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Bone: Haemangiomas and related lesions

Written2008-02Sofie LJ Verbeke, Judith VMG Bovée
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands

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Identity

ICD-Topo C400-C403,C408-C414,C418-C419 BONES & JOINTS
ICD-Morpho 9120/0 Haemangioma
Atlas_Id 5358
Phylum Bones::Haemangioma and related lesions
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).
Haemangioma of bone - HE (10x).

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

    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.

    Genetics

    Note No underlying genetic disorders or aberrations are described or known.

    Bibliography

    Vascular tumors of bone.
    Dorfman HD, Steiner GC, Jaffe HL
    Human pathology. 1971 ; 2 (3) : 349-376.
    PMID 4940691
     
    World Health Organization Classification of Tumours: Pathology and genetics of tumours of soft tissue and bone.
    Fletcher CDM, Krishnan Unni K, Mertens F
    2002.
     
    Massive pelvic osteolysis in the Gorham-Stout syndrome.
    Kulenkampff HA, Richter GM, Hasse WE, Adler CP
    International orthopaedics. 1990 ; 14 (4) : 361-366.
    PMID 2076919
     
    Bone Tumors. Clinical, radiologic and pathologic correlations.
    Mirra JM
    Lea..
     
    Radiologic Atlas of Bone Tumours.
    Mulder JD, Schutte HE, Kroon HM, Taconis WK
    1993.
     
    DIFFUSE SKELETAL HEMANGIOMATOSIS: REPORT OF TWO CASES AND REVIEW OF LITERATURE.
    WALLIS LA, ASCH T, MAISEL BW
    The American journal of medicine. 1964 ; 37 : 545-563.
    PMID 14215843
     
    Benign vascular lesions of bone: radiologic and pathologic features.
    Wenger DE, Wold LE
    Skeletal radiology. 2000 ; 29 (2) : 63-74.
    PMID 10741493
     

    Citation

    This paper should be referenced as such :
    Verbeke, SLJ ; Bove, JVMG
    Bone: Haemangiomas, related lesions
    Atlas Genet Cytogenet Oncol Haematol. 2009;13(2):149-150.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Tumors/HaemangiomBoneID5358.html


    External links

    arrayMap Topo ( C40,C41) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
     
     
    Disease databaseBone: Haemangiomas and related lesions
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed


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