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促结缔组织增生性小圆细胞肿瘤(desmoplastic small round cell tumor,DSRCT)由组织起源未定的小圆肿瘤细胞构成,伴有明显间质硬化和表型分化,由Gerald和Rosai于1989年首先报道~([1]),并于1991年正式命名。DSRCT是一种十分少见的、高侵袭性的恶性肿瘤,且预后差。现报道我院近期收治1例DSRCT,并结合相关文献进行讨论,以提高对本病的认识。1临床资料患者,女性,43岁,因下腹部疼痛伴腹胀1月余入院。入
Desmoplastic small round cell tumor (DSRCT) is composed of small, primitive tumor cells of tissue origin with obvious interstitial sclerosis and phenotypic differentiation reported by Gerald and Rosai in 1989 ~ ([1]) and officially named it in 1991. DSRCT is a rare, highly aggressive malignancy with poor prognosis. It is reported that our hospital recently admitted to a case of DSRCT, combined with the relevant literature to discuss to raise awareness of the disease. 1 Clinical data Patients, female, 43 years old, due to lower abdominal pain with bloating more than 1 month admitted to hospital. Into