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Neoplasms with Neural Differentiation: A Review: Part II: Malignant Neoplasms

Sangüeza, Omar P. M.D.1,2; Requena, Luis M.D.3

American Journal of Dermatopathology:

Malignant peripheral nerve sheath tumors (MPNSTs) encompass a wide and unusual group of neoplasms with features of neural differentiation. They most commonly present as spindle cell neoplasms and it can be difficult to differentiate them from other spindle cell neoplasms such as leiomyosarcomas, fibrosarcomas and synovial sarcomas. Strict criteria need to be applied in order to make the diagnosis of MPNSTs. Helpful features include contiguity with a nerve or an association with von Recklinghausen disease. The use of immunohistochemical stains may also help to confirm the diagnosis. Markers such as S-100 protein, neurofilament, epithelial membrane antigen and Leu-7 (CD57) are frequently used to assess neural differentiation in these neoplasms. In addition to the spindle cell pattern, MPNSTs may also display an epithelioid pattern. Rarely, other elements may be seen including glands in the so-called glandular MPNST or muscle in triton tumors. In more unusual cases cartilage, adipose tissue and even bone are present. Also included in the group of MPNSTs are the peripheral neuroepithelial tumor, neurotropic or desmoplastic melanomas and malignant granular cell tumors. MPNSTs are highly aggressive tumors and should be treated accordingly.

Author Information

From the Departments of Pathology and Medicine (Dermatology) (O.P.S.), Medical College of Georgia, Augusta, Georgia, USA and the Department of Dermatology (L.R.), Fundación Jimenez Díaz, Clínica Nuestra Señora de la Concepción, Universidad Autónoma de Madrid, Spain

Address correspondence and reprint requests to Dr. Omar P. Sangueza at Department of Pathology BA-E-210, Medical College of Georgia, 1120 15th Street, Augusta, Georgia 30912, U.S.A.

© Lippincott-Raven Publishers