Osteogenic Sarcoma Occurring in Fragilitas Ossium

Abstract

The occurrence of osteogenic sarcoma and fragilitas ossium is not uncommon when alone, but a search through the literature failed to reveal a record of a case in which the two appeared together. Lobstein, in 1833, first used the term “osteopsathyrosis” to denote idiopathic as well as other types of fragility of the bones. Looser later called it “osteogenesis imperfecta congenita” and “osteogenesis imperfecta tarda,” the former being prenatal and affecting the bones of the skull and causing a large number of fractures of all the bones; and the latter being postnatal and affecting chiefly long bones. The pathologic picture was described as the same in both by Cinelli (1) and Ford (3). The microscopic picture of fragilitas ossium shows a deficient functional activity of the osteoblasts. The preliminary processes of ossification are normal. There has been no general agreement as to any primary abnormality in the marrow or in the function of the osteoblasts. Metaplasia is frequent but probably compensatory, not primary (Bronson, 2). It is generally felt now, although we know nothing of the etiology, that osteogenesis imperfecta and fragilitas ossium are two separate pictures, the first being non-hereditary and the latter hereditary. There is no evidence to denote any change in the blood levels of phosphatase, chloride, phosphorus, or calcium in fragilitas ossium. The diagnosis of a case of fragilitas ossium showing an associated osteogenic sarcoma prompted us to make the following report. Report of Case.—E. W., white male, 49 years of age, was admitted to the City of Detroit Receiving Hospital on Feb. 2, 1939, with the complaint of pain in the right hip. The patient stated that his present illness started in February, 1938, when he noted a dull, non-radiating pain localized in the region of the right hip. Symptoms were present on motion and from one to three hours after retiring. The condition had slowly progressed for the past year. Occasionally he was unable to work because of the condition of his hip. On Feb. 2, 1939, he fell on the ice, injuring his right hip. He was unable to rise and by virtue of his brittle bones he felt he had fractured his femur. Past Illness.—At the ages of 7, 18, and 35 years the patient had fractured his right femur from minor trauma to the area (Fig. 1). At the age of 21, he had fractured his left femur. It is interesting to note that the patient's father had had his femora fractured several times and is now a cripple. A brother had had at least 12 fractures of the thigh and leg bones. One of the patient's daughters sustained two fractures of the right femur (Fig. 2). His grandmother suffered the same condition, and, in short, the brittle bones could be traced verbally for from 200 to 300 years in his family.