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Histopathological Analysis and Diagnosis of Perineurioma in a Young Female Patient

A 27-year-old female presented with progressive muscle weakness in her right arm. An EMG indicated a "right ulnar blockage," and MRI revealed an enlarged radial nerve, leading to surgical excision. Histological examination showed an abnormal segment of peripheral nerve with onion-bulb-like changes, featuring bland, elongated cells arranged in parallel bundles without atypia or mitotic figures. Immunohistochemical stains (S100, EMA, neurofilament) further characterized the lesion, confirming a diagnosis of perineurioma, classified as WHO grade 1.

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Histopathological Analysis and Diagnosis of Perineurioma in a Young Female Patient

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  1. Case Study 88Leonidas Arvanitis, MD

  2. Question 1: • A 27-year-old female complains of progressive muscle weakness of her right arm • An EMG performed was suggestive of a “right ulnar blockage • An MRI of the right arm showed an enlarged radial nerve • A surgical excision was performed • Describe the histologic findings on the H&E stained section

  3. Answer 1: • Abnormal segment of peripheral nerve with diffuse onion-bulb-like change • The cells appear bland, elongated and arranged in parallel bundles • No atypia or mitotic figures are identified

  4. Question 2: • What other stains would be useful to make an accurate diagnosis of this lesion?

  5. Answer 2: • S100 • EMA • Neurofilament • Click here to vie S100, EMA and NF stains

  6. Question 3: • Describe the findings seen on the immunohistochemical stains

  7. Answer 3: • S100 • Stains the axons in the center of the onion bulbs • EMA • The cells show diffuse membranous staining • Neurofilament • Staining is limited to enwrapped nerve fibers

  8. Question 4: • Based on the histologic features and immunohistochemical profile, what is the most likely diagnosis?

  9. Answer 4: • Perineurioma

  10. Question 5: • How many subtypes of perineurioma exist?

  11. Answer 5: • Intraneural perineurioma • Soft tissue perineurioma • Which includes the sclerosing type variant that typically presents as a solitary nodule on the hand

  12. Question 6: • What WHO grade does intraneural perineurioma correspond to?

  13. Answer 6: • WHO grade 1 • Click here to view a low proliferation index with a Ki67 immunostain

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