A 60-year-old woman in whom progressive systemic sclerosis (PSS) had been diagnosed 2 years previously was admitted because of an abnormal mass shadow on a follow-up chest X-ray. On the plain chest X-ray taken on the admission, right hilar adenopathy and a mass in the right lower lobe were recognized. Ground glass shadow and linear shadows were shown in the bilateral lower lobes. However, on a contrast enhanced CT image, soft tissue density masses, which were concomitant with subpleural curvilinear lines, were observed in the right S9, and right hilar adenopathy was also determined. From the results of these examinations including a biopsy, the lesions were diagnosed as resectable primary lung cancer. Subsequently, a right middle and lower lobectomy was performed. The tumor was determined to be a large cell neuroendocrine carcinoma by microscopic and immunohistochemical studies. The subpleural curvilinear line was also diagnosed as pulmonary fibrosis with PSS by a microscopic study. As a result, we diagnosed this case as a LCNEC with pulmonary fibrosis combined with PSS. We reported this case because large cell neuroendocrine carcinoma IS rarely combined with PSS.
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