De Lena M, Caruso M L, Marzullo F, Mancarella S, Armentano R, Ventrella V, Guida M
Medical Oncology Division, IRCCS Istituto Oncologico, Bari, Italy.
Tumori. 1998 May-Jun;84(3):412-6. doi: 10.1177/030089169808400320.
The authors report the case of a 23-year-old woman affected by intra-abdominal desmoplastic small round cell tumor (DSRCT) who obtained a complete response to multiagent chemotherapy. DSRCT is a rare, highly aggressive neoplasm generally arising in young people and seldom in females (about 20 cases described in the literature).
The patient underwent surgical resection of a large 15 x 15 cm mass located in the right lower abdominal quadrant, but after only 2 months later, two liver metastasis were noted. Thus, she was subjected to an aggressive antineoplastic treatment consisting of three groups of alternating non-cross resistant multiagent regimens administered every 21 days (cis-platin-etoposide-adriamycin-bleomicin; gemcitabine-ifosfamide-dacarbazine; methotrexate-5-fluorouracil-folinic acid) for a total of 9 administrations.
After one cycle of treatment including the administration of all the three alternated schemes of chemotherapy, a complete disappearance of liver disease was noted. The treatment was relatively well-tolerated and the toxicity was acceptable. At present, after 15 months from diagnosis and 12 months after starting chemotherapy, the patient is disease-free and in good health.
Even though this study regards only a single patient, it is noteworthy because of the rarity of this neoplasm and because of the infrequent complete responses reported in the literature. The efficacy and manageability of the treatment, suggests that both the timing and schedule used could constitute an important therapeutical option for this aggressive and poorly chemo-responsive tumor.
作者报告了一例23岁患有腹腔促结缔组织增生性小圆细胞肿瘤（DSRCT）的女性患者，该患者对多药化疗获得了完全缓解。DSRCT是一种罕见的、高度侵袭性的肿瘤，通常发生于年轻人，很少见于女性（文献中描述约20例）。
患者接受了手术切除位于右下腹象限的一个15×15cm的大肿块，但仅2个月后，发现有两处肝转移。因此，她接受了积极的抗肿瘤治疗，包括每21天交替使用三组非交叉耐药的多药方案（顺铂-依托泊苷-阿霉素-博来霉素；吉西他滨-异环磷酰胺-达卡巴嗪；甲氨蝶呤-5-氟尿嘧啶-亚叶酸），共进行9次给药。
在包括所有三种交替化疗方案给药的一个治疗周期后，肝脏病变完全消失。治疗耐受性相对良好，毒性可接受。目前，自诊断后15个月及开始化疗后12个月，患者无疾病且健康状况良好。
尽管本研究仅涉及一名患者，但因其肿瘤罕见且文献中报道的完全缓解情况不常见而值得关注。治疗的有效性和可管理性表明，所采用的时机和方案可能构成针对这种侵袭性强且化疗反应差的肿瘤的一种重要治疗选择。
