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Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor of the Neck: A Diagnostic Challenge. 2025
Manuel Tousidonis, and
Maria J Troulis, and
Carolina Agra, and
Francisco Alijo, and
Ana Alvarez-Gonzalez, and
Carlos Navarro-Cuellar, and
Saad Khayat, and
Gonzalo Ruiz-de-Leon, and
Ana Maria Lopez-Lopez, and
Jose Ignacio Salmeron, and
Santiago Ochandiano
Department of Oral and Maxillofacial Surgery, Gregorio Marañón University Hospital, Calle Doctor Esquerdo 46, 28006 Madrid, Spain.
Malignant gastrointestinal neuroectodermal tumor (MGNET) and clear cell sarcoma (CCS) of soft tissue represent related, extremely rare, malignant mesenchymal neoplasms. Both entities are genetically characterized by the same molecular alterations, EWSR1::CREB1 fusions. Malignant gastrointestinal neuroectodermal tumor has significant morphological overlap with CCS, although it tends to lack overt features of melanocytic differentiation. Recently, rare MGNET cases were reported in extragastrointestinal sites. The diagnosis represents a major challenge and significantly impacts therapeutic planning. In this study, we reported the clinicopathologic features of a molecularly confirmed MGNET of the neck and provided a review of the pertinent literature.
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