Cancer of the lung in progressive systemic sclerosis.

An association between one of the collagen diseases, dermatomyositis, and malignant disease is well recognized; in a recent review Caldwell (1955) states that there are nearly 70 reported cases in which the association has been noted, and an incidence of 18 % malignancy in cases of dermatomyositis has been reported by Curtis, Blaylock, and Harrell (1952). Many authorities (Dowling, 1940; Freudenthal, 1940; Goetz, 1945; Pagel and Treip, 1955) think that dermatomyositis and the condition which is commonly called generalized or diffuse scleroderma, but which we along with others (Goetz, 1945; Cullinan, 1953, Beigelman, Goldner, and Bayles, 1953; Piper and Helwig, 1955), believe is better called progressive systemic sclerosis, are variants of the same disease. It is therefore of interest that a similar association between malignant disease and progressive systemic sclerosis is apparently rare (Talbott and Ferrandis, 1956). Apart from a report by Barlow (1939) of a patient who had diffuse scleroderma and who was found at necropsy to have an apparently simple adrenal adenoma, there were no definite records of neoplasia in cases of progressive systemic sclerosis until 1953 when Zatuchni, Campbell, and Zarafonetis reported three patients who had scleroderma, pulmonary fibrosis, and carcinoma of the lung. Jonsson and Houser (1956) have since reported another similar case. In the last two years we have seen two patients who had this combination of progressive systemic sclerosis and pulmonary carcinoma. The purpose of this paper is to present these two cases in detail, to review briefly the findings in the cases previously reported, and to discuss the association between the two diseases.