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Extra‐acral cutaneous/soft tissue sclerosing perineurioma: an under‐recognized entity in the differential of CD34‐positive cutaneous neoplasms
Author(s) -
Fox Melanie D.,
Gleason Briana C.,
Thomas Antoinette B.,
Victor Thomas A.,
Cibull Thomas L.
Publication year - 2010
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.2010.01549.x
Subject(s) - pathology , cd34 , differential diagnosis , immunohistochemistry , medicine , biopsy , soft tissue , reticular connective tissue , biology , stem cell , genetics
Background: Perineuriomas are an uncommon group of tumors composed of perineurial cells of peripheral nerve sheath lineage. Variants include soft tissue (extraneural), intraneural, sclerosing, reticular and plexiform forms. Sclerosing perineuriomas have been reported to occur almost exclusively on the hands of young men. Herein, we report three extra‐acral cutaneous/soft tissue perineuriomas that show significant associated sclerosis. Methods: Skin biopsy specimens from three patients were evaluated for cytomorphology and degree of associated sclerosis, which was classified as either focal or diffuse. Immunohistochemical expression of epithelial membrane antigen (EMA), CD34 and S‐100 was also assessed to facilitate further classification. Results: Histopathologically, the tumors showed both focal and diffuse sclerosis, and lesional cells were generally spindled in shape. Immunohistochemical staining showed diffuse positivity for CD34 and focal or diffuse positivity for EMA. The cells uniformly lacked expression of S‐100 protein. Conclusions: Sclerosing perineuriomas can occur in extra‐acral locations and should be considered in the differential of EMA‐positive cutaneous spindle‐cell proliferations. It is also important to recognize that perineuriomas can express CD34 and should be considered in the differential diagnosis of CD34‐positive cutaneous neoplasms. Fox M, Gleason BC, Thomas AB, Victor TA, Cibull TC. Extra‐acral cutaneous/soft tissue sclerosing perineurioma: an under‐recognized entity in the differential of CD34‐positive cutaneous neoplasms.