Korean J Intern Med.  2005 Mar;20(1):100-104. 10.3904/kjim.2005.20.1.100.

Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I: Unusual Presentation of Intraabdominal or Intrathoracic Mass

• Kim JG
• Sung WJ
• Kim DH
• Kim YH
• Sohn SK
• Lee KB

• KMID: 1776707
• DOI: http://doi.org/10.3904/kjim.2005.20.1.100

Abstract

A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.

Keyword

Neurofibromatosis; Sarcoma; Chemotherapy

MeSH Terms

Abdominal Neoplasms/*diagnosis
Adult
Female
Humans
Nerve Sheath Tumors/*diagnosis
Neurofibromatosis 1/*complications
Thoracic Neoplasms/*diagnosis