Introduction
Cortical thickening of the tibia adjacent to an intramuscular hemangioma of the leg.
T1 and T2 MRI images of intramuscular hemangioma of the leg. Note the serpentine quality of the vessels and that the hemangioma is high signal on both T1 and T2. This indicates that the hemangioma is predominantly of water density.
High-power view of the histology of an intramuscular hemangioma. Red blood cells are visible within the vascular channels.
Background
A hemangioma is an abnormal proliferation of blood vessels that may occur in any vascularized tissue. Considerable debate exists as to whether these lesions are neoplasms, hamartomas, or vascular malformations. Mulliken strongly supports classification of hemangiomas as neoplasms, whereas Godanich and Capanacci seem to favor a hamartomatous classification.1,2 There seems to be consensus that the term "hemangioma" should refer to "hemangiomas of infancy," which have a predictable natural history that includes absence at birth followed by a period of growth over 6-18 months and then a period of involution that may take several years. "Hemangiomas" affecting the musculoskeletal system are more accurately termed "vascular malformations." These are present from birth and do not involute spontaneously.3,4
Hemangiomas occur most often in skin or subcutaneous tissue, and dermatologists, pediatricians, and primary care medical physicians typically treat these readily identifiable processes. One common example is the senile or cherry hemangioma, which is a benign, self-limited, small, red-purple skin papule seen in elderly patients. Another is the strawberry nevus, which is seen in approximately 0.5% of infants and spontaneously involutes in the vast majority of cases. Visceral hemangiomas are far less common but may have greater consequences when they result in organ dysfunction.
Orthopedists most commonly are called upon to treat hemangiomas of the deep soft tissues and bone. Skeletal muscle is the most common site for hemangioma of the deep soft tissue. Intramuscular hemangiomas may cause symptoms such as pain and swelling for which patients seek treatment. Hemangioma of bone may be symptomatic or may be purely an incidental finding. Most commonly, hemangiomas are localized to a single area, but multiple hemangiomas may occur in a single individual in a process known as hemangiomatosis.5,6,7,8
Some authors have defined hemangiomatosis as multiple hemangiomas occurring in noncontiguous bones. Devaney et al defined skeletal-extraskeletal angiomatosis as a benign vascular proliferation involving the medullary cavity of bone and at least one other type of tissue.9 Rarely, hemangiomas may be associated with other pathologic processes, such as the consumptive coagulopathy of Kasabach-Merritt syndrome and tumor-induced osteomalacia. Gorham disease is a process of massive osteolysis, which is believed to be within the spectrum of hemangiomatous disease. Hemangiomas occurring in the setting of multiple enchondromatosis are part of the spectrum of Maffucci syndrome.
Related Medscape topics:
Specialty site Orthopaedics
Orthopaedics CME and News
Resource Center Pediatric Dermatology
Related eMedicine topics:
Hemangioma, Bone
Kasabach-Merritt Syndrome
Consumption Coagulopathy
Enchondroma and Enchondromatosis
Maffucci Syndrome
History of the Procedure
The first documentation of a case of intramuscular hemangioma is attributed to Liston in 1843. Virchow described the first case of a vertebral hemangioma in 1867. In 1940, Kasabach and Merritt reported a case of a hemangioma involving the skin and deep soft tissue of the thigh that was associated with extensive purpura. The patient's platelet count dropped as low as 16,000/dL, but he responded well to transfusions and radiation therapy. Gorham et al first reported on 2 cases of massive osteolysis in 1954, and, in 1955, they expanded on the pathologic findings and potential etiology.
Problem
Hemangiomas of muscle and bone are abnormal proliferations of blood vessels that may be asymptomatic or may cause symptoms such as pain and swelling. Multiple hemangiomas may be present in a single individual with hemangiomatosis or skeletal-extraskeletal angiomatosis. Gorham disease is an osteolytic process thought to be related to abnormal vasculature, resulting in hyperemia of affected bones. Rarely, hemangiomas may be associated with complications such as tumor-induced osteomalacia or Kasabach-Merritt syndrome.
