Benign spindle cell tumor with morphological and immunophenotypical features (EMA+, claudin1+, S100-, pancytokeratin-, alpha smooth muscle actin-) consistent with soft tissue perineurioma, extending to all the specimen margins. A perineurioma is a rare, non-cancerous tumour that arises from perineurial cells, which are part of the protective sheath that surrounds nerves. These tumours belong to a group of tumours called peripheral nerve sheath tumours.

Understanding the Context

Perineurioma is an uncommon benign peripheral nerve sheath tumour composed exclusively of perineural cells. Perineurioma tumour cells are spindle shaped, with long delicate cell processes amidst a collagenous stroma (figure 1). Perineurioma. This rare benign peripheral nerve tumor arises from perineurial cells, a type of cell that surrounds the peripheral nerve sheath.

Key Insights

A perineurioma can form within a nerve, called an intraneural perineurioma. It also may form in soft tissue next to a nerve, called an extraneural perineurioma. Perineurioma is another name for schwannoma. Perineuriomas are benign tumors. Perineuriomas most frequently occur on the scalp and forehead.

Final Thoughts

Perineuriomas are pathognomonic for neurofibromatosis type 1. A perineurioma is a rare, non-cancerous growth that develops from the protective coverings of nerves. This unique tumor affects the intricate network of nerves throughout the body, potentially leading to various symptoms depending on its location. This study presents a case of colonic perineurioma in the descending colon and reports a systematic review consolidated with a descriptive and subgroup analysis of the included cases and case series of perineurioma and benign fibroblastic polyps in the GI tract. Perineuriomas are rare benign nerve sheath tumours with a slow progression, sometimes difficult to diagnose. Decompression and neurolysis may improve neurological deficits.

High resolution nerve sonography might serve as a helpful additional diagnostic tool in this process. Perineuriomas of soft tissue usually present as painless masses. Subcutaneous tissue is involved more often than deep soft tissue. About 10% of cases are limited to the dermis.