Giant Congenital Nevus with Plexiform Neurofibroma and Malignant Peripheral Nerve Sheath Tumor

Abstract

A female patient with a giant congenital nevus associated with plexiform neurinoma and malignant peripheral nerve sheath tumor (MPNST) on head and neck is presented. We theoretically discuss possibilities of mutual origin all three entities, including the Boland theory of neurochristopaty. The clinical, surgical, and histologic aspects of the disease are discussed. Only one similar case in written literature was described.

Keywords :

• giant congenital nevus
• associated plexiform neurinoma
• malignant peripheral nerve sheath
• neurochristopaties