Cystic angiomatosis is a not so well understood condition which presents with multiple bony cystic lesions in combination with visceral and / or soft tissue cystic lesions. There has been a long standing confusion over the terminology.
A subgroup of patients presenting with this condition present solely with skeletal lesions. This subgroup on follow up revealed spontaneous regression of skeletal lesions. Hence to separate this group of patients a separate term " skeletal-extraskeletal angiomatosis 4" was coined for cases presenting with visceral manifestations
It was defined as a benign vascular proliferation involving the medullary cavity of bone and at least one other type of tissue.
Visceral involvement is commonly of the spleen. Liver, kidneys, lung, mediastinum and retroperitoneum may also be involved. Soft tissue angiomas may be located in the neck, axilla or in the retroperitoneum.
Bony cystic lesions may be present in the axial as well as proximal appendicular skeleton. These lesions may cause bone pain or pathological fracture. It is considered a vascular hamartomatous condition, more common in men with a large number of cases diagnosed around puberty.
Cysts in various locations may either contain blood or proteinaceous fluid and may be the cause of death in infancy.
The differential diagnosis is essentially that of multiple lytic lesions of bone, and inlcudes:
- 1. Ballina-garcía FJ, Queiro-silva MR, Molina-suaréz R et-al. Multiple painful bone cysts in a young man. Ann. Rheum. Dis. 1996;55 (6): 346-9. doi:10.1136/ard.55.6.346 - Free text at pubmed - Pubmed citation
- 2. Levey DS, Maccormack LM, Sartoris DJ et-al. Cystic angiomatosis: case report and review of the literature. Skeletal Radiol. 1996;25 (3): 287-93. - Pubmed citation
- 3. Vanhoenacker FM, Schepper AM, Raeve H et-al. Cystic angiomatosis with splenic involvement: unusual MRI findings. Eur Radiol. 2003;13 Suppl 6 : L35-9. doi:10.1007/s00330-002-1805-4 - Pubmed citation
- 4. Clayer M. Skeletal angiomatosis in association with gastro-intestinal angiodysplasia and paraproteinemia: a case report. J Orthop Surg (Hong Kong). 2002;10 (1): 85-8. J Orthop Surg (Hong Kong) (link) - Pubmed citation
- 5. Devaney K, Vinh TN, Sweet DE. Skeletal-extraskeletal angiomatosis. A clinicopathological study of fourteen patients and nosologic considerations. J Bone Joint Surg Am. 1994;76 (6): 878-91. J Bone Joint Surg Am (link) - Pubmed citation
- 6. Murphey MD, Fairbairn KJ, Parman LM et-al. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995;15 (4): 893-917. Radiographics (abstract) - Pubmed citation
- 7. Pulido-Zamudio, Orr, Lertzman, Cystic angiomatosis of the bone appearing as intrathoracic lung masses, Can Respir J Vol 8 No 3 May/June 2001, 187-190, PMID # 11420593
- 8. Seckler, Rubin, Rabinowitz, Systemic Cystic Angiomatosis, Amer J Medicine, Vol 37 Dec 1964, 976-986 PMID: 14246097