Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
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.
Kim
Jong Gwang
JG
Department of Oncology/Hematology, Kyungpook National University Hospital, Daegu, Korea.
Sung
Woo Jin
WJ
Kim
Dong Hwan
DH
Kim
Young Hwan
YH
Sohn
Sang Kyun
SK
Lee
Kyu Bo
KB
D000008:Abdominal Neoplasms / Q000175:diagnosis*; D000328:Adult; D005260:Female; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000150:complications*; D013899:Thoracic Neoplasms / Q000175:diagnosis*