Multiple isolated cutaneous plexiform schwannomas

Abstract

Plexiform schwannoma is a rare neurogenic tumor, arising from skin and subcutaneous tissue. The presence of multiple schwannomas suggests a possible association with neurofibromatosis type 2 (NF2). A 50-year old male patient presented with multiple papulo-nodular cutaneous lesions on both arms and forearms. Histopathological examination revealed a dermal multinodular pattern of well-circumscribed masses of closely packed cells, with peripheral myxoid tissue, well-encapsulated in a thin collagenous capsule. S-100 immunohistochemical staining was diffusely and strongly positive. Neuron-specific enolase was positive, confirming a neural tissue tumor. An audiogram and Magnetic Resonance Imaging (MRI) of cerebro-pontine angle showed no detected abnormality, excluding acoustic neuroma. Thus, we present a case of multiple bilateral isolated cutaneous plexiform schwannomas, not associated with NF2. Multiple plexiform schwannomas is a very rare entity, distinct from neurofibromatosis (NF), and being confined to the dermis is even more rarely reported.

Cutaneous schwannoma masquerading as trichilemmal cyst over scalp in a young male

“…[14] Plexiform schwannoma was first described by Masson, which he called “plexiform schwannogliosis”. [15] Few reports of malignant transformation in schwannomas have been reported in literature.…”

Enlarging Neck Mass in an Adolescent Boy

“…Surgical enucleation is the recommended treatment modality for schwannomas, and given the well‐encapsulated nature of these tumors, treatment typically leaves patients without neurologic deficits . Deficits seen in the immediate postoperative period have a high incidence of spontaneous resolution .…”