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Desmoplastic Primitive Nonneural Granular Cell Tumor of the Skin

Agustí, Jaime MD; Martínez-Ciarpaglini, Carolina MD; Monteagudo, Carlos MD, PhD

The American Journal of Dermatopathology: April 2014 - Volume 36 - Issue 4 - p e84–e86
doi: 10.1097/DAD.0b013e318296fe84
Extraordinary Case Report

Abstract: Primitive nonneural granular cell tumor of the skin was first described by LeBoit et al in 1991 as “primitive polypoid granular cell tumor.” Few cases have been reported to date, all being polypoid or deep well-delimited lesions and formed by large spindle or polygonal granular cells with moderate nuclear atypia and increased mitotic activity. This granular cell population does not have a Schwannian, myogenic, melanocytic, fibroblastic, histiocytic, or epithelial differentiation. We report a case that fully satisfies the criteria for primitive nonneural granular cell tumor of the skin and, in addition, shows an extensive desmoplastic stroma. This desmoplastic variant of primitive nonneural granular cell tumor, which to our knowledge has not been previously reported, should be recognized to appropriately face the differential diagnosis with the malignant granular cell tumor, whose criteria for malignancy cannot be applied to primitive granular cell tumors.

Department of Pathology, Hospital Clínico Universitario-INCLIVA, University of Valencia, Valencia, Spain.

Reprints: Jaime Agustí, MD, Department of Pathology, Hospital Clínico Universitario de Valencia, Avenida Blasco Ibáñez No. 17, CP 46010, Valencia, Spain (e-mail:

The authors declare no conflicts of interest.

© 2014 by Lippincott Williams & Wilkins.