Difference between revisions of "Perineurioma"

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(molecular)
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*EMA +ve.
*EMA +ve.
*CD34 ~65% +ve.<ref name=pmid15958848/>
*CD34 ~65% +ve.<ref name=pmid15958848/>
==Molecular==
*Frequent TRAF7 mut.<ref name="pmid28019650">{{cite journal |vauthors=Klein CJ, Wu Y, Jentoft ME, Mer G, Spinner RJ, Dyck PJ, Dyck PJ, Mauermann ML |title=Genomic analysis reveals frequent TRAF7 mutations in intraneural perineuriomas |journal=Ann. Neurol. |volume=81 |issue=2 |pages=316–321 |date=February 2017 |pmid=28019650 |pmc=5725965 |doi=10.1002/ana.24854 |url=}}</ref>


==See also==
==See also==

Revision as of 08:25, 10 September 2020

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]

Molecular

  • Frequent TRAF7 mut.[5]

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.
  5. "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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5725965/.