Angiomatosis: a vascular malformation of infancy and childhood. Report of 17 cases.
Angiomatosis is a complex vascular malformation of infancy and childhood consisting of proliferating blood vessels with accompanying mature fat and fibrous tissue, lymphatics and sometimes nerves, that may involve skin, subcutaneous tissue, skeletal muscle and occasionally bone; lesions are non-encapsulated with poorly defined infiltrative borders. Treatment is surgical, with local recurrence being common. We report 17 cases of angiomatosis presenting in children. Recurrences occurred in 10 patients, with multiple recurrences occurring in four. One child was treated with foot amputation followed two years later by mid-thigh amputation in an attempt to control local disease. Histology in all cases showed a mixture of small and medium-sized blood vessels, fat, connective tissue and lymphatics; nerves were increased in several cases. All lesions showed nests of proliferating capillaries, arranged in a lobular pattern, pushing into adjacent muscle and fat. This appearance was not seen in a large comparison group of vascular soft tissue lesions, and may serve as an indicator of angiomatosis with its associated risk of recurrence.
Howat
A J
AJ
Department of Anatomical Pathology, Royal Children's Hospital, Melbourne.
Campbell
P E
PE
D000798:Angiomatosis / Q000151:congenital / Q000473:pathology*; D001165:Arteriovenous Malformations / Q000473:pathology; D001808:Blood Vessels / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local; D012189:Retrospective Studies; D009626:Terminology as Topic