title: [Gastro-intestinal neuroectodermal tumor (GNET): A case report of a small intestine tumor with hepatic metastases].
abstract: The gastro-intestinal neuroectodermal tumor (GNET) is a rare sarcoma of the digestive tract, which was recently recognised. The knowledge of the morphological, immunohistochemical and molecular diagnostic criteria is necessary to not mistake it for the metastasis of a melanoma or for another sarcoma of the digestive tract as the gastro-intestinal clear cells sarcoma or the malignant peripheral nervous system tumor (MPNST). We report the case of a 41-year-old patient with a GNET of the small intestine with hepatic metastasis. The histological examination showed a diffuse proliferation of epithelioid cells, which only express PS100. The presence EWSR1-ATF1 gene fusions with any melanocytic differentiation leads to the diagnosis of GNET.
authors.affiliation: Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France. Electronic address: thibault.kervarrec@etu.univ-tours.fr.
authors.forename: Thibault
authors.initials: T
authors.lastname: Kervarrec
authors.affiliation: Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Lecointre
authors.affiliation: Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
authors.forename: Rémy
authors.initials: R
authors.lastname: Kerdraon
authors.affiliation: Service de dermatologie, hôpital Porte-Madeleine, 1, rue Porte-Madeleine, 45000 Orléans, France.
authors.forename: Guido
authors.initials: G
authors.lastname: Bens
authors.affiliation: Service de chirurgie viscérale, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Piquard
authors.affiliation: Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Michenet
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D003937:Diagnosis, Differential; D005334:Fever / Q000209:etiology; D006801:Humans; D007414:Intestinal Neoplasms / Q000235:genetics / Q000473:pathology*; D008113:Liver Neoplasms / Q000175:diagnosis / Q000556:secondary*; D008297:Male; D008545:Melanoma / Q000175:diagnosis; D017599:Neuroectodermal Tumors / Q000175:diagnosis / Q000235:genetics / Q000556:secondary*; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D009418:S100 Proteins / Q000032:analysis; D018227:Sarcoma, Clear Cell / Q000175:diagnosis; D015431:Weight Loss
chemical_list: D014408:Biomarkers, Tumor; C560860:EWSR1-ATF1 fusion protein, human; D015514:Oncogene Proteins, Fusion; D009418:S100 Proteins
keywords: Clear cell sarcoma; Digestive sarcoma; ESWR1; EWSR1; Gastro-intestinal neuroectodermal tumor; Melanoma; Mélanome; Sarcome à cellules claires; Sarcomes digestifs; Tumeur neuroectodermique gastro-intestinale