Alveolar soft part sarcoma of the bladder with ASPSCR1-TFE3 gene fusion as a secondary malignancy Journal Article


Authors: Rhee, D. S.; Dunkel, I. J.; Shukla, N. N.; Walsh, M. F.; Gilheeney, S. W.; Price, A. P.; Antonescu, C. R.; Heaton, T. E.
Article Title: Alveolar soft part sarcoma of the bladder with ASPSCR1-TFE3 gene fusion as a secondary malignancy
Abstract: Alveolar soft part sarcoma (ASPS) represents <1% of all soft tissue sarcomas and harbors the ASPSCR1-TFE3 translocation, which is found in pediatric renal cell carcinomas arising after chemotherapy. We present the case of a female patient, treated for metastatic retinoblastoma (Rb) with surgery, radiation, and chemotherapy at age 21 months, who was diagnosed with ASPS of the bladder 5 years later when imaging revealed a polypoid mass arising from the left bladder wall. Endoscopic biopsy and tumor resection were performed. After histopathologic confirmation of ASPSCR1-TFE3 fusion-positive ASPS, negative margins were achieved with wide local excision. At 18 months post-surgery, she remains recurrence-free. © 2017 The Authors
Keywords: retinoblastoma; bladder tumor; alveolar soft part sarcoma; secondary malignancy; aspscr1-tfe3 translocation
Journal Title: Journal of Pediatric Surgery Case Reports
Volume: 27
ISSN: 2213-5766
Publisher: Elsevier Inc.  
Date Published: 2017-12-01
Start Page: 19
End Page: 22
Language: English
DOI: 10.1016/j.epsc.2017.09.007
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 2 October 2017 -- Source: Scopus
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MSK Authors
  1. Anita P Price
    53 Price
  2. Ira J Dunkel
    371 Dunkel
  3. Cristina R Antonescu
    895 Antonescu
  4. Neerav Shukla
    159 Shukla
  5. Todd Erin Heaton
    44 Heaton
  6. Michael Francis Walsh
    156 Walsh
  7. Daniel Rhee
    2 Rhee