Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm.
ABSTRACT Perineuriomas are benign peripheral nerve sheath neoplasms composed of perineurial cells with characteristic immunohistochemical and ultrastructural features. They have been traditionally classified into two main types according to their location--intraneural and extraneural--and overlap histologically with many other tumors, which may be diagnostically challenging to general surgical pathologists.
To review the clinical, morphologic, immunohistochemical, ultrastructural, cytogenetic, and molecular genetic aspects of perineurioma, as well as to discuss its clinicopathologic variants and differential diagnosis.
English-language literature published between 1966 and 2005 was reviewed.
The correct identification of perineuriomas is important to avoid unnecessary overtreatment. The histologic diagnosis should be confirmed through immunohistochemical studies (including epithelial membrane antigen, S100 protein, and more recently described antibodies such as claudin-1 and GLUT1) or electron microscopy. Cytogenetic and molecular genetic studies are still of limited value for the diagnosis of perineuriomas but may play a fundamental role in excluding important differential diagnoses and also in helping elucidate the biology of these poorly known neoplasms.
- SourceAvailable from: PubMed CentralArchives of plastic surgery. 05/2014; 41(3):296-9.
- [Show abstract] [Hide abstract]
ABSTRACT: The authors describe a patient with recurrent perineurioma arising in the subcutaneous tissue of the dorsal forearm and extending along the forearm fascia. Soft tissue perineurioma is a rare, originally benign peripheral nerve sheath neoplasm arising from the perineurium, a protective cell barrier surrounding the individual fascicles in peripheral nerves. Perineurioma has only recently been recognized as an entity distinct from other nerve sheath tumors, such as schwannoma and neurofibroma, with unique morphologic, ultrastructural, and immunoreactive features. The recurrent tumor had converted into malignant perineurioma, defined as increased nuclear pleomorphism and cellularity. The ill-marginate feature extending along the fascia required wide resection, leaving a substantial defect on the distal forearm. Surgical repair of large forearm skin defects is challenging because of limited skin extensibility for flap creation, the prominence of the site in terms of aesthetic outcome, and the risk of damage to extrinsic muscles that control delicate hand movements. The reverse forearm adipofascial flap, which was based on distal perforators of the radial artery, was suitable for the current case to cover the exposed myotendinous junctions of the forearm extensor muscles. This flap did not sacrifice skin, a major vessel, or skeletal muscles, and preserved function at both the donor and the recipient sites. The texture of the graft was similar to that of the surrounding skin. The clinical and histopathologic features of this rare tumor are also described to aid in the differential diagnosis and as a reference for surgeons who treat soft tissue neoplasms and may encounter this type of soft tumor.Orthopedics 07/2014; 37(7):e661-e664. · 0.98 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Perineuriomas are rare peripheral nerve sheath tumors with one or few chromosomal rearrangements or numerical changes. Two main types and three subtypes have been defined but with few specific genetic associations. Chromosome 10 aberrations are found involved in 3 cases of the sclerosing perineuroma subtype. Chromosome 22 abnormalities have been described in different types of perineuroma. None of these aberrations has been described at the molecular level. We report on a complex rearrangement characterized by FISH and array-comparative genome hybridization revealing submicroscopic deletions at 2p23 and 9q34 involving the ABL1 gene in a soft tissue perineuroma case.Cancer Genetics 06/2014; · 2.42 Impact Factor