Metastatic angiosarcoma arising in malignant peripheral nerve sheath tumor in a young patient with neurofibromatosis type 1. Issue 12 (26th May 2017)

• Record Type: Journal Article
• Authors: Winters, Amanda C.; Black, Jennifer O.; Cost, Carrye R.
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 12(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2017-05-26
• Subjects: angiosarcoma -- chemotherapy -- metastases -- MPNST -- NF1 -- PET/CT; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26643
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Neurofibromatosis type 1 (NF1) is a cancer predisposition syndrome with an incidence of approximately one in 3, 000 and a lifetime risk of malignancy estimated at 8–13%. Here, we report the case of a patient with NF1 who developed synchronous malignant peripheral nerve sheath tumors, one with a focus of angiosarcoma. He succumbed to metastatic angiosarcoma despite local resection and adjuvant chemotherapy. This case highlights the need for monitoring for malignancy in NF1 patients, the risks of sampling error during tumor biopsy, and the clinical decision ‐ making involved in choosing a therapeutic plan for a patient with multiple simultaneous malignancies.