MASSIVE OSTEOLYSIS AND ANGIOMATOSIS.

Abstract

Massive osteolysis (phantom bone, spontaneous absorption of bone, or disappearing bone disease) is a bizarre entity characterized pathologically by angiomatosis. Its etiology is unknown, the clinical progression and prognosis are unpredictable, and treatment is uncertain. Absorption, initially localized to one bone, often extends to involve those adjacent to such a degree that clinically the ultimate impression can hardly be considered anything less than “malignant.” The associated angiomas, however, are histologically benign and no pathologically malignant tissue is present. Deformity and disability may be extensive; widespread involvement of ribs and vertebrae may cause death. Since the first case of spontaneous absorption of bone recorded by Jackson (7, 22) in 1838, at least 38 additional cases have been documented. Gorham and Stout (9, 10), in a review of 24 of these, emphasized the hemangiomatous nature of the bone in the zones of osteolysis. Three years later, Johnson and McClure (17) reviewed 8 additional cases. We have found reports of an additional 7 (1, 2, 4, 5, 8, 18, 26, 27), bringing the total to 39. Two other reported cases may be massive osteolysis and should be observed for further progression (20, 21). Four previously unreported cases are the subject of this report. Case Reports Case I: A 23-year-old white man entered the Mayo Clinic in November 1962 because of increasing pain in the left hip of twenty-eight months duration and the appearance of a limp. A roentgenogram obtained elsewhere in June 1961 had shown an osteolytic process involving the left innominate bone and the adjacent sacrum (Fig. 1, A); roentgenograms taken on admittance to the Clinic revealed that the destruction had become more severe and more extensive (Fig. 1, B). A bone survey disclosed no other areas of involvement. At operation, the outer table of the ilium near the crest was found to have been destroyed. A copious amount of serous fluid exuded from the tissues immediately outside the ilium, and little bleeding was noted. Sections of this tissue revealed cavernous lymphangioma (Fig. 2, A). The inner table of the ilium was smooth and the periosteum stripped with ease. Specimens of the partially destroyed bone showed an increased content of thinwalle vessels, some of which contained erythrocytes (Fig. 2, B). A postoperative lymphangiogram was normal, as was a subsequent aortogram, and no obvious connection with the vascular or lymphatic circulation was noted (Fig. 1, C). Postoperative roentgen therapy was given. Comment. A critical observation disclosed that clear fluid rather than blood exuded from the angiomatous tissues adjacent to bone.