Disease | Haemangioma |
Phenotype / cell stem origin | Endothelial cell |
Etiology | It is suggested that these lesions are congenital or developmental disorders, although the etiology remains unknown. |
Epidemiology | Haemangiomas are relatively common. Autopsy studies report in 10 to 12% of the population spinal haemangiomas. |
Clinics | Haemangiomas are in general asymptomatic, accidental radiographic findings, which are mostly located in skull (flat bones) and spine although extraspinal locations can occur. They are described at all ages, with a peak incidence at the fifth decade. There is a slight female predilection (M:F is 2:3). Large lesions can be symptomatic and symptoms as pain, cord compression and neurological deficit are reported. |
Pathology | Haemangiomas have variable histological features, which are similar to haemangiomas elsewhere in the body. Cavernous/capillary haemangiomas: with blood filled, thin-walled spaces lined by a single layer of flat, not atypical endothelial cells. Epithelioid haemangioma: well formed, mature vessels filled with erythrocytes. The vessels are lined by epithelioid cells, characterized by a large amount of eosinophilic cytoplasm. Intracytoplasmic vacuoles can be present. When the cells protrude in the lumen, a tombstone aspect can be seen. A variable inflammatory infiltrate containing lymphocytes and eosinophils is present. There is no hyalinization or myxoid changes of the surrounding stroma. |
Treatment | When necessary, surgical intervention (curettage or resection) can be considered with or without reconstruction. |
Evolution | Although progression towards an angiosarcoma is described, it is absolute extremely rare. |
Prognosis | Haemangiomas have a good prognosis and low recurrence rate. |
Disease | (Haem)angiomatosis |
Note | (Haem)angiomatosis of bone can be associated with |
Phenotype / cell stem origin | Endothelial cell |
Etiology | is still unknown. |
Epidemiology | It is a rare disease and in literature only few cases, mostly case reports, are described. Rarely haemangiomatosis is associated with massive regional osteolysis. Multiple, bone-associated haemangiomas can occur within some syndromes, such as: Maffucci's syndrome, Kasabach-Merrit syndrome, Klippel-Trenaunay / Parkes-Weber syndrome and Osler-Weber-Rendu disease. |
Clinics | Clinical features are variable and depend on the number of lesions, location and size. Pain and pathological fractures are the most common reported clinical features. Gorham-Stout syndrome or massive osteolysis affects the young adults and is a typical radiological finding. Half of the cases of Gorham-Stout syndrome are associated with trauma. |
Pathology | Histological pattern is identical to that of haemangioma. |
Treatment | Depends on the extent and location of the haemangiomas. Bone-associated haemangiomatosis syndromes can require a more specific approach. |
Evolution | Similar as haemangiomas. |
Prognosis | Extended visceral lesions have a more aggressive course mostly due to massive hemorrhaging. |
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