Perineuriomas are uncommon benign peripheral nerve sheath tumors that include soft tissue, sclerosing, and intraneural variants.
Perineuriomas are rare tumors of the peripheral nerve sheath that share some characteristics with meningioma.
Synopsis
age: mean age, 46 years; range, 10-79 years
tumor size: from 0.3 to 20 cm (mean, 4.1 cm)
anatomic distribution: lower limb, upper limb, trunk, head and neck, retroperitoneum, paratesticular
subcutis, deep soft tissue, limited to the dermis
grossly well circumscribed
focal microscopically infiltrative margins
storiform growth pattern and focally whorled growth pattern
fascicular areas
collagenous stroma or myxoid stroma or mixed collagenous and myxoid stroma
mitoses: from 0 to 13 per 30 high power fields (mean, 1)
Variants
atypical perineuriomas
- scattered pleomorphic cells
- abrupt transition from typical morphology to a markedly hypercellular, fascicular area with cytologic atypia
- diffuse infiltration of skeletal muscle
Immunochemistry
epithelial membrane antigen (EMA+)
CD34+ (64%)
claudin-1 (29%)
+/- smooth muscle actin (21%)
+/- S-100 protein (5%)
negative
- glial fibrillary acidic protein
- neurofilament protein
- desmin
Prognosis
rare local recurrence
Predisposition
neurofibromatosis type 2 (NF2 syndrome) (17122521)
Differential diagnosis
meningioma
schwannoma
neurofibroma
Cytogenetics
46,XX,t(8;9)(q13;q22) (16096405)
References
Brock JE, Perez-Atayde AR, Kozakewich HP, Richkind KE, Fletcher JA, Vargas SO. Cytogenetic aberrations in perineurioma: variation with subtype. Am J Surg Pathol. 2005 Sep;29(9):1164-9. PMID: 16096405
Hornick JL, Fletcher CD. Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features. Am J Surg Pathol. 2005 Jul;29(7):845-58. PMID: 15958848
Balarezo FS, Muller RC, Weiss RG, Brown T, Knibbs D, Joshi VV. Soft tissue perineuriomas in children: report of three cases and review of the literature. Pediatr Dev Pathol. 2003 Mar-Apr;6(2):137-41. PMID: 12545405
Folpe AL, Billings SD, McKenney JK, Walsh SV, Nusrat A, Weiss SW. Expression of claudin-1, a recently described tight junction-associated protein, distinguishes soft tissue perineurioma from potential mimics. Am J Surg Pathol. 2002 Dec;26(12):1620-6. PMID: 12459629
