[1例与多发性肺大疱和肺癌相关的神经纤维瘤病]

[A case of Recklinghausen's disease associated with multiple emphysematous bullae and lung cancer].

作者信息

Nagai H, Kurashima A, Yoneda R, Komatsu H, Takahashi K

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1989 May;27(5):625-9.

Abstract

A 36-year-old man was admitted with cough and sputum. He had cafe-au-lait spots and multiple subcutaneous neurofibromas. Chest X-ray revealed multiple emphysematous bullae in bilateral upper lobes and a tumor in the bulla of the right upper lobe. Needle aspiration biopsy of the tumor showed small cell carcinoma. Although chemotherapy and radiation resulted in decrease in tumor size, it subsequently increased in size and he died 11 months after admission. Including this case there have been 7 reports of Recklinghausen's disease associated with multiple lung cysts and 8 reports with lung cancer in Japan. However, cases with lung cysts and cancer are very rare. The cancer of this case was considered to be associated with emphysematous bulla rather than Recklinghausen's diseases.

摘要

一名36岁男性因咳嗽、咳痰入院。他有牛奶咖啡斑和多个皮下神经纤维瘤。胸部X线显示双侧上叶多个肺大疱，右上叶肺大疱内有一个肿瘤。肿瘤针吸活检显示为小细胞癌。尽管化疗和放疗使肿瘤大小减小，但随后又增大，入院11个月后死亡。包括本病例在内，日本有7例关于神经纤维瘤病合并多发性肺囊肿的报告，8例关于神经纤维瘤病合并肺癌的报告。然而，肺囊肿合并癌症的病例非常罕见。本病例的癌症被认为与肺大疱有关，而非神经纤维瘤病。