Temporal progression of skeletal cystic angiomatosis Authors
First Online: 03 October 2007 Received: 16 April 2007 Revised: 31 July 2007 Accepted: 14 August 2007 DOI:
Cite this article as: Shivaram, G.M., Pai, R.K., Ireland, K.B. et al. Skeletal Radiol (2007) 36: 1199. doi:10.1007/s00256-007-0378-3 Abstract
Cystic angiomatosis is a rare, benign, multifocal disorder of bone and viscera, in which angiomatous deposits of both vascular and lymphatic elements result in bone lysis and organ dysfunction. We report on a case of late-onset cystic angiomatosis in a Caucasian woman who first presented at age 35 years with a lytic expansile lesion of the proximal humerus, initially diagnosed as low-grade hemangio-endothelioma. This was treated with injection of cement and prophylactic pinning. However, the lesion continued to grow, and, 5 years later, she was discovered to have disseminated bony involvement, initially thought to represent metastatic disease. However, further investigation revealed a diagnosis of cystic angiomatosis, and the patient was treated with bisphosphonates. Follow-up over a 15-year period since her initial presentation at age 35 years has shown osteosclerotic conversion of many of the lesions, with development of numerous pathologic stress fractures that have failed to heal, despite operative intervention.
Keywords Cystic angiomatosis Skeletal Osteosclerosis Bisphosphonate therapy Stress fracture References
Boyle WJ. Cystic angiomatosis of bone. A report of three cases and review of the literature. J Bone Joint Surg Br 1972; 54: 626–636.
Joshi SM, Pai PM, Jankharia GR. Cystic angiomatosis of bone. Indian Pediatr 1985; 22: 241–244.
Reid AB, et al. Familial diffuse cystic angiomatosis of bone. Clin Orthop 1989; 238: 211–218.
Schajowicz F, et al. Cystic angiomatosis (hamartous haemolymphagiomatosis) of bone. A clinicopathological study of three cases. J Bone Joint Surg Br 1978; 60: 100–106.
Seckler SG, Rubin H, Rabinowitz JG. Systemic cystic angiomatosis. Am J Med 1964; 37: 976–986.
Ishida T, et al. Cystic angiomatosis of bone with sclerotic changes mimicking osteoblastic metastases. Skeletal Radiol 1994; 23: 247–252.
Lateur L, et al. Skeletal cystic angiomatosis. Skeletal Radiol 1996; 25: 92–95.
Wallis LA, Asch T, Maisel BW. Diffuse skeletal hemangiomatosis: report of two cases and review of literature. Am J Med 1964; 37: 545–563.
Graham DY, Gonzales J, Kothari SM. Diffuse skeletal angiomatosis. Skeletal Radiol 1978; 2: 131–135.
Gutierrez RM, Spjut HJ. Skeletal angiomatosis: report of three cases and review of the literature. Clin Orthop Relat Res 1972; 85: 82–97.
Soler R, et al. Diffuse skeletal cystic angiomatosis: MR and CT findings. Eur J Radiol 1996; 22: 149–151.
Brunzell JD, Shankle SW, Bethune JE. Congenital generalized lipodystrophy accompanied by cystic angiomatosis. Ann Intern Med 1968; 69: 501–516.
Levey DS, et al. Cystic angiomatosis: case report and review of the literature. Skeletal Radiol 1996; 25: 287–293.
Boyse TD, Jacobson JA. Case 45: cystic angiomatosis. Radiology 2002; 223: 164–167.
Graham JJ, Yang WC. Vertebral hemangioma with compression fracture and paraparesis treated with preoperative embolization and vertebral resection. Spine 1984; 9: 97–101.
Ritchie G, Zeier FG. Hemangiomatosis of the skeleton and the spleen. J Bone Joint Surg Am 1956; 38: 115–122.
Wallace MJ, Ross M. Bone lymphangiomatosis: treatment with percutaneous cementoplasty. Spine 2005; 30: E336–E339.
Tran D, Fallat ME, Buchino JJ. Lymphangiomatosis: a case report. South Med J 2005; 98: 669–671.
Nazarian GK, Gebarski SS, Niparko JK. Cranial lymphangiomatosis causing CSF otorrhea and recurrent meningitis: CT features. J Comput Assist Tomogr 1990; 14: 121–123.
Abdelwahab IF. Sclerosing haemangiomatosis: a case report and review of the literature. Br J Radiol 1991; 64: 894–897.
Reid IR. Bisphosphonates. Skeletal Radiol 2007; 36: 711–714.
Selvaggi G, Scagliotti GV. Management of bone metastases in cancer: a review. Crit Rev Oncol Hematol 2005; 56: 365–378.
Odvina CV, et al. Severely suppressed bone turnover: a potential complication of alendronate therapy. J Clin Endocrinol Metab 2005; 90: 1294–1301.
Mashiba T, et al. Effects of suppressed bone turnover by bisphosphonates on microdamage accumulation and biomechanical properties in clinically relevant skeletal sites in beagles. Bone 2001; 28: 524–531.
Ott SM. Fractures after long-term alendronate therapy. J Clin Endocrinol Metab 2001; 86: 1835–1836.
Weinstein RS. True strength. J Bone Miner Res 2000; 15: 621–625.
Evans HL, Raymond AK, Ayala AG. Vascular tumors of bone: a study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma. Hum Pathol 2003; 34: 680–689.
Kleer CG, Unni KK, McLeod RA. Epithelioid hemangioendothelioma of bone. Am J Surg Pathol 1996; 20: 1301–1311.