Child with Wilms' tumor and von Willebrand disease at diagnosis and apparent complete response to chemotherapy after multiple relapses
✍ Scribed by Barr, Ronald D.; Winthrop, Andrea; deSa, Derek; Gill, Gerald; Langer, Jacob; Fitzgerald, Peter; Whitton, Anthony C.
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 577 KB
- Volume
- 26
- Category
- Article
- ISSN
- 0098-1532
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✦ Synopsis
CR presented, as a 334-year-old girl in October 1989, with a large mass (measuring 11 X 12 X 13 cm on CT scan) in the right kidney. Approximately 2 years earlier she had undergone renal ultrasonography (for investigation of recurrent urinary tract infection) and no structural abnormalities were detected. The only unusual feature was the identification of acquired von Willebrand disease (VWD) which was demonstrated on routine pre-operative assessment in the absence of a hemorrhagic diathesis. Surgery was performed after the administration of DDAVP (to which a prior response had been demonstrated; see Table 1) and cryoprecipitate. Dr. Winthrop, please describe your operative findings.
Andrea Winthrop, MD (Pediatric Surgeon)
At nephrectomy , the mass was essentially replacing the right kidney but was mobile and did not involve the liver. The tumor capsule was intact and dissection was accomplished outside Gerota's fascia with complete resection of the tumor and residual kidney. An estimated