Massive Osteolysis Due to Hemangiomatosis

Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone) is a rare disease, only a few cases having been described in detail. Nothing was definitely known as to its possible etiology until Gorham, Wright, Shultz and Maxon (1) reported the presence of an overgrowth of small thinwalled blood vessels (angiomatosis) in 2 cases and suggested that the resulting active hyperemia might disturb the balance between osteoblasts and osteoclasts, so that bone destruction exceeds bone production. Added weight was given to this theory in a later publication by Gorham and Stout (2), who described this same histologic picture in 8 cases previously published, the original biopsy slides having been submitted to them by various authors. From this study it was concluded that a hitherto unrecognized syndrome exists, characterized by a striking similarity in its clinical, roentgenographic, and histologic findings. However it is actually accomplished, the progressive osteolysis appears to be always associated with an angiomatosis of blood vessels, and sometimes of lymph channels, which seemingly is responsible for it. The term hemangiomatosis was first applied by Stout (3) in 1944 to describe the proliferation of capillaries which he had observed in the soft tissues distal to congenital arteriovenous fistulae. Later, he suggested this term to King (4) as a suitable one for a case of massive osteolysis described by that observer in 1946. The following case closely resembles the cases collected by Gorham and Stout, and is believed to represent only the second instance where autopsy was performed upon a patient with this unusual condition. J. B., a 16-year-old boy, son of a farmer, was admitted to the Surgical Service of the State Hospital at Pelhrimov, on July 25, 1955. The initial complaint was pain in the right shoulder and inability to move the right arm, which developed suddenly while the patient was working in the field. The personal history contained no points of a pertinent nature. There had been no previous injury. X-ray examination, two days after admission (Fig. 1), showed absence of a major portion of the right scapula. The only remnants were the axillary margin of the bone, a part of the coracoid process, and a few other small isolated areas. The border of the remaining portion of the scapula was poorly defined and osteoporotic changes were present in the residual bone tissue. The right clavicle was affected in a similar manner. The outer third of this bone had almost entirely disappeared. Roentgenographic examination of the remainder of the skeleton was negative. Laboratory tests showed normal values with the exception of the alkaline phosphatase, which was slightly increased 12.5 mg. per cent. Because this patient presented features highly suspicious of malignant disease, a biopsy was taken from the residual part of the right scapula. Histologically this specimen resembled an ordinary hemangioma of bone, showing proliferation of capillaries filled with red blood cells.