A roentgen study of osteogenic sarcoma developing in Paget's disease.

Abstract

In 1889, Paget (16) in a paper dealing with the relationship of osteitis deformans and osteogenic sarcoma concluded that he could not venture to guess what the nature of the connection was between these two diseases. His study was based on the observation of 5 cases of cancer or sarcoma developing in 23 patients having osteitis deformans. To date, records of more than a hundred osteogenic sarcomas occurring with Paget's disease have appeared in the literature and yet no further insight is provided regarding the fundamental relationship between these two conditions. Summey and Pressly (20) in 1946 summarized the available reports and added a number of cases of their own, making a total of 76. Sear (18) reported additional material in 1949 and commented in a general way upon the roentgen appearance of these two diseases when found together. Since there has been no study devoted solely to an analysis of the roentgen findings based upon a representative volume, it was thought worth while to examine the material available at Memorial Center. Nineteen biopsy-proved cases of osteogenic sarcoma occurring in areas of Paget's disease were selected. All had adequate x-ray coverage, often extending over a period of some time, so that interval change could be observed. The objective, therefore, was to set up criteria for prompt x-ray detection of osteogenic sarcoma associated with Paget's disease in order that this dangerous complication might be treated as early as possible. To this end, general information regarding the incidence of osteogenic sarcoma in Paget's disease will be pertinent, as will a brief discussion of roentgenologic types of both conditions. It also seems indicated to include a few statements summarizing this material as to age, sex, symptoms, signs, and course of the cancerous disease, since information of this nature may be of some value in formulating intelligent roentgen opinion. The incidence of sarcomatous degeneration in Paget's disease varies according to different observers. The following is a tabulation of some of the estimates: It is conceivable that the incidence is not actually as high as many statistics show, because of the difficulty of finding every patient with osteitis deformans. Roentgenologists well know that many cases are picked up unexpectedly in the course of studies directed to other objectives. Furthermore, many are never recognized because no x-ray examinations are made, a significant proportion of patients with the disease being quite asymptomatic. As Paget recognized nearly a century ago, it is quite evident that the relation between osteitis deformans and bone sarcoma is real. From this standpoint, it is interesting to note other diseases of bone associated with the development of sarcoma. The possibility of malignant change in the various forms of internal and external osteochondromata is well known. Several instances of bone sarcoma developing in fibrous dysplasia have been noted (5, 9).