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Ann Pathol. 2019 Apr;39(2):167-171. doi: 10.1016/j.annpat.2018.10.004. Epub 2018 Dec 13.

[Clear cell sarcoma or gastrointestinal neuroectodermal tumor (GNET) of the tongue? Case report and review of the literature of an extremely rare tumor localization].

[Article in French]

Author information

1
Service d'anatomie pathologique, hôpital André-Mignot, centre hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay, France.
2
Service d'ORL, hôpital André-Mignot, centre hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay, France.
3
Service d'hématologie-oncologie, centre hospitalier de Versailles, hôpital André-Mignot, 177, rue de Versailles, 78150 Le Chesnay, France.
4
Service de médecine nucléaire, hôpital André-Mignot, centre hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay, France.
5
Service de pathologie, institut Curie, hôpital René-Huguenin, 35, rue Dailly, 92210 Saint-Cloud, France.
6
Service d'anatomie pathologique, hôpital André-Mignot, centre hospitalier de Versailles, 177, rue de Versailles, 78150 Le Chesnay, France. Electronic address: ydenoux@ch-versailles.fr.

Abstract

Clear cells sarcomas (CCS) are exceptionally rare in the tongue, with, to our knowledge, only three previous reports in anglo-saxon literature. Through our case, we will discuss the differential diagnosis of clear cells tumors of the tongue and bring this tumour closer to the newly described entity of the gastrointestinal tract named "clear cells sarcoma-like gastrointestinal (SCCLGI)", recently renamed "gastrointestinal neuroectodermal tumour (GNET)". SCCLGI/GNET share morphological and molecular characteristics with SCC but had until then been observed only in the digestive tract. Our case could be a lingual localization of a SCCLGI/GNET. SCC and SCCLGI/GNET characteristic molecular profil involves EWSR1-ATF1 [t(12; 22) (q13; q12)] and EWSR1-CREB1 [t(2; 22) (q34; q12)] fusion genes, but it is not specific of these tumours.

KEYWORDS:

Clear cell sarcoma; Gastrointestinal neuroectodermal tumour; Langue; Sarcome à cellules claire; Tongue; Tumeur neuroectodermique gastro-intestinale

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