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- Title
Clear cell sarcoma-like/malignant gastrointestinal neuroectodermal tumor of the tongue: a clinicopathologic and molecular case report.
- Authors
Sbaraglia, Marta; Zanatta, Lucia; Toffolatti, Luisa; Spallanzani, Andrea; Bertolini, Federica; Mattioli, Francesco; Lami, Federico; Presutti, Livio; Dei Tos, Angelo P.
- Abstract
Malignant gastrointestinal neuroectodermal tumor (M-GNET) and clear cell sarcoma (CCS) of soft tissue represent closely related, extremely rare, malignant mesenchymal neoplasm of uncertain differentiation. Both entities are characterized genetically by the same molecular alterations represented by the presence of EWSR1-ATF1 and, more rarely, EWSR1-CREB1 fusion genes. The latter translocation seems to be more represented in M-GNET that, despite significant morphologic overlap with CCS, tends to lack overt features of melanocytic differentiation. Most M-GNET occur in the lower gastrointestinal tract, whereas occurrence in the upper tract has been reported only exceptionally. The differential diagnosis represents a major challenge, and accurate diagnosis impact significantly on therapeutic planning. We herein report the clinicopathologic features of a molecularly confirmed M-GNET that arose at the base of the tongue and review the pertinent literature.
- Subjects
GASTROINTESTINAL tumors; NEUROECTODERMAL tumors; GENE fusion; DIAGNOSIS; GASTROINTESTINAL system; DIFFERENTIAL diagnosis
- Publication
Virchows Archiv: European Journal of Pathology, 2021, Vol 478, Issue 6, p1203
- ISSN
0945-6317
- Publication type
journal article
- DOI
10.1007/s00428-020-02933-2