title: Malignant peripheral nerve sheath tumor with glandular differentiation in a patient with neurofibromatosis type 1.
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.
authors.affiliation: *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.
authors.forename: Alison A
authors.initials: AA
authors.lastname: Galatian
authors.forename: A Neil
authors.initials: AN
authors.lastname: Crowson
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Fischer
authors.forename: Edward H
authors.initials: EH
authors.lastname: Yob
authors.forename: Igor
authors.initials: I
authors.lastname: Shendrik
mesh_terms: D002454:Cell Differentiation; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D009442:Neurilemmoma / Q000150:complications / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D012983:Soft Tissue Neoplasms / Q000150:complications / Q000473:pathology*