The Journal of Bone and Joint Surgery, Vol 76, Issue 6 878-891, Copyright © 1994 by Journal of Bone and Joint Surgery, Inc
Skeletal-extraskeletal angiomatosis. A clinicopathological study of fourteen patients and nosologic considerations
K Devaney, TN Vinh and DE Sweet
Department of Pathology, Brown University, Rhode Island Hospital, Providence 02903.
We reviewed the consultation files of the ARmed Forces Institute of
Pathology for 1951 through 1989 and identified fourteen patients who had
had skeletal-extraskeletal angiomatosis. Skeletal-extraskeletal
angiomatosis was defined as a benign vascular proliferation involving the
medullary cavity of bone and at least one other type of tissue. The age of
the patients at the time of initial biopsy ranged from nine months to
sixty-nine years (average, twenty-two years; median, ten years). Ten of the
patients were male and four were female. The presenting signs and symptoms
were highly variable; they included pain (four patients), a mass noted at
birth (three patients), a painless mass that developed after birth (two
patients), both pain and a mass (one patient), a localized deformity of the
thoracic spine (one patient), and anemia associated with chronic bleeding
of the gastrointestinal tract (one patient); in this last patient, skeletal
lesions subsequently were found and biopsied. Skeletal-extraskeletal
angiomatosis was an incidental finding in the remaining two patients.
Multiple bones were involved in thirteen of the fourteen patients.
Histologically, three patterns of lesion could be identified: cavernous
lymphangioma (six patients), cavernous hemangioma (six patients), and
arteriovenous hemangioma (two patients). Five of the patients died (three
of sepsis associated with persistent lesions of angiomatosis and two of
unrelated causes); eight of the patients survived but had residual disease,
and one survived and had no evidence of residual disease.