Gastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor (CCS/GNET) in a Young Filipino Adult: A Case Report
- Author:
Kristine Joy S. Uichanco
1
;
Erick Martin H. Yturralde
1
Author Information
- Publication Type:Other Types
- MeSH: Jejunal Neoplasms; Neuroectodermal Tumors; Sarcoma
- From: Philippine Journal of Pathology 2026;(75th PSP Research Competition Abstracts):1-
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Gastrointestinal neuroectodermal tumors (GNETs) are rare but
distinctive sarcomas that often arise from the small intestines.
Case Description:We report a 24-year-old Filipino male who initially presented with abdominal pain; imaging showed an enhancing circumferential mass involving the jejunum along with a peripherally enhancing focus in the liver and several enlarged superior mesenteric lymph nodes.
Discussion:Histopathologic evaluation of the tumor revealed a monomorphic population of epithelioid cells with pale eosinophilic to clear cytoplasm and round nuclei with open chromatin and occasional conspicuous nucleoli. The tumor cells assume various architectural patterns with admixed osteoclast-like giant cells. Immunohistochemistry studies showed diffuse positivity with SOX10 and S100 and negativity for HMB45. Fluorescence in situ hybridization demonstrated EWSR1 gene rearrangement confirming the diagnosis of GNET. Despite their relatively bland morphology, these tumors demonstrate aggressive behavior.
Conclusion:In light of more recent reports of similar neoplasms occurring in extraenteric sites, heightened clinical suspicion and familiarity with this peculiar malignancy is recommended. - Full text:2026090216150201245PJ Pathology Abstract 6.pdf