Frequency
Intramuscular hemangiomas
Most deep soft tissue hemangiomas probably are asymptomatic and small and go completely unnoticed; therefore, the exact incidence and prevalence are impossible to determine with any degree of certainty. That said, intramuscular hemangiomas are uncommon compared with other types of hemangiomas. Muscle hemangiomas accounted for 10 of 570 hemangiomas reported by Geschickter and Keasbey.10 Watson and McCarthy estimated that intramuscular hemangiomas accounted for 0.8% of all benign vascular tumors.11 Intramuscular hemangiomas occur most often in young people (range 2 mo to 66 y), with 80-90% presenting in persons younger than 30 years. Males and females are affected with nearly equal frequency.
Synovial hemangiomas
Synovial hemangiomas are extremely rare. They can arise from any surface that is lined by synovium, particularly tendon or joint space. They typically occur in young patients.
Osseous hemangiomas
Hemangiomas of bone accounted for approximately 1% of primary bone tumors of which biopsies were taken in Dahlin and Mirra's series.12 Hemangiomas of bone may occur in patients of any age. Approximately 25% present in persons in the fifth decade of life; however, hemangiomas have been reported in patients as young as 2 years and as old as 77 years. Approximately two thirds of osseous hemangiomas occur in the cranium or vertebrae, and hemangioma is the most common benign tumor of vertebrae. Vertebral hemangiomas are found in approximately 10% of autopsy specimens.
Hemangiomatosis
Hemangiomatosis and skeletal-extraskeletal angiomatosis are rare conditions.
Gorham disease
Radiograph of a patient with Gorham disease showing dissolution of bone.
Gorham disease (ie, massive osteolysis, disappearing bone disease) is very rare. Most patients are younger than 40 years.
Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is a rare complication of large hemangiomas in which platelets are trapped and a consumptive coagulopathy ensues.
Etiology
Hemangiomas
The etiology of hemangiomas is unclear. Angiogenesis likely plays a role in the vascular excess present. Cytokines, such as basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) are known to stimulate angiogenesis. Excesses of these angiogenic factors or decreases of angiogenesis inhibitors (eg, gamma-interferon, tumor necrosis factor–beta, transforming growth factor–beta) have been implicated in the development of hemangiomas.13
Gorham disease
The etiology of Gorham disease is unknown but is thought to be related to increased vascularity of the affected bones. The resultant hyperemia has been hypothesized to uncouple the balance between osteoblasts and osteoclasts, leading to bone resorption at a far greater rate than bone formation.14
Kasabach-Merritt syndrome
Although the etiology is not entirely clear, development of Kasabach-Merritt syndrome seems to be related to stagnation of blood flow within a large hemangioma, which leads to platelet trapping and a subsequent consumptive coagulopathy.
Tumor-induced osteomalacia
The mechanisms behind osteomalacia have not been fully elucidated.
Related Medscape topic:
Article Tumor-Induced Osteomalacia
Pathophysiology
Hemangiomas are benign lesions with increased numbers of blood vessels. They can affect numerous tissue types (individually or in combination), including skin, subcutaneous tissue, viscera, muscle, synovium, and bone, but they do not spread to avascular tissue such as cartilage.
Gorham disease is a process in which variably progressive dissolution of bone occurs. This process may affect a single bone or may cross joint spaces. The etiology of this process is unknown but is thought to be related to excess vascularity of the involved bone.
