A case of large cell neuroendocrine carcinoma with pulmonary fibrosis combined with progressive systemic sclerosis

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.