[Clinical characteristics and treatment of desmoplastic small round cell tumor].
OBJECTIVE
To investigate the clinical characteristics and treatment of desmoplastic small round cell tumor.


METHODS
Five patients with DSRCT were diagnosed and treated in our Hospital from January 1999 to May 2009. Forty-eight cases with complete clinical data were collected and reviewed from 23 published reports. Therefore totally 53 patients with DSRCT were analysed. The survival rate was calculated by Kaplan-Meier method and compared by log-rank test.


RESULTS
The median age of all cases was 23 (1.5 - 66) years old at the time of diagnosis. 75.5% of patients were male. The most common presenting complaint was intra-abdominal mass or pain (77.4%). In 46 patients (86.8%), the primary tumor was located in the abdomen or pelvis. Fifteen (28.3%) had positive lymph nodes or distant parenchymal metastases. The median follow-up was 1.8 years (range, 0.1 - 10.0 years). The overall 1-, 3- and 5-year survivals were 45.8%, 20.8% and 5.7%, respectively. Forty-seven patients underwent surgery. Complete tumor resection was significantly correlated with long survival. The 1- and 3-year survival rates were 70.5% and 53.7% in patients treated with complete tumor resection compared to 37.2% and 4.8% in the incomplete tumor resection cohort (P = 0.0020). Thirty-four patients received chemotherapy and the 1- and 3-year survival rates were 60.1% and 35.2%, respectively, however, only 29.7% and 12.7% in patients without chemotherapy (P = 0.0396). Twelve patients had radiotherapy and the 1- and 3-year survival rates were 75.0% and 38.9%, respectively, compared with 36.9% and 14.8% in those without radiotherapy (P = 0.0314).


CONCLUSIONS
Complete tumor resection results in improved survival in patients with DSRCT. Chemotherapy and radiotherapy correlate with improved patient outcome. Multimodal therapy may improve the survival in patients with DSRCT.
Xing
Pu-yuan
PY
Department of Medical Oncology, Cancer Institute & Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Shi
Yuan-kai
YK
Feng
Feng-yi
FY
Qin
Yan
Y
Liu
Peng
P
D000008:Abdominal Neoplasms / Q000473:pathology* / Q000628:therapy*; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000556:secondary; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D058405:Desmoplastic Small Round Cell Tumor / Q000473:pathology* / Q000556:secondary / Q000628:therapy*; D004317:Doxorubicin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D008113:Liver Neoplasms / Q000556:secondary; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D011471:Prostatic Neoplasms / Q000473:pathology / Q000628:therapy; D020266:Radiotherapy, Conformal; D013514:Surgical Procedures, Operative; D015996:Survival Rate; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D007069:Ifosfamide