Malignant Peripheral Nerve Sheath Tumor of the Inguinum and Angiosarcoma of the Scalp in a Child with Neurofibromatosis Type 1

Abstract

Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first presented with MPNST of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp.

Angiosarcoma arising in a neurofibromatosis-1 patient as a de novo malignancy?

“…Only a few cases have been reported in the literature. [10][11][12][13][14][15][16][17] We report here the case of a 35-year-old woman known with a NF-1 disease, who developed a metastatic angiosarcoma, without MPNST components on histologic tumor examination.…”