Cystic angiomatosis of bone.

Abstract

The classical descriptions of Bucy and Capp (3) established the presently accepted roentgen criteria for the diagnosis of cavernous hemangioma of bone. In recent years, a rare and somewhat atypical form of cavernous hemangioma, aptly labeled “cystic angiomatosis” by Jacobs and Kimmelstiel (8), has been reported. As the name implies, there is widespread distribution of purely cystic lesions, which show none of the usual x-ray findings associated with hemangioma of vertebrae or flat bones. Nor are the lesions in the long bones entirely typical. A case of cystic angiomatosis, correctly diagnosed prior to definitive biopsy, will be presented in this article. So far as we know, it is the first to appear in the radiologic literature. A 15-year-old Negro boy was admitted to the Rochester Municipal Hospital on Oct. 28, 1955, with a fracture of the left femur sustained while playing football. Emergency roentgenography showed that the fracture had occurred in a cystic area and that several other cysts were present in the femoral shaft. There had been a fracture of the right humerus in 1950 and a review of the roentgenograms obtained at that time showed that this too had occurred in a cystic lesion, and that cysts were present in the humerus and scapula. The family history was non-contributory. Physical examination demonstrated, in addition to the fracture, a harsh Grade I systolic murmur. On palpation no masses were found to be in the abdomen. Blood studies showed: hemoglobin, 14.4 gm. per cent; hematocrit, 42; corrected sedimentation rate, 13 mm. in an hour; white cells, 5,800; calcium, 9.4 mg. per cent; phosphorus, 5.5 mg. per cent; acid phosphatase, 1.9 Bodansky units; alkaline phosphatase, 12.5 Bodansky units; total protein, 7.4 gm. per cent, with an A/G ratio of 4.5/2.9; cholesterol, 145 mg. per cent. Analyses of the urine and feces were negative. A roentgen survey of the skeleton demonstrated widespread involvement of many bones by benign-appearing cystic lesions (Figs. 1–5). When the films were first seen, the possibility of reticuloendotheliosis was suggested. Exposure of the skull for biopsy revealed a circular area darker than the surrounding normal bone. A small hole was bored through the outer table. At this point, brisk bleeding originating between the tables of the skull, was encountered. A circular area about 1 cm. in diameter was removed with a scalpel and an empty cavity was entered. Bleeding was controlled with suction and bone wax. On pathological study the material obtained at biopsy was reported as normal bone. Five months later, the patient was seen with a spontaneous hemarthrosis of the knee, which responded to general supportive measures and aspiration of blood from the joint. Roentgenograms showed no local bony defects. It was at this point that the diagnosis of cystic hemangiomatosis was suggested, based on the x-ray appearance of the lesions and their tendency to bleed.