Cervicothoracic malignant peripheral nerve sheath tumor in a 12-year-old girl with neurofibromatosis type 1.
We encountered a 12-year-old girl with neurofibromatosis type 1 (NF1) who developed a low-grade cervicothoracic malignant peripheral nerve sheath tumor (MPNST). Computed tomography of the neck showed an elastic firm tumor measuring 7 x 6 x 4 cm and arising from the upper mediastinum. She had no pain or neurological symptoms, but the tumor enlarged to ca. 8 x 8 x 4 cm over the following 7 months. Although we had diagnosed a neurofibroma by preoperative incisional biopsy, the resected tumor showed low-grade MPNST in the central portion of the tumor. In treating a tumor in a patient with NF1, we must recognize that partial biopsies do not necessarily establish a definitive diagnosis and that a growing tumor may indicate malignant transformation.
Imazu
M
M
Department of Surgery, Yamashiro Public Hospital, Kyoto, Japan.
Nakamura
Y
Y
Nakatani
H
H
Kaneda
H
H
Okamura
K
K
Sato
O
O
Hayase
Y
Y
D002648:Child; D005260:Female; D006258:Head and Neck Neoplasms / Q000473:pathology*; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D013899:Thoracic Neoplasms / Q000473:pathology*