Sack Jason E, Kuchnir Louis, Demierre Marie-France
Department of Dermatology, Boston University Medical Center, Boston, Massachusetts, USA.
Pediatr Dermatol. 2011 Sep-Oct;28(5):568-9. doi: 10.1111/j.1525-1470.2010.01244.x. Epub 2010 Nov 12.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant spindle-cell tumor usually presenting in adulthood. The epidemiology of DFSP has recently been reviewed, and there have been 152 reported cases of DFSP in patients below the age of sixteen. We present the case of a DFSP arising in a young patient with Shwachman-Diamond syndrome (SDS).
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的恶性梭形细胞瘤，通常在成年期出现。最近对DFSP的流行病学进行了综述，已有152例16岁以下患者的DFSP报告病例。我们报告了1例发生于患有施瓦赫曼-戴蒙德综合征（SDS）的年轻患者的DFSP病例。
