Cutaneous malignant peripheral nerve sheath tumor – A case report and literature review

Abstract

Primary occurrence of Malignant Peripheral Nerve Sheath Tumor (MPNST) in the skin is rare. Superficial primary MPNST with a cutaneous or subcutaneous origin represents a small subset of MPNST thought to be derived from cutaneous neurofibromas or small peripheral nerves Cutaneous MPNST. We report a case of a 36-year-old man with no history of neurofibromatosis presented to the surgical clinic with a swelling and an infection over his Left thumb for six months. Clinically, there was concern over the Panaritium. An excisional biopsy was performed and revealed a Cutaneous MPNST. Tumor composed of malignant epithelioid and spindle cells arising in the dermis and subcutis. The malignant cells were positive for Vimentin, S-100, SOX10, CD99, β-Catenin, CD10, SMA, H3K27me3, P53, They were negative for Desmin, CD34 (-), ERG, Fli1, H-Cald , MyoD1, Myogenin, Pan-mel, TLE1, NF, ALK, HMB45 and MelanA. The Ki-67 labelling index was 40%. Pulmonary metastasis, Pancreatic metastasis, Left Axillary metastasis, and Left Elbow fossa metastasis were confirmed by PET-CT two months after tumor tissue had been excised. Five courses of chemotherapy and radiation have been offered. He remained well with no symptoms or development at five months of follow-up.

Keywords

• Cutaneous MPNST
• Cutaneous conventional MPNST
• S-100
• SOX10
• H3K27me3