title: [Analysis of the diagnosis and treatment of desmoplastic small round cell tumor].
abstract: OBJECTIVE
  To explore the clinical diagnostic features and treatment of desmoplastic small round cell tumor (DSRCT), and to improve the understanding and management of this tumor.
  
  
  METHODS
  The clinicopathological data of nine patients treated in our hospital from October 2004 to June 2014 were retrospectively analyzed and a review of the literature was made. The clinical manifestations, pathological characteristics, diagnosis and differential diagnosis, treatment and prognosis of this tumor were summarized and analyzed.
  
  
  RESULTS
  Nine patients with DSRCT, 5 males and 4 females, with an average age of 21 years (range 8-56 years) were included in this study. Ultrasound examination revealed irregular low-density mass shadow in the abdominal cavity. CT examination found that 6 cases had abdominal and retroperitoneal multiple solid tumor nodules, uneven density, and visible low density fluid area. Postoperative pathological examination revealed that the tumor cells were small, mostly elliptic, gathered to form clear structure of nests with clear irregular boundaries. The central portion of large tumor nests often showed necrosis. Scattered fibroblasts and large amount of hyalinization of collagen fibers were seen in the interstitial tissue around the nests. Six patients received laparotomy surgery, however, all failed to resect the tumor completely. Three patients received postoperative chemotherapy, i. e. two cases had carboplatin and paclitaxel chemotherapy, and one case of chemotherapy regimen not specified. Two patients had radiation and chemotherapy (no concrete plan was available). Another case was lost to follow-up. Two of the three patients without surgery received chemotherapy with CAP (cyclophosphamide+adriamycin+carboplatin) and total rectal lesions, pelvic and inguinal lymph nodes, ilium metastases radiation therapy. Another one patient received EP regimen (DDP+VP16) which was then changed into a TP chemotherapy alone. Eight of the nine cases died shortly after surgery, and only one patient treated with chemotherapy alone was still alive after 11 months of follow-up.
  
  
  CONCLUSIONS
  Desmoplastic small round cell tumor is a very rare, special type of soft tissue tumor, with very poor prognosis. This tumor may be preliminarily diagnosed according to the imaging characteristics and detection of tumor markers, however, final diagnosis is made by pathology. Surgery is the priority of treatment, combined with complementary radiation and chemotherapy.
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Baojian
authors.initials: B
authors.lastname: Lu
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Zhiqun
authors.initials: Z
authors.lastname: Shang
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Erlin
authors.initials: E
authors.lastname: Sun
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Xuewu
authors.initials: X
authors.lastname: Nian
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Jingda
authors.initials: J
authors.lastname: Gao
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China.
authors.forename: Chengquan
authors.initials: C
authors.lastname: Ma
authors.affiliation: Department of Urology, Second Hospital of Tianjin Medical University, Tianjin Urology Institute, Tianjin Key Laboratory of Basic Urology, Tianjin 300211, China; Email: Han-ruifa@hotmail.com.
authors.forename: Ruifa
authors.initials: R
authors.lastname: Han
mesh_terms: D000008:Abdominal Neoplasms / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D003131:Combined Modality Therapy / Q000379:methods; D003520:Cyclophosphamide / Q000008:administration & dosage; D058405:Desmoplastic Small Round Cell Tumor / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017239:Paclitaxel / Q000008:administration & dosage / Q000032:analysis; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D014408:Biomarkers, Tumor; D004317:Doxorubicin; D003520:Cyclophosphamide; D016190:Carboplatin; D017239:Paclitaxel