Perineurioma: a clinicopathological study of eight cases.

Aims: Perineuriomas (PN) are uncommon, benign neoplasms that mimic a number of benign and malignant soft tissue lesions. There are two main forms: a rare intraneural PN (IPN), and a relatively more common extraneural soft tissue PN (STPN) including a conventional form (STPNc), sclerosing (SPN), reticular and lipomatous variants. Their diagnosis requires immunohistochemical (IHC) and/or ultrastructural (US) confirmation of perineurial cell differentiation. This study aims to review the clinicopathological features of eight PN we encountered recently, to raise awareness of PN as an entity and to highlight the differential diagnoses which include potentially aggressive lesions.

Methods: Clinical, histological, IHC and US features of seven STPN and one IPN were studied.

Results: The eight PN arose in the limbs of six females and two males aged 30-58 years. Five STPN occurred in subcutis, one intramuscularly and one intradermally. The STPN were well-circumscribed, multinodular growths. STPNc contained bland spindle cells with long cytoplasmic processes arranged in lamellae, storiform patterns and whorls. Three SPN were acrally located and additionally contained small epithelioid cells in cords and clusters in a myxohyaline stroma with extensive sclerosis. One SPN had giant collagen rosettes of spiral collagen. The IPN showed 'pseudo-onion bulbs' of perineurial cells. All PN were at least focally EMA positive, six of eight were Claudin-1 positive and all were S100 protein negative. Common US features were organelle-poor cell processes, many pinocytotic vesicles, sparse intermediate filaments, and tight junctions and patchy external lamina. There were no recurrences (follow-up 1-49 months).

Conclusion: PN has a variable morphology and can mimic many benign, borderline and malignant lesions, the differential diagnoses of which are discussed. When confronted with a subcutaneous (in particular) spindle and/or epithelioid cell lesion, EMA/Claudin-1 stains and/or US are essential to identify PN and thereby avoid inappropriately aggressive therapy.