title: Cervicothoracic malignant peripheral nerve sheath tumor in a 12-year-old girl with neurofibromatosis type 1.
abstract: 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.
authors.affiliation: Department of Surgery, Yamashiro Public Hospital, Kyoto, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Imazu
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamura
authors.forename: H
authors.initials: H
authors.lastname: Nakatani
authors.forename: H
authors.initials: H
authors.lastname: Kaneda
authors.forename: K
authors.initials: K
authors.lastname: Okamura
authors.forename: O
authors.initials: O
authors.lastname: Sato
authors.forename: Y
authors.initials: Y
authors.lastname: Hayase
mesh_terms: 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*