Abstract
Cystic angiomatosis is a rare, benign, multifocal disorder of bone and viscera. Angiomatous deposits result in bone lysis and organ dysfunction. Bony cystic lesions occur in the axial and proximal appendicular skeleton. Lesions may cause bone pain or pathological fracture. Diagnosis is difficult, of exclusion and demands a biopsy. The prognosis varies upon whether the lesions are solely skeletal or there is visceral involvement. A 71-year-old man reports increasing symptoms of painful swelling in the right thoracic wall for over a month. The swelling was bony hard in consistency. Except for his bony swelling, the patient’s physical examination was within normal limits, as were all his laboratory studies. X-ray imagery showed multicystic expansive lytic areas involving the right ribs. Computerized tomography, magnetic resonance imagery and gallium bone scan revealed lytic lesions of multiple right ribs, and cervical, dorsal, lumbar and sacrum iliac spine. A right rib biopsy has shown a cystic formation with endothelial walls. Five years later, the patient remained stable, with no clinical, laboratory or imagilogic progression of disease and without visceral involvement. This case is presented in his rarity and differential diagnosis challenge.
Highlights
Cystic angiomatosis (CA) of bone is a rare pathological entity characterized by multifocal hemangiomatous and/or lymphangiomatous lesions of the skeleton with possible visceral organ involvement, especially the spleen [1,2,3,4,5,6]
The aetiology and exact pathogenic mechanism of the disease are still unknown, but most authors believe that it is congenital malformation: a vascular hamartoma [1,2,3, 8,9]. It is characterized by substitution of bone tissue with hamartomatous malformation of primitive vessels disseminated and multifocal [1,10]
CA affects mainly young adults of the male gender with half the cases being diagnosed around puberty [3]
Summary
Cystic angiomatosis (CA) of bone is a rare pathological entity characterized by multifocal hemangiomatous and/or lymphangiomatous lesions of the skeleton with possible visceral organ involvement, especially the spleen [1,2,3,4,5,6]. It was first described in 1953 by Jullian E. A definitive diagnosis of CA is difficult to establish and is frequently one of exclusion [6] It should be considered in patients presenting with diffuse cystic lesions of the skeleton on x-ray examination and with minimal or no associated clinical or laboratory findings [6]. The authors present this case report because of its extreme rarity and scientific interest concerning differential diagnosis
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