CASE REPORT article
Front. Med.
Sec. Pathology
A Rare Case of Clear Cell Sarcoma-like/Malignant Gastrointestinal Neuroectodermal Tumor in the Pancreas: Case Report and Literature Review
Jing Huang
Lu Zhou
Xiaoyu Chen
Yuzhen Huang
Ruwei Mo
Lixia Zeng
Shengming Wu
Wenqi Luo
Guangxi Medical University Cancer Hospital, Nanning, China
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Abstract
Malignant gastrointestinal neuroectodermal tumor (M-GNET), also known as clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) or clear cell sarcoma-like tumor of the gastrointestinal tract with osteoclast-like giant cells, is a rare malignant tumor that typically arises in the gastrointestinal tract. It demonstrates primitive neural or neuroectodermal differentiation but lacks melanocytic features. M-GNET is closely related to clear cell sarcoma of soft tissue (CCSST), sharing highly overlapping morphological features and molecular genetic characteristics, particularly EWSR1-ATF1 gene fusion and, more rarely, EWSR1-CREB1 gene fusion. Most M-GNETs occur in the lower gastrointestinal tract, while only a few cases have been reported in the upper gastrointestinal tract and outside the digestive tract. We present the primary M-GNET in the pancreas, confirmed by molecular fluorescence in situ hybridization (FISH) detection of EWSR1-ATF1 gene fusion. This article will summarize the clinicopathological features and differential diagnosis and review the relevant literature.
Summary
Keywords
fluorescence in situ hybridization, Gastrointestinal clear cell sarcoma-like tumor, malignant gastrointestinal neuroectodermal tumor, Pancreas, Soft tissue neoplasm
Received
15 December 2025
Accepted
19 February 2026
Copyright
© 2026 Huang, Zhou, Chen, Huang, Mo, Zeng, Wu and Luo. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Shengming Wu; Wenqi Luo
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