J Korean Surg Soc.
2006 Nov;71(5):365-370.
Retroperitoneal Malignant Peripheral Nerve Sheath Tumors Complicated with Type I Neurofibromatosis
- Affiliations
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- 1Departments of Surgery, College of Medicine, Chungbuk National University, Cheongju, Korea. webjwpark@chungbuk.ac.kr
- 2Departments of Pathology, College of Medicine, Chungbuk National University, Cheongju, Korea.
- 3Departments of Diagnostic Radiology, College of Medicine, Chungbuk National University, Cheongju, Korea.
- 4Departments of Radiation Oncology, College of Medicine, Chungbuk National University, Cheongju, Korea.
- 5Departments of Internal Medicine, College of Medicine, Chungbuk National University, Cheongju, Korea.
Abstract
- Neurofibromatosis is an infrequent genetic disorder that was first clinically described by von Recklinghausen and it was classified into 7 types by Riccardi. Type I neurofibromatosis is the most common type, and it is characterized by neurofibromas of variable size and multiple cafe-au-lait spots with minimal CNS lesion, if any. Malignant tumors develop in 2 to 13% of patients with type I neurofibromatosis, but they are rarely found in the retroperitoneum. We surgically resected huge retroperitoneal malignant peripheral nerve sheath tumors in 2 patients with sporadic type I neurofibromatosis. One patient survived 10 months and the other survived 3 months after operation. Retroperitoneal malignant peripheral nerve sheath tumor shows a poor prognosis even after surgical resection because of frequent recurrence and the lack of effective adjuvant therapy. Early diagnosis and development of new adjuvant therapy are required to effectively treat this malady.
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