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Expression of MiTF May be Helpful in Differentiating Cellular Neurothekeoma From Plexiform Fibrohistiocytic Tumor (Histiocytoid Predominant) in a Partial Biopsy Specimen

Fox, Melanie D. DO*; Billings, Steven D. MD; Gleason, Briana C. MD; Moore, Jocelyn MD§; Thomas, Antoinette B. MD*; Shea, Christopher R. MD; Victor, Thomas A. MD*; Cibull, Thomas L. MD*

The American Journal of Dermatopathology: April 2012 - Volume 34 - Issue 2 - p 157–160
doi: 10.1097/DAD.0b013e3182286a03
Original Study

Background Overlapping histopathologic features of cellular neurothekeoma (CNT) and plexiform fibrohistiocytic tumor (PFHT), when both are predominantly composed of histiocytoid cells, make distinction between these entities challenging. Some have suggested that CNT and PFHT are related entities. No prior study has demonstrated a reliable immunohistochemical panel to differentiate these entities.

Methods Skin biopsies diagnosed as CNT and PFHT, from 2004 to 2010 were retrieved with accompanying pathology reports. Each case was reviewed by at least 2 dermatopathologists and 2 soft tissue pathologists for confirmation of diagnosis. All cases were then evaluated for immunohistochemical expression of PAX2, NKIC3, CD10, and microphthalmia transcription factor (MiTF).

Results Histopathologically, the histiocytoid areas of each tumor shared similar architecture, demonstrating nests and fascicles of histiocytoid to spindled cells, with some separation of nests by collagen bands. Both CNT and PFHT were uniformly positive for NKIC3 and CD10, and both were frequently PAX2 positive. MiTF was strongly and diffusely positive in CNT and was consistently negative in the PFHT.

Conclusions CNT and PFHT share many histopathologic features and immunohistochemical staining patterns. Of the stains we evaluated, we found that expression of MiTF may be a reliable marker for distinguishing CNT from histiocytoid-predominant PFHT, especially in instances where only a small part of the tumor is sampled for evaluation.

*Department of Pathology, NorthShore University HealthSystem, Evanston, IL

Department of Anatomic Pathology, Cleveland Clinic, Cleveland OH

Diagnostic Pathology Medical Group, Sacramento, CA

§Department of Pathology, University of Chicago, Chicago, IL

Section of Dermatopathology, University of Chicago, Chicago, IL.

Reprints: Thomas L. Cibull, MD, Department of Pathology, NorthShore University HealthSystem, Evanston, IL 60201 (e-mail:

The authors have no funding or conflicts of interest to declare.

© 2012 Lippincott Williams & Wilkins, Inc.