Department of Orthopedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.
Anticancer Res. 2010 Sep;30(9):3769-74.
Desmoplastic small round cell tumour (DSRCT) is a rare tumour, usually arising in the abdominal cavity. DSRCT remains an aggressive malignancy, with a poor prognosis despite multi-modality treatments. In the published literature, there has been no patient who lived for three years or more without surgical excision. This report describes a case of DSRCT arising from the brachial plexus and successfully treated with caffeine-assisted chemotherapy. A 29-year-old male presented with pain and numbness in his left forearm. Radiological findings were suggestive of malignant tumour. Histology, immunohistochemical stain and fluorescence in situ hybridisation (FISH) results confirmed the diagnosis of DSRCT. He underwent caffeine-potentiated chemotherapy and the tumour disappeared. The tumour was not removed surgically as it was intertwined in the brachial plexus. Four years after the initial diagnosis, no local relapse and no distant metastases have been observed. Therefore, it is concluded that caffeine-assisted chemotherapy should be one of the treatment options for DSRCT.
促肾上腺皮质激素辅助化疗治疗臂丛起源的促结缔组织增生性小圆细胞肿瘤 促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的肿瘤，通常发生在腹腔内。尽管采用了多种治疗方法，DSRCT 仍然是一种侵袭性恶性肿瘤，预后不良。在已发表的文献中，没有患者在不进行手术切除的情况下存活三年或更长时间。本报告描述了一例起源于臂丛的 DSRCT，成功接受了咖啡因辅助化疗治疗。一名 29 岁男性因左前臂疼痛和麻木就诊。影像学检查结果提示恶性肿瘤。组织学、免疫组化染色和荧光原位杂交（FISH）结果证实了 DSRCT 的诊断。他接受了咖啡因增强化疗，肿瘤消失。由于肿瘤与臂丛交织在一起，未进行手术切除。初始诊断后 4 年，未观察到局部复发和远处转移。因此，结论是咖啡因辅助化疗应该是 DSRCT 的治疗选择之一。
