Complete response to chemotherapy in intra-abdominal desmoplastic small round cell carcinoma. A case report

Mario De Lena, Maria Lucia Caruso, Franco Marzullo, Sergio Mancarella, Raffaele Armentano, Vincenzo Ventrella, Michele Guida

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Aims and background: 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). Methods: 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- fluorouracilfolinic acid) for a total of 9 administrations. Results: 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. Conclusions: 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.

Original languageEnglish
Pages (from-to)412-416
Number of pages5
Issue number3
Publication statusPublished - May 1998


  • Chemotherapy
  • Intra-abdominal desmoplastic tumor

ASJC Scopus subject areas

  • Cancer Research


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