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

Background: Neurofibromatosis-1 (NF-1) is the most common genetic disease belonging to the group of neural tissue cell growth disorders, known to be complicated by malignant peripheral nerve sheath tumors or MPNST. On the contrary, angiosarcoma is rarely associated with NF-1. Only few cases reported angiosarcoma arising as a sarcomatous transformation within a preexisting MPNST.Methods and results: We report here the remarkable case of a 35-year-old NF-1 woman, with a history of excised MPNST 8 years before a new hospitalization for the development of multi localized angiosarcoma, whose discovery is related to an oropharyngeal involvement, with a dramatic presentation. Review of the literature and differences from previous reports are discussed.Conclusion: Our case report supports the hypothesis of an angiosarcoma developing as a malignant proliferation apparently not arising from contiguous neurofibroma or MPNST lesions, but representing a second de novo malignancy.