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Malignant Peripheral Nerve Sheath Tumor With Glandular Differentiation in a Patient With Neurofibromatosis Type 1

Galatian, Alison A. MD*; Crowson, A. Neil MD†,‡; Fischer, Robert J. MD, MPH§; Yob, Edward H. DO†,¶; Shendrik, Igor MD†,‡

The American Journal of Dermatopathology: December 2013 - Volume 35 - Issue 8 - p 859–863
doi: 10.1097/DAD.0b013e318284a611
Extraordinary Case Report

Abstract: The authors report an unusual case of malignant peripheral nerve sheath tumor with malignant differentiation arising as a subcutaneous nodule in the thigh of a 53-year-old woman with a history significant for neurofibromatosis type 1. Peripheral nerve sheath tumors containing a glandular component, commonly referred to as glandular peripheral nerve sheath tumors, are rare neoplasms found largely in patients with neurofibromatosis type 1. These tumors are frequently malignant; recognition of metastatic potential is made based on the atypical spindle-cell component. Rarely, as in our case, the glandular component is also histologically malignant. Only 5 such tumors have been described in the literature to date. Glandular differentiation, particularly with malignant features, can be a potentially misleading feature when found as a component of malignant peripheral nerve sheath tumors and raise a wide spectrum of differential diagnoses, including metastatic Sertoli-Leydig tumors. The patient is free of disease for 22 months after wide tumor reexcision, which contrasts with previously reported devastatingly poor prognosis of these tumors.

*Department of Dermatology, University of Oklahoma, College of Medicine, Oklahoma City, OK;

Oklahoma Department of Dermatology, University of Oklahoma, College of Medicine, Oklahoma City, OK;

Dermatopathology Section, Regional Medical Laboratory, Tulsa, OK;

§Department of Internal Medicine, University of Oklahoma, College of Medicine, Oklahoma City, OK; and

Tulsa Cancer Institute – Skin Cancer Center, Tulsa, OK.

Reprints: Alison A. Galatian, MD, 619 NE 13th Street, Oklahoma City, OK 73104 (e-mail:

The authors declare no conflicts of interest.

© 2013 by Lippincott Williams & Wilkins.