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Metastatic angiosarcoma arising in malignant peripheral nerve sheath tumor in a young patient with neurofibromatosis type 1
Author(s) -
Winters Amanda C.,
Black Jennifer O.,
Cost Carrye R.
Publication year - 2017
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26643
Subject(s) - medicine , neurofibromatosis , malignancy , angiosarcoma , malignant peripheral nerve sheath tumor , biopsy , radiology , cancer , nerve sheath neoplasm , incidence (geometry) , pathology , physics , optics
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.