Cystic angiomatosis

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  

Pathology

It was defined as a benign vascular proliferation involving the medullary cavity of bone and at least one other type of tissue.

Location

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.

Differential Diagnosis

The differential diagnosis is essentially that of multiple lytic lesions of bone, and inlcudes:

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Article Information

rID: 6349
Tag: cases
Synonyms or Alternate Spellings:
  • Skeletal angiomatosis

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