Multimodal treatment of abdominal and pelvic desmoplastic small round cell tumor with relative good prognosis.
OBJECTIVE
To investigate the clinicopathologic features and survival outcomes of desmoplastic small round cell tumor (DSCRT).


METHODS
The retrospective cohort study was performed on clinical and pathological data of 18 DSCRT patients. Among them, two subgroups were classified according to treatment modalities. 10 cases underwent operation and adjuvant chemotherapy (group 1, 10/18, 55.6%) and 8 cases were diagnosed by fine needle aspiration biopsy without surgical intervention (group 2, 8/18, 44.4%). All cases received six courses of multiple agents chemotherapy.


RESULTS
All cases were histologically confirmed as DSRCT and Cox regression revealed that sex, tumor localization and treatment modality affected patient outcomes. Kaplan-Meier analysis revealed that the median survival time was 22.0 ± 4.0 mo in group 1 versus 9.0 ± 0.7 mo in group 2.


CONCLUSIONS
DSRCT is highly aggressive malignance with poor prognosis, surgical excision with combination of chemotherapy can significantly improve the survival outcomes.
Hebei North University, Laboratory Medicine College, Zhangjiakou, Hebei Province, China.
Yuhong
Y
Tang
Department of Urology, The Second Hospital of Tianjin Medical University, Tianjin Institute of Urology, Tianjin, China.
Hualin
H
Song
Department of Pathology, The Second Hospital of Tianjin Medical University, Tianjin, China.
Yali
Y
Bao
Department of Ultrasound, Binzhou Medical University Hospital, Binzhou, Shandong Province 256600, China. Electronic address: suixiang1@126.com.
Yang
Y
Zhi
D000008:Abdominal Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D000293:Adolescent; D000328:Adult; D002648:Child; D002681:China / Q000453:epidemiology; D003131:Combined Modality Therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D055815:Young Adult
Chemotherapy; Desmoplastic small round cell tumor; Diagnosis; Prognosis; Surgery