Perineurioma

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Perineurioma is an uncommon benign peripheral nerve sheath tumour.

General

  • Benign tumour derived from perineurial cells (intraneural or soft tissue).
    • ICD-O 9571/0
    • WHO grade I
  • Rarely malignant soft tissue perineurioma.

Variant:

  • Reticular perineurioma.[1]

Macroscopy

  • Intraneural perineurioma: segmental tubular enlargement of the nerve.
  • Soft tissue perineurioma: Well circumscribed, but not encapsulated.

Microscopic

Features:[2]

  • Perineural epithelioid cells.
    • Abundant pale, fluffy appearing cytoplasm.

Note:

  • May be intraneural.[2]
    • Typical pseudo-onion bulbs.
    • Long considered hypertrophic neuropathy.
    • Rare (less than 1% of all nerve sheath tumours).

DDx:

Images:

IHC

Features:[3][4]

  • S100 -ve.
  • EMA +ve.
  • CD34 ~65% +ve.[3]

See also

References

  1. Graadt van Roggen, JF.; McMenamin, ME.; Belchis, DA.; Nielsen, GP.; Rosenberg, AE.; Fletcher, CD. (Apr 2001). "Reticular perineurioma: a distinctive variant of soft tissue perineurioma.". Am J Surg Pathol 25 (4): 485-93. PMID 11257623.
  2. 2.0 2.1 2.2 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 424. ISBN 978-0781779425.
  3. 3.0 3.1 Hornick, JL.; Fletcher, CD. (Jul 2005). "Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features.". Am J Surg Pathol 29 (7): 845-58. PMID 15958848.
  4. Tsang, WY.; Chan, JK.; Chow, LT.; Tse, CC. (Aug 1992). "Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma.". Am J Surg Pathol 16 (8): 756-63. PMID 1497116.