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: firstname.lastname@example.org).
The authors declare no conflicts of interest.