Clinical Characteristics And Treatment Of Desmoplastic Small Round Cell Tumor

Purposes:This retrospective study was performed to evaluate the clinical characteristics and treatment of desmoplastic small round cell tumor.Methods:We retrospectively analyzed12patients with DSRCT diagnosed and treated in The First Affiliated Hospital of Zhejiang University School of Medicine between January,2003and January,2014. Data were collected on patient demographics, presenting symptoms, treatment regimen, progression free survival and overall survival.Results:A majority of patients were male (91.7%), with a median age of31(16-18) years old at diagnosis. The most common presenting complaint was abdominal distension (50%), mass (42%), constipation (33%). Most primary tumors were located in abdomen or pelvis (75%).3cases of extra-abdomen tumors were in testicle, lungs and adrenal gland respectively. The median tumor diameter was7.5cm (4-18cm).4cases (33.3%) had been distant metastases at diagnosis,3cases in liver and spleen. Serum CA125was elevated in9cases (81.8%), decreased to normal level after effective treatment, and increased significantly before disease progression proved by imaging. Overall,1-year,2-year,3-year and5-year survivals were75%,41.7%,16.7%and0%, respectively.5cases who received complete or debulking surgery relapsed after a median7.8months (7.5-10.1months). For the first-line chemotherapy, the ORR was85.7%, the median PFS was9.5months (0.9-13.5months). For the recurrence disease, a combination chemotherapy regimen included irinotecan, the ORR was66.7%, PFS was15.4and10.8months respectively; a combination chemotherapy regimen included docetaxel, the ORR was14.3%, PFS was15.4months; The DCR of Sunitinib was66.7%. The median OS was24months in patients received chemotherapy compared with6month in no chemotherapy, p=0.001. The median OS was no difference between patients with surgery and no surgery. The median OS was19months in patients received surgery and chemotherapy combination compared with10months in palliative surgery only, p=0.022, and24months in chemotherapy only, no statistical discrepancy.Conclusions:DSRCT has a poor prognosis, serum CA125might be a potential marker for predicting effectiveness of treatments and suggesting the progression of disease. Chemotherapy, and surgery combined with chemotherapy may prolong survival. Recurrent patients received Sunitinib or a combination chemotherapy regimen of irinotecan or docetaxel may have a benefit survival.