Type 1 neurofibromatosis with a giant intrathoracic lesion: A case report with 25 years of follow-up
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- 作者：Shinji Tsukamoto ; Aikra Kido ; Kanya Honoki ; Manabu Akahane ; Yoshio Mii ; Yasuhito Tanaka
- 关键词：Neurofibromatosis 1 ; Intrathoracic lesion ; Long-term follow-up
- 刊名：Pathology - Research and Practice
- 出版年：2010
- 出版时间：15 June 2010
- 年：2010
- 卷：206
- 期：6
- 页码：408-410
- 全文大小：458 K
文摘
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.