Musculoskeletal Angiomatous Lesions

Radiologic-Pathologic Correlation

Abstract Introduction Hemangioma Lymphangioma

Glomus Tumor Angiomatosis Angiomatous Syndromes and Associated Conditions Hamangioendothelioma, Hemangiopericytoma, and Angiosarcoma

Biopsy, Treatment, and Prognosis Summary Source Information References

Angiomatous Syndromes and Associated Conditions

Maffucci Syndrome

First described in 1881 (8 years before Ollier disease), Maffucci syndrome is characterized by multiple enchondromas and soft-tissue cavernous hemangiomas (59). The syndrome is a nonhereditary, mesodermal dysplasia with a mild male predilection. Hands and feet often show the greatest extent of involvement. Malignant transformation occurs for both enchondromas (to chondrosarcoma in 15% 20% of cases) and soft-tissue hemangiomas (to vascular sarcoma in 3% 5% of cases) (1,5). Patients with Maffucci syndrome have an increased prevalence of other malignancies, including ovarian and pancreatic carcinoma and central nervous system glioma (60,61,62).

At radiologic examination, matrix calcification is frequently seen in enchondromas, and phleboliths are commonly found in soft-tissue hemangiomas (Figure 15).

Osler-Weber-Rendu Syndrome

Osler-Weber-Rendu syndrome, also called hereditary hemorrhagic telangiectasia, is inherited in an autosomal dominant pattern and is caused by a systemic fibrovascular dysplasia of all vessels (63,64,65). This dysplasia results in telangiectasias, arteriovenous malformations (arteriovenous hemangioma), and aneurysms. Bleeding due to the vascular weakness of these lesions in the mucous membranes, skin, lungs, and genitourinary and gastrointestinal systems frequently occurs. Arteriovenous shunting can lead to congestive heart failure. Osseous involvement is quite rare but ranges from classic vertebral hemangioma to calvarial angiomatosis and synovial hemangioma.

Klippel-Tr­naunay-Weber Disease

Klippel-Tr­naunay-Weber disease consists of the triad of cutaneous hemangioma, bone and soft-tissue hypertrophy, and varicose veins (66). The disease is nonhereditary, and male and female subjects are equally affected. The syndrome is usually unilateral and involves the lower extremities. Arteriovenous fistula may also be present, and this manifestation is often called Parke-Weber syndrome.

The cutaneous hemangiomas are usually large and infiltrative and primarily of the capillary subtype (Figure 16). Gigantism involves both soft-tissue and osseous structures and can affect the entire extremity or only the distal digits. Venous varicosities are due to abnormalities of the deep venous system.

Kasabach-Merritt Syndrome

Kasabach-Merritt syndrome represents an association of hemangioma, hemangioendothelioma, or angiosarcoma with thrombocytopenia and purpura (67,68,69,70). Intravascular coagulation in the soft-tissue or osseous hemangioma leads to a bleeding diathesis. Repetitive hemarthrosis can also result, leading to a hemophilia-like arthropathy (68).

Gorham Disease

Gorham disease (massive osteolysis of Gorham), also referred to as vanishing or disappearing bone disease, was first reported in 1838, and more than 100 cases have now been described (71,72). Patients are usually younger than 40 years of age, and 50% have an associated history of trauma. The upper arm, shoulder, and mandible are most frequently involved. Pathologically, the condition is characterized by proliferation of thin-walled, endothelial-lined, anastomosing vascular channels, resembling hemangioma or lymphangioma.

At radiography in early stages, focal medullary and subcortical bone resorption that resembles osteoporosis is seen; this is followed by progressive bone destruction and fragmentation (Figure 17). Eventually, the entire bone may be involved, and the process can extend across joints (Figure 17). The osseous resorption may stabilize, but there is usually little regeneration. Recently, CT and MR imaging of Gorham disease has been described, and these modalities show changes similar to those of other angiomatous lesions (73,74).

Associated Conditions

Tumor-induced (oncogenic) osteomalacia can be caused by numerous osseous and soft-tissue neoplasms; however, it is most frequently associated with musculoskeletal angiomatous lesions, particularly hemangiopericytoma (75). Oncogenic osteomalacia is likely related to a tumor-produced humoral substance that decreases renal tubular resorption of phosphate, which results in osteomalacia. The osteomalacia is often debilitating, and detection of the underlying cause is important because removal of the lesion is curative.

Abstract Introduction Hemangioma Lymphangioma

Glomus Tumor Angiomatosis Angiomatous Syndromes and Associated Conditions Hamangioendothelioma, Hemangiopericytoma, and Angiosarcoma

Biopsy, Treatment, and Prognosis Summary Source Information References