title: Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
abstract: To investigate the clinical and computed tomography (CT) features of desmoplastic small round cell tumor (DSRCT), we retrospectively analyzed the clinical presentations, treatment and outcome, as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology. The CT manifestations of DSRCT were as follows: (1) multiple soft-tissue masses or diffuse peritoneal thickening in the abdomen and pelvis, with the dominant mass usually located in the pelvic cavity; (2) masses without an apparent organ-based primary site; (3) mild to moderate homogeneous or heterogeneous enhancement in solid area on enhanced CT; and (4) secondary manifestations, such as ascites, hepatic metastases, lymphadenopathy, hydronephrosis and hydroureter. The prognosis and overall survival rates were generally poor. Commonly used treatment strategies including aggressive tumor resection, polychemotherapy, and radiotherapy, showed various therapeutic effects. CT of DSRCT shows characteristic features that are helpful in diagnosis. Early discovery and complete resection, coupled with postoperative adjuvant chemotherapy, are important for prognosis of DSRCT. Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.
authors.affiliation: Xun-Ze Shen, Department of Radiology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China.
authors.forename: Xun-Ze
authors.initials: XZ
authors.lastname: Shen
authors.affiliation: Xun-Ze Shen, Department of Radiology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China.
authors.forename: Jian-Guo
authors.initials: JG
authors.lastname: Zhao
authors.affiliation: Xun-Ze Shen, Department of Radiology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China.
authors.forename: Jian-Jun
authors.initials: JJ
authors.lastname: Wu
authors.affiliation: Xun-Ze Shen, Department of Radiology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing 312000, Zhejiang Province, China.
authors.forename: Fang
authors.initials: F
authors.lastname: Liu
mesh_terms: D000008:Abdominal Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D058405:Desmoplastic Small Round Cell Tumor / Q000737:chemistry / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000556:secondary / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010386:Pelvic Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D011237:Predictive Value of Tests; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D018615:Ultrasonography, Doppler, Color; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: Clinical features; Computed tomography; Desmoplastic small round cell tumor; Pathology; Peritoneum