Presence of Malignant Peripheral Nerve Sheath Tumor in a Patient With Diffuse Neurofibromatosis-1; a Rare Case Report Publisher



Asilian A¹ ; Shoushtarizadeh M¹ ; Aminjavaheri M² Show Affiliations
Source: Iranian Journal of Dermatology Published:2024

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas, that impact approximately 0.001% of the population. Individuals with neurofibromatosis type 1 (NF1) have a 10% lifetime risk of developing these tumors, which account for up to 50% of all MPNST cases. A 49-year-old woman with diffuse NF1 was examined for a painful, growing, and hemorrhagic ankle lesion that had existed from the age of two. The mass was excised using Mohs surgery, and the pathology revealed a malignant peripheral nerve sheath tumor. This study presented a case of neurofibromatosis type 1, which was distinguished by a rare extent of lesion involvement throughout the body. The emergence of such a tumor in this condition is infrequent, even according to existing literature, making it a remarkable observation for expert dermatologists. © Iranian Journal of Dermatology.