Frappaz D, Bouffet E, Dolbeau D, Bouvier R, Carrie C, Louis D, Pondarre C, Tabone E, Philip T, Brunat-Mentigny M
Pediatric and Pathology Department, Centre Léon Bérard, France.
Cancer. 1994 Mar 15;73(6):1753-6. doi: 10.1002/1097-0142(19940315)73:6<1753::aid-cncr2820730633>3.0.co;2-0.
Desmoplastic small round cell tumors (DSRCT) have been only recently identified.
The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization.
These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT:
The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted.
促结缔组织增生性小圆细胞肿瘤（DSRCT）直到最近才被发现。
作者报告了两名儿科患者（一名8岁男孩和一名12岁男孩）的DSRCT。两名患者最初均被诊断为横纹肌肉瘤。对标准化疗和放射治疗的耐药性促使作者重新检查最初的活检标本并进行补充免疫表型特征分析。
这些分析显示肿瘤细胞对角蛋白上皮标记抗原、结蛋白、波形蛋白、神经特异性烯醇化酶和S100蛋白呈强阳性，这与DSRCT的多形性分化特征相符。
作者建议对所有腹部小圆细胞肿瘤病例进行广泛的免疫组织学特征分析，以免漏诊DSRCT。这些罕见肿瘤对化疗耐药，初始应积极进行手术。
