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Updates on the multimodality management of desmoplastic small round cell tumor.

Kallianpur, Ashwin A; Shukla, Nootan K; Deo, Suryanarayan V S; Yadav, Praveen; Mudaly, Dillip; Yadav, Rajni; Palaniappan, Ramanathan M.

J Surg Oncol ; 105(6): 617-21, 2012 May.

Article in English | MEDLINE | ID: mdl-22215508

ABSTRACT

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and an aggressive malignancy with poor outcome. This tumor can co-express epithelial, neural, and mesenchymal markers. The molecular hallmark of DSRCT is the EWS-WT1 fusion protein. Despite the diversities in treatment modality, the best results have been seen with radical surgery and adjuvant or neoadjuvant chemotherapy.

Subject(s)

Abdominal Neoplasms/therapy , Desmoplastic Small Round Cell Tumor/therapy , Abdominal Neoplasms/genetics , Abdominal Neoplasms/mortality , Abdominal Neoplasms/pathology , Antibodies, Monoclonal/therapeutic use , Chemotherapy, Adjuvant , Chemotherapy, Cancer, Regional Perfusion , Desmoplastic Small Round Cell Tumor/genetics , Desmoplastic Small Round Cell Tumor/mortality , Desmoplastic Small Round Cell Tumor/pathology , Humans , Hyperthermia, Induced , Immunohistochemistry , Neoplasm Recurrence, Local , Neoplasm Staging , Oncogene Proteins, Fusion/genetics , Radiotherapy, Adjuvant , Tomography, X-Ray Computed , Translocation, Genetic

ABSTRACT

Subject(s)

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