• 作者：Marí ; a Alastuey Aisa^a ; ^{mariaalastuey@yahoo.es" class="auth_mail} ; Carlos Hö ; rndler Argarate^a ; David S. Rosero Cuesta^a ; Alberto Valero Torres^a ; Enrique de Á ; lava Casado^b
• 关键词：Tumor desmoplá ; sico de cé ; lulas pequeñ ; as y redondas ; Pared torá ; cica ; Translocació ; n t(11 ; 22)(p13 ; q12) ; EWS-WT1
• 刊名：Revista Espa帽ola de Patolog铆a
• 出版年：January-March, 2014
• 年：2014
• 卷：47
• 期：1
• 页码：41-44
• 全文大小：2104 K

Desmoplastic small round cell tumour is a rare, aggressive neoplasm usually found in young males in the abdominal or pelvic peritoneum. It is characterized by the presence of small undifferentiated cells with hyperchromatic nuclei.

Its main diagnostic feature is the co-expression of epithelial, muscular and neural markers in addition to the reciprocal translocation t(11; 22)(p13; q12), which affects the WT1 and EWS gene.

We present a case of a 7 year old girl with a tumour in the thoracic wall. The unusual location, together with an atypical IHC profile meant that diagnosis was only possible after molecular studies. The patient underwent postoperative chemotherapy and is alive and well 7 months after surgery.