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Presented by Hind Nassar, M.D. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 1: 8 year old boy with ear mass.
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Question 1 of 1
1. Question
Week 379: Case 1
8 year old boy with ear massimages/nassar012609-1a.jpg
images/nassar012609-1b.jpg
images/nassar012609-1c.jpgCorrect
Answer: Plexiform schwannoma
Histology: see below
Discussion: Plexiform schwannoma is a benign peripheral nerve sheath tumor composed of Schwann cells, which is defined by a plexiform or multinodular growth pattern. It usually occurs in children and young adults and is most often located within the dermis and subcutis, although deep seated lesions have been described. Unlike plexiform neurofibroma, plexiform schwannoma is typically not associated with neurofibromatosis. In some cases, mainly in children, the lesion can be cellular with mild atypia and few mitotic figures, these lesions have been considered MPNST in the past. However based on immunohistochemistry (diffusely positive for S100) and electron microscopy (diffuse Schwann cells) and their indolent behavior and inability to metastasize these lesions are classified as schwannomas. Plexiform schwannoma can locally recur, however, due to the irregular multinodularity obscuring limits of tumor margins at the time of resection, warranting a wider excision.
Incorrect
Answer: Plexiform schwannoma
Histology: see below
Discussion: Plexiform schwannoma is a benign peripheral nerve sheath tumor composed of Schwann cells, which is defined by a plexiform or multinodular growth pattern. It usually occurs in children and young adults and is most often located within the dermis and subcutis, although deep seated lesions have been described. Unlike plexiform neurofibroma, plexiform schwannoma is typically not associated with neurofibromatosis. In some cases, mainly in children, the lesion can be cellular with mild atypia and few mitotic figures, these lesions have been considered MPNST in the past. However based on immunohistochemistry (diffusely positive for S100) and electron microscopy (diffuse Schwann cells) and their indolent behavior and inability to metastasize these lesions are classified as schwannomas. Plexiform schwannoma can locally recur, however, due to the irregular multinodularity obscuring limits of tumor margins at the time of resection, warranting a wider excision.
