Skip Navigation LinksHome > December 2013 - Volume 35 - Issue 8 > Malignant Peripheral Nerve Sheath Tumor With Glandular Diffe...
American Journal of Dermatopathology:
doi: 10.1097/DAD.0b013e318284a611
Extraordinary Case Report

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†,‡

Collapse Box

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.

© 2013 Lippincott Williams & Wilkins

Login

Article Tools

Share

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.