Ariza-Prota Miguel Angel, Pando-Sandoval Ana, Fole-Vázquez David, Casan Pere
Pulmonologist - Hospital Universitario Central de Asturias (HUCA), Instituto Nacional de Silicosis (INS), Área del Pulmón, Oviedo, Spain.
Respir Med Case Rep. 2015 Sep 2;16:112-6. doi: 10.1016/j.rmcr.2015.08.010. eCollection 2015.
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that is characterized by nests of small tumor cells surrounded by a cellular and vascular collagenous stroma and predominantly affects young adolescent males. This tumor most commonly originates in the abdomen; however, in rare cases, DSRCT can originate in other body regions. The main manifestations of DSRCT are chest pain and respiratory symptoms, and patients' average survival after diagnosis is less than two years. In this report, we describe a case involving DSRCT of the lung that proved to be difficult to diagnose, and we conduct a literature review.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见、侵袭性强的恶性肿瘤，其特征是小肿瘤细胞巢被富含细胞和血管的胶原性间质所包绕，主要影响青少年男性。该肿瘤最常见于腹部起源；然而，在罕见情况下，DSRCT也可起源于身体其他部位。DSRCT的主要表现为胸痛和呼吸道症状，患者确诊后的平均生存期不到两年。在本报告中，我们描述了一例肺部DSRCT病例，该病例诊断困难，并进行了文献综述。
