Bone: Haemangiomas and related lesions

2008-02-01   Sofie LJ Verbeke  , Judith VMG Bovée  

1.Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands

Summary

Note

Haemangiomas are benign, vasoformative lesions of endothelial origin.
Multiple lesions, involving two or more distant sites in bone, are defined as (haem)angiomatosis. Rarely these lesions are associated with massive osteolysis (Gorham-Stout syndrome).
Atlas Image
Haemangioma of bone - HE (10x).

Classification

Classification

Multiple variants of haemangiomas are reported, depending on variable histological features.
  • Haemangioma: cavernous, capillary, epithelioid.
  • Angiomatosis: non-aggressive (regional), disseminated (cystic angiomatosis), aggressive (massive osteolysis).
  • Clinics and Pathology

    Phenotype stem cell 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.

    Note

    (Haem)angiomatosis of bone can be associated with

    Phenotype stem cell 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: Maffuccis 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.

    Clinics

    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.

    Genetics

    Note

    No underlying genetic disorders or aberrations are described or known.

    Article Bibliography

    Pubmed IDLast YearTitleAuthors
    49406911971Vascular tumors of bone.Dorfman HD et al
    20769191990Massive pelvic osteolysis in the Gorham-Stout syndrome.Kulenkampff HA et al
    142158431964DIFFUSE SKELETAL HEMANGIOMATOSIS: REPORT OF TWO CASES AND REVIEW OF LITERATURE.WALLIS LA et al
    107414932000Benign vascular lesions of bone: radiologic and pathologic features.Wenger DE et al

    Citation

    Sofie LJ Verbeke ; Judith VMG Bovée

    Bone: Haemangiomas and related lesions

    Atlas Genet Cytogenet Oncol Haematol. 2008-02-01

    Online version: http://atlasgeneticsoncology.org/solid-tumor/5358/bone-haemangiomas-and-related-lesions