Schwarz R E, Gerald W L, Kushner B H, Coit D G, Brennan M F, La Quaglia M P
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Ann Surg Oncol. 1998 Jul-Aug;5(5):416-22. doi: 10.1007/BF02303860.
Desmoplastic small round cell tumors (DSRCT or DSCT) are rare aggressive cancers of adolescence and early adulthood. There are few reported series to guide clinical therapy. This study correlates survival with treatment variables, including aggressive surgical debulking.
Thirty-two patients with documented DSRCT received treatment at our institution. Demographic, clinical, and treatment variables were correlated with progression-free survival using log-rank statistics.
Thirty patients were male (96%), and two were female (4%), with a median age at diagnosis of 22 years. The primary site of disease in 97% of cases was the abdomen or pelvis. Twenty-nine patients (91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. All 32 patients received systemic chemotherapy. Fifteen (47%) underwent tumor debulking greater than 90% at diagnosis or during therapy. A complete or very good response to therapy occurred in 13 patients, and depended on surgical removal of bulk disease in all. Thirteen patients remained progression-free, but three of these patients died from treatment toxicity. Improved survival was correlated with a complete or very good partial response to multimodality therapy, surgical debulking of more than 90% either before or after chemotherapy, and use of the P6 protocol.
DSRCT is an aggressive cancer that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.
促结缔组织增生性小圆细胞肿瘤（DSRCT或DSCT）是青少年和成年早期罕见的侵袭性癌症。鲜有报道的系列研究可指导临床治疗。本研究将生存率与治疗变量相关联，包括积极的手术减瘤。
32例确诊为DSRCT的患者在我们机构接受治疗。使用对数秩统计分析将人口统计学、临床和治疗变量与无进展生存期相关联。
30例患者为男性（96%），2例为女性（4%），诊断时的中位年龄为22岁。97%的病例中疾病的原发部位是腹部或骨盆。29例患者（91%）有广泛病变，累及腹膜表面、淋巴结或不连续器官。所有32例患者均接受了全身化疗。15例（47%）患者在诊断时或治疗期间进行了大于90%的肿瘤减瘤。13例患者对治疗有完全或非常好的反应，且均依赖于大块疾病的手术切除。13例患者保持无进展，但其中3例患者死于治疗毒性。生存率的提高与对多模式治疗的完全或非常好的部分反应、化疗前后大于90%的手术减瘤以及使用P6方案相关。
DSRCT是一种主要发生在年轻男性的侵袭性癌症。生存率的提高与强化化疗和积极切除相关。
