Malignant Peripheral Nerve Sheath Tumor Not Associated with Neurofibromatosis.
- Author:
Hwa Mi KIM
1
;
Dong Hwa PARK
;
Chi Yeon KIM
Author Information
1. Department of Dermatology, School of Medicine, Gyeongsang National University, Jinju, Korea.
- Publication Type:Case Report
- Keywords:
Malignant nerve sheath tumor;
Malignant schwannoma;
Prognosis
- MeSH:
Actins;
Epithelioid Cells;
Muscle, Smooth;
Nerve Sheath Neoplasms;
Neurilemmoma;
Neurofibromatoses;
Peripheral Nerves;
Phosphopyruvate Hydratase;
Prognosis;
S100 Proteins;
Sarcoma;
Thigh
- From:Korean Journal of Dermatology
2012;50(4):362-366
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Malignant peripheral nerve sheath tumor (MPNST) is a rare entity of all soft tissue sarcomas. It has four different types of glandular, melanocytic, rhabdomyoblastic, and epithelioid. Epithelioid MPNST is composed of predominantly epithelioid cells. In contrast, typical MPNST is consisted of spindle cells, which is arranged in a fascicular or whirling pattern. A 33-old man showed two skin-color to reddish brown protruding nodules on the left thigh. Microscopically, one nodule was made up of epithelioid cell, primarily. In addition, the other nodule was composed of epithelioid cells and spindle cells, which also showed Antoni A and B. Immunohistochemical study showed the cells being positive for S-100 protein (weak and focal) and neuron-specific enolase, and negative for HMB-45, smooth muscle actin. Herein, we report a case of two different forms of malignant nerve sheath tumor on the left thigh, which was not associated with neurofibromatosis-1.
