Bisogno G, Roganovich J, Sotti G, Ninfo V, di Montezemolo L C, Donfrancesco A, Mascarin M, Carli M
Division of Haematology/Oncology, Department of Paediatrics, University of Padova, Padova, Italy.
Med Pediatr Oncol. 2000 May;34(5):338-42. doi: 10.1002/(sici)1096-911x(200005)34:5<338::aid-mpo4>3.0.co;2-0.
Desmoplastic small round cell tumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce.
We report six cases of children and adolescents (median age 14 years, range 6.9-17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period. Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy. Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin).
Complete surgical resection was possible only for the paratesticular tumour. Among the patients with abdominal lesions, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients. No relapses were evident in the irradiated fields in the 4 patients who received radiotherapy. Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis.
DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy. Our results support surgical tumour removal and radiotherapy to achieve local control. Our experience and a review of the literature suggest that patients with localised abdominal tumours or a paratesticular primary may have a better prognosis.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的高侵袭性肿瘤，临床研究较少。
我们报告了意大利软组织肉瘤合作组（ICG）在9年期间登记的6例DSRCT患儿及青少年患者（中位年龄14岁，范围6.9 - 17.5岁）（5例腹部肿瘤，1例睾丸旁肿瘤）。患者接受了多学科治疗，包括积极的初始或延迟手术及放疗。化疗方案基于异环磷酰胺、长春新碱、放线菌素D的使用，以及几剂蒽环类药物（多柔比星或表柔比星）。
仅睾丸旁肿瘤可行完整手术切除。在腹部病变患者中，仅1例可进行宏观上的根治性切除。所有患者均接受了多药化疗，4例可评估患者实现了肿瘤缩小。接受放疗的4例患者照射野内均未出现明显复发。2例患者在诊断后22个月和63个月无疾病进展，1例处于第三次完全缓解期，而3例在诊断后10 - 25个月死亡。
DSRCT是一种对化疗敏感的肿瘤，但尽管采用了积极的多模式治疗，生存率仍然令人失望。我们的结果支持通过手术切除肿瘤和放疗来实现局部控制。我们的经验及文献综述表明，局限性腹部肿瘤或睾丸旁原发性肿瘤患者可能预后较好。
