title: Type 1 neurofibromatosis with a giant intrathoracic lesion: a case report with 25 years of follow-up.
abstract: We report a case of neurofibromatosis 1 with a huge intrathoracic lesion followed-up for 25 years. When the patient was 5 years of age, we performed a partial resection as an excisional biopsy. Microscopically, small spindle cells and collagen were spread between fat cells. A CT scan revealed a large intrathoracic mass, but tumor growth increasingly retarded as the patient grew older. During the entire 25-year follow-up period, from 5 to 30 years of age, the patient showed no neurological signs or other clinical symptoms. The findings of our case suggest that for a neurofibromatosis 1 patient, a good prognosis can be expected even if a huge destructive intrathoracic intrusion is detected. To assess the risk of malignant transformation, follow-up is required, accompanied by appropriate diagnostic modalities and physical examinations.
authors.affiliation: Department of Orthopedic Surgery, Nara Medical University, Nara, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Tsukamoto
authors.forename: Aikra
authors.initials: A
authors.lastname: Kido
authors.forename: Kanya
authors.initials: K
authors.lastname: Honoki
authors.forename: Manabu
authors.initials: M
authors.lastname: Akahane
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Mii
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Tanaka
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D006801:Humans; D009456:Neurofibromatosis 1 / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000601:surgery; D013909:Thorax / Q000473:pathology*