title: Desmoplastic small round cell tumor of the abdomen and pelvis: clinicopathological characters of 12 cases.
abstract: OBJECTIVE
  To study the clinical, radiological, and pathological characteristics of abdominal desmoplastic small round cell tumor (DSRCT) and investigate the optimal therapy modalities.
  
  
  METHODS
  A retrospective cohort study was performed on 12 abdominal DSRCT patients; all pathological, radiological, and prognostic data were analyzed. There were 3 patients (25%) with metastatic disease at presentation. In all 12 cases, 6 cases underwent operation and adjuvant chemotherapy (group 1, 6/12, 50%). The other 6 cases were diagnosed by fine needle aspiration or exploratory laparotomy biopsy (group 2, 6/12, 50%); all cases received four to six courses of multiple agents chemotherapy, respectively.
  
  
  RESULTS
  All cases were finally diagnosed as DSRCT pathologically. Among group 1, all cases underwent en bloc resection (2/6, 33%) or tumor debulking (4/6, 67%) and, following four courses of multiple agents chemotherapy, Kaplan-Meier analysis revealed that 3-year survival was 50% in group 1 versus 16.7% in group 2 (P < 0.05). Gross tumor resection was highly significant in prolonging overall survival; patients with localized solitary lesion have a better prognosis, most likely due to increased feasibility of resection.
  
  
  CONCLUSIONS
  DSRCT is a rare malignant tumor with poor prognosis. Surgical excision with combination chemotherapy as an adjunct is mandatory for nonmetastatic cases because these modalities used in isolation may have less impact.
authors.affiliation: Department of General Surgery, Wuqing District People's Hospital, Tianjin 301700, China.
authors.forename: Guangzhao
authors.initials: G
authors.lastname: Zhang
authors.affiliation: Department of General Surgery, Wendeng Central Hospital, Shandong, China.
authors.forename: Guangjun
authors.initials: G
authors.lastname: Liu
authors.affiliation: Department of Pathology, Affiliated Hospital of Binzhou Medical University, Binzhou, Shandong, China.
authors.forename: Dahua
authors.initials: D
authors.lastname: Zhao
authors.affiliation: Department of General Surgery, Wendeng Central Hospital, Shandong, China.
authors.forename: Xijun
authors.initials: X
authors.lastname: Cui
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Institute of Urology, Tianjin 300211, China.
authors.forename: Gang
authors.initials: G
authors.lastname: Li
mesh_terms: D000005:Abdomen / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D001706:Biopsy; D003131:Combined Modality Therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D010388:Pelvis / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult