Pulmonary Adenomatosis

Abstract

Pulmonary adenomatosis in man is a rare disease of the lungs characterized by the development of multiple nodular adenomatous tumors or by a diffuse hyperplasia of the pulmonary alveolar lining cells. Typically the disease spreads throughout both lungs, interfering greatly with the gaseous exchange in the alveoli and eventually causing death, often with a terminal pneumonia and without the occurrence of metastases elsewhere in the body. The lesion has stimulated considerable speculation and interest, chiefly among pathologists, because of its close resemblance histologically to an infectious disease of the lungs which is widely encountered in sheep and to a lesser degree in some other animals. The present report is made for the purpose of reviewing the pathologic and clinical aspects of the disease, and more particularly the roentgen features, and to report our experiences in four cases, three of which have not previously been recorded in the literature. A fifth case will be discussed briefly because of interesting findings on histologic study. Writers on the subject of human pulmonary adenomatosis have been impressed by its resemblance to the condition found in sheep and known variously as jaagsiekte, epizootic adenomatosis, Montana progressive pneumonia of sheep, etc. (3, 9, 12). This disease has been endemic in many areas throughout the world and has been the subject of considerable study. There is sufficient evidence to indicate that it is of infectious origin, though the causative organism has not as yet been found. That a virus is responsible is the opinion of those who have made extensive studies. In 1939, Bonne (3) described a peculiar tumor-like process of the lungs found in a 30-year-old Chinese, characterized by a diffuse involvement of the alveolar walls of most of both lungs and a replacement of the alveolar cells by darkly staining cells in which mitoses were frequently seen. These cells lined the alveolar sacs and often protruded into their lumina forming papillary buds. There was practically no invasive growth or necrosis, and no metastases were present. Bonne noted the close resemblance to jaagsiekte in sheep and commented on the possible relationship. He did not believe the term carcinoma should be applied to the tumor in his case and suggested that it be called carcinosis. Previous to this report, Dungal (4), who had been studying the condition in sheep, had reviewed the literature in a search for cases of adenomatosis in man and found instances reported by Helly (1907), Löhlein (1908), and Oberndorfer (1930). Of these, the one reported by Helly more closely resembled the condition described. In Oberndorfer's case, metastases were present; Löhlein's patient had a single apple-sized tumor in the lower lobe of the right lung. In 1943, Sims (9) reported a case which he classified as pulmonary adenomatosis (Case 1 in our series) and also stressed the resemblance to jaagsiekte.