{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T15:22:12Z","timestamp":1785338532297,"version":"3.55.0"},"reference-count":23,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T00:00:00Z","timestamp":1678924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>(1) Background: A malignant gastrointestinal neuroectodermal tumor (GNET) is an ultra-rare primary neoplasm with a distinctive histopathological, immunohistochemical, molecular, and ultramicroscopic profile, synonymous terminology with clear cell sarcoma-like tumor of the gastrointestinal tract. This case report aims to describe a case of GNET with challenging mesenchymal, lymphoid, and melanic tumor differential diagnosis. (2) Case presentation: We discuss the case of a 67-year-old male patient who presented with diffuse abdominal pain, intermittent lack of intestinal transit, and frequent episodes of nausea, followed by segmental resection of the jejunum and sigmoid colon. The patient had no relevant medical history. The surgical specimen underwent immunohistochemical staining and morphological evaluation. (3) Results: Histopathological analysis reveals a moderately homogeneous polyhedral-epithelioid and spindle cell neoplastic proliferation with a zonal discohesive pattern and extensive and focal fasciculated architecture. Twenty monoclonal antibodies were used for immunostaining, which allowed GNET to be diagnosed on the basis of the tumoral immune profile, characterized by positive reactivity of S100, SOX10, and CD 56. (4) Conclusions: The poor prognosis of GNET is highlighted in the present study, along with the vital importance of differential diagnosis issues with mesenchymal, lymphoid, and melanic tumors, which make the diagnosis difficult for both pathologists and clinicians.<\/jats:p>","DOI":"10.3390\/diagnostics13061131","type":"journal-article","created":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T02:29:59Z","timestamp":1679020199000},"page":"1131","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["The Malignant Gastrointestinal Neuroectodermal Tumor (GNET): A Distinct Entity and the Challenging Differential Diagnosis with Mesenchymal, Lymphoid, and Melanic Tumors: A Case Report and Brief Review of the Literature"],"prefix":"10.3390","volume":"13","author":[{"given":"M\u0103d\u0103lina","family":"Bo\u0219oteanu","sequence":"first","affiliation":[{"name":"Faculty of Medicine, \u201cOvidius\u201d University of Constanta, 900470 Constanta, Romania"},{"name":"Department of Clinical Pathology, \u201cSf. Apostol Andrei\u201d Emergency County Hospital, 900591 Constanta, Romania"},{"name":"Department of Pathology, \u201cOvidius\u201d Clinical Hospital, 905900 Constanta, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5191-2585","authenticated-orcid":false,"given":"Miruna","family":"Cristian","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cOvidius\u201d University of Constanta, 900470 Constanta, Romania"},{"name":"Department of Clinical Pathology, \u201cSf. Apostol Andrei\u201d Emergency County Hospital, 900591 Constanta, Romania"},{"name":"Center for Research and Development of the Morphological and Genetic Studies of Malignant Pathology\u2014CEDMOG, \u201cOvidius\u201d University of Constanta, 900591 Constanta, Romania"},{"name":"Institute of Doctoral Studies, School of Medicine, \u201cOvidius\u201d University of Constanta, 900573 Constanta, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana","family":"A\u0219chie","sequence":"additional","affiliation":[{"name":"Department of Clinical Pathology, \u201cSf. Apostol Andrei\u201d Emergency County Hospital, 900591 Constanta, Romania"},{"name":"Center for Research and Development of the Morphological and Genetic Studies of Malignant Pathology\u2014CEDMOG, \u201cOvidius\u201d University of Constanta, 900591 Constanta, Romania"},{"name":"Academy of Medical Sciences, 030167 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Radu Andrei","family":"Baz","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cOvidius\u201d University of Constanta, 900470 Constanta, Romania"},{"name":"Department of Radiology, \u201cSf. Apostol Andrei\u201d Emergency County Hospital, 900591 Constanta, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alina Marta","family":"Zielonka","sequence":"additional","affiliation":[{"name":"Clinica Sante, 060754 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgeta Camelia","family":"Cozaru","sequence":"additional","affiliation":[{"name":"Department of Clinical Pathology, \u201cSf. Apostol Andrei\u201d Emergency County Hospital, 900591 Constanta, Romania"},{"name":"Center for Research and Development of the Morphological and Genetic Studies of Malignant Pathology\u2014CEDMOG, \u201cOvidius\u201d University of Constanta, 900591 Constanta, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luana Andreea","family":"Bo\u0219oteanu","sequence":"additional","affiliation":[{"name":"Institute of Doctoral Studies, School of Medicine, \u201cOvidius\u201d University of Constanta, 900573 Constanta, Romania"},{"name":"Dermatology Department, \u201cElias\u201d Emergency University Hospital, 011461 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/j.pathol.2018.05.001","article-title":"Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumor: Distinct or related entities? A review","volume":"50","author":"Green","year":"2018","journal-title":"Pathology"},{"key":"ref_2","unstructured":"Goldblum, J.R., Lazar, A.J., Miettinen, M., and Tsui, W.M.W. (2019). WHO Classification of Tumours Editorial Board. Digestive System Tumours, International Agency for Research on Cancer. [5th ed.]. Available online: https:\/\/publications.iarc.fr\/579."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1016\/j.prp.2015.06.001","article-title":"An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma","volume":"211","author":"Insabato","year":"2015","journal-title":"Pathol. Res. Pract."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/s13000-017-0620-9","article-title":"Malignant gastrointestinal neuroectodermal tumor: A case report and review of the literature","volume":"12","author":"Alyousef","year":"2017","journal-title":"Diagn. 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