摘要
目的探讨促纤维组织增生性小圆细胞瘤（DSRCT）的临床诊断与治疗特点，以提高对其认识和诊疗水平。方法回顾性分析2004年10月至2014年6月9例DSRCT患者的临床资料，对DSCRT的临床表现、病理特点、诊断与鉴别诊断、治疗与预后进行总结和分析。结果9例DSRCT患者中，男5例，女4例；年龄8—56岁，平均年龄21岁。超声检查示，腹腔内形态不规则低密度肿块影。CT检查示，6例腹腔及腹膜后多发实性肿块结节，密度不均，可见液性低密度区。术后病理检查示，肿瘤细胞体积小，多为椭圆形，聚集成无规则但界限清晰的巢状结构。较大的细胞巢中央有坏死，包绕癌巢的间质组织中可见散在分布的纤维母细胞，并产生大量胶原纤维伴玻璃样变。6例患者行手术治疗，均不能完整切除肿瘤，术后3例患者化疗，其中2例TP方案（卡铂＋紫杉醇）化疗；1例化疗方案不详。2例患者放化疗。1例失访。未行手术治疗的3例患者中，2例患者行CAP方案（环磷酰胺＋阿霉素＋卡铂）化疗及全直肠病灶、盆腔、腹股沟淋巴结、髂骨转移灶照射；1例行EP方案（顺铂＋依托泊苷）化疗后改为TP方案化疗。9例患者中，8例术后短期内死亡，仅1例单纯化疗患者，随访11个月目前仍生存。结论DSRCT是一种临床罕见、预后极差的特殊类型软组织肿瘤。利用影像学检查特征性图像及肿瘤标志物检测，可以初步诊断DSRCT，最终确诊依据病理诊断。治疗采用手术为主，辅以放化疗的综合性治疗方案。
Objective To explore the clinical diagnostic features and treatment of desmoplastic small round cell tumor （ DSRCT）, and to improve the understanding and management of this tumor. Methods The clinicopathological data of nine patients treated in our hospital from October 2004 to June 2014 were retrospectively analyzed and a review of the literature was made. The clinical manifestations, pathological characteristics, diagnosis and differential diagnosis, treatment and prognosis of this tumor were summarized and analyzed. Results Nine patients with DSRCT, 5 males and 4 females, with an average age of 21 years （range 8-56 years） were included in this study. Ultrasound examination revealed irregular low- density mass shadow in the abdominal cavity. CT examination found that 6 cases had abdominal and retroperitoneal multiple solid tumor nodules, uneven density, and visible low density fluid area. Postoperative pathological examination revealed that the tumor cells were small, mostly elliptic, gathered to form clear structure of nests with clear irregular boundaries. The central portion of large tumor nests often showed necrosis. Scattered fibroblasts and large amount of hyalinization of collagen fibers were seen in the interstitial tissue around the nests. Six patients received laparotomy surgery, however, all failed to resect the tumor completely. Three patients received postoperative chemotherapy, i.e. two cases had carboplatin and paclitaxel chemotherapy, and one case of chemotherapy regimen not specified. Two patients had radiation and chemotherapy （no concrete plan was available）. Another case was lost to follow-up. Two of the three patients without surgery received chemotherapy with CAP （cyclophosphamide ＋ adriamycin ＋ carboplatin） and total rectal lesions, pelvic and inguinal lymph nodes, ilium metastases radiation therapy. Another one patient received EP regimen （DDP＋VP16） which was then changed into a TP chemotherapy alone. Eight of the nine cases died shortly after surgery, and onl
出处
《中华肿瘤杂志》
CAS
CSCD
北大核心
2015年第9期686-690,共5页
Chinese Journal of Oncology
基金
国家自然科学基金（81302211）
天津市卫生局科技基金（2013KZ107）
