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Research progresses of the treatment of desmoplastic small round cell tumor

 XU Xiao1,2, ZHANG Feng-chun1，3, XU Ying-chun4   

  1. 1.Department of Oncology, Kowloon Hospital, Medical College of Shanghai Jiao Tong University, Suzhou 215021, China; 2.Department of Radiation, First Hospital of Guangxi Medical University, Nanning 530021, China; 3.Department of Oncology, Ruijin Hospital，Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China; 4.Department of Oncology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
  • Online:2016-05-28 Published:2016-05-26
  • Supported by:

    National Natural Science Foundation of China, 81301858

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Abstract:

Desmoplastic small round cell tumor (DSRCT) is a rare, highly malignant tumor with very poor prognosis. It often develops in the abdominal and pelvic peritoneum or extra-abdominal sites of adolescents and young males. Patients typically present with symptoms of abdominal sarcomatosis. The histopathological manifestation is featured with nest-distributed small round blue cells surrounded by fibrous tissue. This tumor can co-express epithelial, neural and mesenchymal markers. The molecular characteristic of DSRCT is the production of EWS-WT1 fusion protein via the translocation of chromosome t(11:22) (p13; q12). Standard management of DSRCT has not been established so far. Treatments include debulking or radical surgery, high intensity adjuvant or neoadjuvant polychemotherapy, and whole abdominal radiation. Some studies suggested that methods such as hematopoietic stem cell transplantation, targeted therapies, and postoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and intensity-modulated radiation therapy (IMRT) can improve survival. This paper reviews recent evidence-base medicine supported options for the treatment of DSRCT.

Key words: desmoplastic small round cell tumor; , diagnosis; , treatment