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Epithelial sheath neuroma

Authors: Dr Akshay Flora, Resident Medical Officer, Royal Prince Alfred Hospital, University of Sydney, Central Clinical School, Sydney, NSW, Australia; Dr Roger (Hyun Joon) Kim, Anatomical Pathology Registrar, Douglas Hanly Moir Pathology, Sydney, NSW, Australia. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. June 2020.


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What is an epithelial sheath neuroma?

Epithelial sheath neuroma is an uncommon benign cutaneous tumour composed of dermal nerve fibres surrounded by squamous epithelium [1,2].

Who gets epithelial sheath neuroma?

Epithelial sheath neuroma has so far only been reported in 13 people; all were older than 40 years of age. It is more common in females [1].

What causes an epithelial sheath neuroma?

The pathogenesis of epithelial sheath neuroma has not been fully established. However, several possible mechanisms have been suggested.

  • A reactive hyperplasia of peripheral nerves and epithelium in response to an external stimulus or to entrapment of epithelium within the perineurium (a sheath of connective tissue) [3]
  • Squamous metaplasia of the perineurium of enlarged nerve bundles as a reaction to localised inflammation [4]
  • Neural crest remnants from embryonal development that differentiated into neural and squamous epithelial elements [5]
  • Interleukin 6-mediated hyperplasia in response to localised inflammation or minor trauma at the site of a previous skin biopsy [6].

What are the clinical features of epithelial sheath neuroma?

Epithelial sheath neuroma presents as a persistent erythematous papule or nodule located on the upper or mid-back. Some patients report tenderness, pruritus, or paraesthesia when the lesion is palpated [1].

How is epithelial sheath neuroma diagnosed?

Epithelial sheath neuroma is diagnosed by histopathological examination of a skin biopsy. It is characterised by multiple enlarged peripheral nerve fibres that are sheathed by mature squamous epithelium. Epithelial sheath neuroma is sometimes surrounded by myxoid (mucus-like) stroma and a lymphocytic infiltrate.

Histology of epithelial sheath neuroma

What is the differential diagnosis for epithelial sheath neuroma?

The clinical differential diagnosis for epithelial sheath neuroma includes:

The histological differential diagnosis for epithelial sheath neuroma includes reactive neuroepithelial aggregates, perineural invasion of a well-differentiated cutaneous carcinoma, or after a previous biopsy [7,8].

What is the treatment and outcome of epithelial sheath neuroma?

Epithelial sheath neuroma is benign. The treatment of choice is excision [1].

To date, there have been no reports of recurrence after excision [1].

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References

  1. Flora A, Kim RH, Lara Rivero AD, Carr U, Isaacs F. Epithelial sheath neuroma: A case series. JAAD Case Reports 2020; 6: 240–2. doi: 10.1016/j.jdcr.2020.01.018. PubMed Central
  2. Requena L, Grosshans E, Kutzner H, Ryckaert C, Cribier B, Resnik KS, LeBoit PE. Epithelial sheath neuroma: a new entity. Am J Surg Pathol 2000; 24: 190–6. doi: 10.1097/00000478-200002000-00004. PubMed
  3. Zelger BG, Zelger B. Epithelial sheath neuroma: a benign neoplasm? Am J Surg Pathol 2001; 25: 696–8. doi: 10.1097/00000478-200105000-00024. PubMed
  4. Kutzner H. For Valentine's Day. Cancer 2001; 91: 804–5. doi: 10.1002/1097-0142(20010215)91:4<804::Aid-cncr1067>3.0.Co;2-t. PubMed
  5. Dunn M, Morgan MB, Beer TW, Chen KT, Acker SM. Histologic mimics of perineural invasion. J Cutan Pathol 2009; 36: 937–42. doi: 10.1111/j.1600-0560.2008.01197.x. PubMed
  6. Wang JY, Nuovo G, Kline M, Magro CM. Reexcision Perineural Invasion and Epithelial Sheath Neuroma Possibly on a Spectrum of Postinjury Reactive Hyperplasia Mediated by IL-6. Am J Dermatopathol 2017; 39: 49–52. doi: 10.1097/DAD.0000000000000671. PubMed
  7. Chen KT. Reactive neuroepithelial aggregates of the skin. Int J Surg Pathol 2003; 11: 205–10. doi: 10.1177/106689690301100307. PubMed
  8. Stern JB, Haupt HM. Reexcision perineural invasion. Not a sign of malignancy. Am J Surg Pathol 1990; 14: 183–5. doi: 10.1097/00000478-199002000-00010. PubMed

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