicine , anatomy , microbiology and biotechnology , embryo , biochemistry , gene
We report a case of an extremely rare type of duodenal gastrointestinal stromal tumor (GIST) that included neuronal components. Although gastrointestinal autonomic nerve tumors (GANTs), a subtype of GISTs, exhibit ultrastructural features of the nerve plexus, neuronal cells have not been observed within GANTs or GISTs. GISTs originate from interstitial cells of Cajal (ICCs), which are markedly different from the progenitor cells of neural elements and neural‐crest‐derived stem cells. This may explain why GISTs typically lack neuronal elements. It remains unclear that the neuronal components of this tumor are neoplastic or hyperplastic, but proliferation and survival of ICCs have recently been reported to be closely related to neurons. Although we could not find the KIT, PDGFR , and BRAF mutation as far as we examined, it may have had a rare mutation in NF1 , a fusion of EVT6‐NTRK3 , or an as‐yet‐unknown KIT mutation that affected neurogenesis. Further investigation of related genetic mutations and accumulation of data from other similar cases is needed.

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