Malignant Peripheral Nerve Sheath Tumor of the Retroperitoneum in Patient with Neurofibromatosis.
10.4111/kju.2006.47.11.1240
- Author:
Il Sang KANG
1
;
Seung Hwa CHOI
;
Hyung Bae MOON
;
Ill Young SE
Author Information
1. Departments of Urology and Pathology, Wonkwang University School of Medicine, Iksan, Korea.
- Publication Type:Case Report
- Keywords:
Malignant peripheral nerve sheath tumors;
Retroperitoneal space;
Neurofibromatosis
- MeSH:
Adult;
Cafe-au-Lait Spots;
Extremities;
Head;
Humans;
Liver;
Lung;
Male;
Neck;
Neurilemmoma;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1;
Peripheral Nerves*;
Retroperitoneal Space
- From:Korean Journal of Urology
2006;47(11):1240-1242
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A malignant peripheral nerve sheath tumor (MPNST), also known as a malignant neurilemmoma or malignant schwannoma, is significantly associated with neurofibromatosis type 1 (NF 1). NF 1 is characterized by multiple dermal neurofibromas, cafe-au-lait spots, MPNST and other abnormalities. MPNST is often metastatic, and is detected in the extremities, frequently in the head and neck, but rarely in the retroperitoneal and pelvic spaces. We experienced a case of MPNST in the retroperitoneal space of a 38-year-old male with NF 1, who also had metastatic lesions of the lung and liver.
