Kretschmar C S, Colbach C, Bhan I, Crombleholme T M
Division of Hematology-Oncology, Department of Pediatrics, Floating Hospital for Children at the New England Medical Center, Boston, Massachusetts 02111, USA.
J Pediatr Hematol Oncol. 1996 Aug;18(3):293-8. doi: 10.1097/00043426-199608000-00012.
Desmoplastic round cell tumor (DSCT) is a highly malignant abdominal tumor first described in 1991, with subsequent cases predominantly noted in pathologic case reports. The authors evaluated response to alternating, intensive chemotherapy in three patients with DSCT, and reviewed the clinical experience with this newly described tumor as reported in the literature.
Three adolescent boys with DSCT were treated intravenously with vincristine 2 mg/m2, doxorubicin 75 mg/m2, cyclophosphamide 1.8 g/m2, alternating with 5-day cycles of etoposide 100 mg/m2/day, ifosfamide 1.8 g/m2/day for a total of 11-15 courses.
Each patient showed initial tumor regression during chemotherapy, but developed progressive disease within 8-18 months. One patient subsequently showed a transient response to doxorubicin 45 mg/m2 plus 5-fluorouracil 500-600 mg/m2. All three patients died of disease within 20 months of diagnosis. A comprehensive literature review of clinical data on 101 reported cases of DSCT is presented. The median age was 21 years (range 6-38 years) with 78 male patients and 23 female patients. Ninety-nine cases involved tumor mass in the abdominal-pelvic cavity in proximity to the mesentery. Metastatic seeding to the omentum was most common, followed by spread of disease to liver, distant lymph nodes, lung, and occasionally to scrotum or to ovary. Tumor response to chemotherapy was noted in approximately 50% of 40 patients who received combinations of doxorubicin, cisplatin, cyclophosphamide, etoposide, and/or 5-fluorouracil. Four of 13 patients who received additional radiotherapy were alive at 24-48 months. Median survival was 17 months (range: 3-72 months), with only two patients reported disease free beyond 2 years at 40 and 48 months.
DSCT should be included in the differential diagnosis of small round cell tumors in children and young adults. Tumor regression has been noted during multiagent chemotherapy, but prolonged survival is rare with current therapies.
促结缔组织增生性圆细胞肿瘤（DSCT）是一种高度恶性的腹部肿瘤，于1991年首次被描述，后续病例主要在病理病例报告中被提及。作者评估了3例DSCT患者对交替强化化疗的反应，并回顾了文献中报道的关于这种新描述肿瘤的临床经验。
3例患有DSCT的青春期男孩接受静脉化疗，长春新碱2mg/m²、阿霉素75mg/m²、环磷酰胺1.8g/m²，与依托泊苷100mg/m²/天、异环磷酰胺1.8g/m²/天的5天疗程交替进行，共11 - 15个疗程。
每位患者在化疗期间均出现初始肿瘤退缩，但在8 - 18个月内病情进展。1例患者随后对阿霉素45mg/m²加5 - 氟尿嘧啶500 - 600mg/m²出现短暂反应。所有3例患者在诊断后20个月内均死于疾病。本文对101例报道的DSCT临床资料进行了全面的文献综述。中位年龄为21岁（范围6 - 38岁），男性患者78例，女性患者23例。99例病例的肿瘤肿块位于腹腔 - 盆腔靠近肠系膜处。最常见的转移播散至大网膜，其次是疾病扩散至肝脏、远处淋巴结、肺，偶尔扩散至阴囊或卵巢。在接受阿霉素、顺铂、环磷酰胺、依托泊苷和/或5 - 氟尿嘧啶联合化疗的40例患者中，约50%的患者肿瘤对化疗有反应。13例接受额外放疗的患者中有4例在24 - 48个月时存活。中位生存期为17个月（范围：3 - 72个月），只有2例患者在40个月和48个月时报告无病生存超过2年。
DSCT应纳入儿童和青年小圆细胞肿瘤的鉴别诊断中。多药化疗期间已观察到肿瘤退缩，但目前的治疗方法很少能延长生存期。
