Perineurioma

From Libre Pathology
Jump to navigation Jump to search

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.
  • Sclerosing perineurioma: Predilection for hands.[2]

Microscopic

Features:[3]

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

Note:

  • May be intraneural.[3]
    • Typical pseudo-onion bulbs.
    • Long considered hypertrophic neuropathy.
    • Rare (less than 1% of all nerve sheath tumours).

DDx:

Images:

IHC

Features:[4][5]

  • S100 -ve.
  • EMA +ve.
  • CD34 ~65% +ve.[4]

Molecular

  • Frequent TRAF7 mut.[6]

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. "Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults". Am. J. Surg. Pathol. 21 (12): 1433–42. December 1997. doi:10.1097/00000478-199712000-00005. PMID 9414186.
  3. 3.0 3.1 3.2 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.
  4. 4.0 4.1 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.
  5. 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.
  6. "Genomic analysis reveals frequent TRAF7 mutations in intraneural perineuriomas". Ann. Neurol. 81 (2): 316–321. February 2017. doi:10.1002/ana.24854. PMC 5725965. PMID 28019650. //www.ncbi.nlm.nih.gov/pmc/articles/PMC5725965/.