Key result
A 35-year-old woman with neurofibromatosis-1 developed a multi-localized angiosarcoma as a second de novo malignancy, distinct from her previously excised malignant peripheral nerve sheath tumor.
Case Report (n=1)
This case report suggests that angiosarcoma in NF-1 patients can develop as a second de novo malignancy rather than exclusively arising from contiguous neurofibroma or MPNST lesions.
Raises possibility of de novo angiosarcoma in NF-1; leaves open need for broader malignancy surveillance.
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.
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Dubernard et al. (2019) conducted a case report in Neurofibromatosis-1 and Angiosarcoma (n=1). Neurofibromatosis-1 was evaluated on Histopathological diagnosis. A 35-year-old woman with neurofibromatosis-1 developed a multi-localized angiosarcoma as a second de novo malignancy, distinct from her previously excised malignant peripheral nerve sheath tumor.
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