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- Title
Gastrointestinal neuroectodermal tumour: A case report of a rare tumour.
- Authors
Macpherson, James; Ganugapati, Usharani; Herath, Chaturika
- Abstract
Gastrointestinal neuroectodermal tumours (GNET) are rare mesenchymal neoplasms, originally described under the name clear cell sarcoma-like tumour of the gastrointestinal tract (CCSLTGT). GNETs have a poor prognosis and often reoccur as metastatic disease after primary resection. The differential diagnosis of GNET includes gastrointestinal stromal tumour (GIST), clear cell sarcoma of the gastrointestinal tract (CCS-GI), and melanoma. GNET are characterized by a sheet-like or nested population of cells which can be epithelioid or spindled. Immunohistochemistry demonstrates a strong diffuse staining for S100. Melanocytic makers, markers for GIST, and epithelial markers are negative. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is frequently rearranged in GNET cases although these rearrangements are neither diagnostic for, nor specific to, GNET. This report describes a case of GNET that presented as a small bowel obstruction in a 65-year-old man and, later, as metastatic disease in the liver. The tumours in this case comprised sheets of poorly differentiated spindle and epithelioid cells with an immunohistochemical profile consistent with GNET. The case did not, however, show EWSR1 gene rearrangement. GNET is a rare and aggressive tumour with a broad differential. It can be difficult to diagnose especially if the common features are not present.
- Subjects
NEUROECTODERMAL tumors; COMMUNICABLE diseases; HISTOPATHOLOGY; PATHOLOGICAL physiology; PATHOLOGISTS
- Publication
Canadian Journal of Pathology, 2021, Vol 13, Issue 1, p47
- ISSN
1918-915X
- Publication type
Article