COMPLETE RESPONSE TO CHEMOTHERAPY IN INTRAABDOMINAL DESMOPLASTIC SMALL ROUND-CELL CARCINOMA - A CASE-REPORT

Aims and background: The authors report the case of a 23-year-old woma n affected by intra-abdominal desmoplastic small round cell tumor (DSR CT) who obtained a complete response to multiagent chemotherapy, DSRCT is a rare,highly aggressive neoplasm generally arising in young peopl e and seldom in females (about 20 cases described in the literature). Methods: The patient underwent surgical resection of a large 15 x 15 c m mass located in the right lower abdominal quadrant, but after only 2 months later, two liver metastasis were noted. Thus, she was subjecte d to an aggressive antineoplastic treatment consisting of three groups of alternating non-cross resistant multiagent regimens administered e very 21 days (cis-platin-etoposide-adriamycin-bleomicin; gemcitabine-i fosfamide-dacarbazine; methotrexate-5-fluorouracil-folinic acid) for a total of 9 administrations. Results: After one cycle of treatment inc luding the administration of all the three alternated schemes of chemo therapy, a complete disappearance of liver disease was noted, The trea tment was relatively well-tolerated and the toxicity was acceptable. A t present, after 15 months from diagnosis and 12 months after starting chemotherapy, the patient is disease-free and in good health. Conclus ions: Even though this study regards only a single patient, it is note worthy because of the rarity of this neoplasm and because of the infre quent complete responses reported in the literature, The efficacy and manageability of the treatment, suggests that both the timing and sche dule used could constitute an important therapeutical option for this aggressive and poorly Chemo-responsive tumor.