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sclerosing perineurioma

Wednesday 17 December 2003

Cutaneous sclerozing perineurioma is a recently characterized, uncommon tumor composed of perineurial cells, which exhibits immunoreactivity for epithelial membrane antigen (EMA).

Images

- https://twitter.com/DrLaurenStuart/status/700401453865832448

Synopsis

- well-circumscribed masses
- thick collagen
- small, epithelioid cells exhibiting corded, trabecular and whorled growth patterns
- spindle cells with wavy nuclei end elongated cytoplasmic processes
- dense collagen stroma
- focal whorling formation

Histogenesis

- perineurial cells

Immunochemistry

- EMA + (epithelial membrane antigen +) (12836021)
- GLUT1 + (12836021)
- CD99 +
- vimentin +
- collagen type IV +
- CD10 +
- S-100 -
- smooth muscle actin - (SMA -)
- CD34 -
- cytokeratin - AE1-3-
- CD57 -

Epidemiology

- children and young adults
- dermal or subcutaneous nodules in the hands or palms
- rarely bilateral (11841380)

Predisposition

- neurofibromatosis type 2 (NF2)

Cytogenetics

- 46XX,del(10)(q22q24),der(10),del(22)(q11-12q?)/47, idem,+der(10)
- 46,XY,t(2;10)(p23;q24) (16096405)
- 47,XX,add(3)(q23),add(6)(q21),-5,-9,-10,-22,+mar1,+mar2,+mars (16096405)

Target regions

- 2p23
- 10q22-10q24

Molecular biology

- mutations in the NF2 gene (11290539, 10403310)

References

- Yamaguchi U, Hasegawa T, Hirose T, Fugo K, Mitsuhashi T, Shimizu M, Kawai A, Ito Y, Chuman H, Beppu Y. Sclerosing perineurioma: a clinicopathological study of five cases and diagnostic utility of immunohistochemical staining for GLUT1. Virchows Arch. 2003 Aug;443(2):159-63. Epub 2003 Jun 26. PMID: 12836021

- Balarezo FS, Muller RC, Weiss RG, Brown T, Knibbs D, Joshi VV. Soft tissue perineuromas in children: report of three cases and review of the literature. Pediatr Dev Pathol. 2003 Mar-Apr;6(2):137-41. Epub 2003 Jan 28. PMID: 12545405

- Fetsch JF, Miettinen M. 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. 1997 Dec;21(12):1433-42. PMID: 9414186