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Desmoplastic Small Round Cell Tumor (DSRCT) is a rare soft tissue tumor. Less than 300 cases have been reported in English literature to date and only more than 70 domestic cases have been reported. The onset, the biological entity, the diagnosis and standard treatment remain to be elucidated.Objective:Through analysis seven cases of DSRCT registered in the First Hospital Affiliated to Zhejiang University School of Medicine, in order to strengthen the understanding of DSRCT and then finally improve the effect of treatment based on domestic and foreign experience of DSRCT therapy. Methods:7 cases of DSRCT of treated in t the First Hospital Affiliated to Zhejiang University School of Medicine, from January 2003 to January 2010 were collected to this study including the clinical and pathological features, diagnosis and treatment and follow-up results. Results:7 cases treated in the hospital,6 male and 1 female (male:female= 6:1), age 16-44 years, median age 32 years,71.2% originated in the abdominal cavity. All 74 cased including other 67 patients reported in the domestic region, onset age 5-83 years, median 26 years, male 47 and female 20 (male:female 2.52:1),73.1% originated in the abdominal pelvic cavity. DSRCT in pathology, immunohistochemistry, genetics are typical manifestations for correct diagnosis. Above characteristics is similar to foreign reports. DSRCT is a highly malignent tumor so that clinical treatment is very difficult and the response rate is low. To date there is no uniform clinical treatment guidelines to reference. Current treatment options for the international mainstream is alkylating agent-based chemotherapy, the maximum cytoreductive surgery (> 90%) and the whole abdominal radiatherapy. Other 67 reported domestic cases were treated limited. In our 7 patients,6 patients underwent surgery,2 patients did not have further treatment due to personal reasons, and 1 was found rapid decline in platelet count (bone marrow biopsy indicated bone marrow metastasis) after operation which was given up further therapy. For the other 4 cases, we selected the chemotherapy (CT) VEC and/or IE regim based on foreign reports in the literature. All four cases of DSRCT were partial response (PR) 2 months after chemotherapy with good quality of life and low toxicity. Case 3 was given VEC before maximum cytoreductive surgery (> 90%), postoperative IE 3 cycles, and PR were acquired. It progressed 4 months later.If recurrence or refractory, there are rarely reported in the current articles. In 1999 Rosoff et al reported two cases and so retreated patients with CPT-11 monotherapy, although the clinical benefit, but little tumor shrinkage. In 2005, Bisogno et al reported three cases of retreatment patients with CPT-11 monotherapy,after 2month all PD.So we tried CPT-11 combined with CDDP in the case, later achieved PR more than a year with good quality of life of 14 months later, it progressed.On second-line treatment after failure of conventional chemotherapy in patients with DSRCT, rarely reported in the foreign literature. Successful application of targeted therapy such as Iressa and Bevacizumab attracted our attention. Thus, we tried docetaxel+nedaplatin+bevacizumab as a three-line treatment. Symptom relief was acquired 4 days later, but rapid deterioration 17 days later, It seemed no significant improvement after the second cycle of chemotherapy, The patient died of intestinal obstruction after 1 month.The majority of patients with DSRCT were poor prognosis. Ordonez et al reported 35 cases of DSRCT results in 25 patients with overall survival from 8 to 50 months, average 25.2 months,10 patients survived. In the domestic 74 cases including 7 cases in the hospital,47 patients with follow-up results reported average survival of 31 months (95% CI,17.2-44.8 months month), up more than 10 years. Survival analysis in domestic cases, the prognosis of patients is in accordance with complete surgical resection, originated in the abdominal cavity, chemotherapy.Conclusion:DSRCT is a rare, highly malignant invasive tumor, familiar to young men, mostly in the abdomen and pelvis. Correct diagnosis depends on pathological, immunohistochemical characteristics, further ectopic EWS-WT1 fusion gene detection needed if possible. A unified treatment of the standard maybe include alkylating agent-based chemotherapy, maximum tumor resection (90%), total abdominal radiotherapy. the prognosis of patients is in accordance with complete surgical resection, originated in the abdominal cavity, chemotherapy. Elevated CA125 is often accompanied in intra-abdominal DSRCT patients and maybe progressed with the disease.
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