Presentation
Intramuscular hemangiomas
Intramuscular hemangiomas occur most often in young adults, with 80-90% presenting in individuals younger than 30 years. They occur most often in the lower extremities, especially the thigh, and typically present with a palpable mass, but the overlying skin typically is not discolored. Intramuscular hemangiomas can be asymptomatic or can present with symptoms including increased girth of the extremity, increased temperature in the area, discoloration of the overlying skin, and pain. Intramuscular hemangiomas typically are compressible and decrease in size with elevation of the extremity. Exercise often exacerbates the symptoms of pain and swelling due to vascular dilation from increased blood flow through the hemangioma. Larger hemangiomas may be associated with a bruit or thrill. Often, intramuscular hemangiomas cannot be distinguished definitively from soft-tissue sarcomas based on clinical examination alone.
Large intramuscular hemangiomas occasionally may be associated with significant shunting of blood flow. This is uncommon, but in rare cases, it may lead to heart murmurs and/or congestive heart failure. If significant shunting exists within the hemangioma, the presentation may be similar to that of an arteriovenous fistula. In this case, it may be possible to elicit the Branham sign, a reflex bradycardia following compression of the arteriovenous fistula, due to reduction in the shunt.
Synovial hemangiomas
Synovial hemangiomas are rare. In tendinous synovium, they typically present as a painless mass. In the synovium of a joint, they may present with recurrent effusions, pain, and even mechanical symptoms suggesting intra-articular derangement. A palpable, spongy, compressible mass may be present, and it may decrease in size with elevation of the extremity. The knee is by far the most common joint involved, where the presentation may be confused with meniscal or ligamentous pathology.15 Both localized and diffuse forms exist.
Osseous hemangiomas
Hemangiomas of bone often are incidental findings, but they may cause pain and swelling. Those in the skull may be associated with swelling, erythema, tenderness, or facial deformity. On rare occasions, vertebral hemangiomas may cause cord compression and fractures, but most vertebral hemangiomas are asymptomatic.
Osseous hemangiomas may be solitary (affecting a single bone) or focal (affecting one bone or, according to some authors, contiguous bones in a focal site). Various authors define hemangiomatosis differently. Some authors define hemangiomatosis as multiple hemangiomas located in noncontiguous bones. The condition of multiple bony hemangiomas also has been referred to as cystic angiomatosis of bone when no soft-tissue component is present. Skeletal-extraskeletal angiomatosis has been defined as hemangiomas affecting the medullary canal of a bone, as well as 1 nonosseous site. The nonosseous site most often is adjacent soft tissue, but, alternatively, the nonosseous site may be noncontiguous viscera.
Other authors define hemangiomatosis as lesions involving skin, muscle, and bone, which usually become symptomatic during childhood, with diffuse, persistent swelling and discoloration, with or without pain. Hemangiomatosis can also present with pathologic fracture.
Rarely, hemangiomas may be associated with induction of osteomalacia. They are one of many tumors that may cause osteomalacia.
Gorham disease
Gorham disease can present with dull, aching pain or insidious weakness, and rarely is suspected prior to radiographic evaluation. Patients usually are younger than 40 years.
Kasabach-Merritt syndrome
Kasabach-Merritt syndrome can present with diffuse petechiae and ecchymosis in association with a large, soft-tissue mass. If platelet counts drop low enough, spontaneous hemorrhage may result.
Related eMedicine topic:
Arteriovenous Fistulas
Indications
Intramuscular hemangiomas
The natural history of many intramuscular hemangiomas is that of gradual fatty replacement, atrophy, and involution over time, as suggested by their greater frequency in individuals younger than 30 years and relative rarity in older adults. Many intramuscular hemangiomas are asymptomatic or produce only mild symptoms with activity, even during the active adolescent years. Treatment may be considered if pain is substantial, but because of the poor success rate of treatment and the apparent self-limited nature of most intramuscular hemangiomas, indications for treatment are few. The more localized the extent of the disease, the more likely it is to be controlled successfully by surgical excision.
Synovial hemangiomas
The natural history of synovial hemangiomas may be similar to that of their intramuscular counterparts, but their rarity makes this difficult to document. The focal type more frequently is amenable to surgical excision than is the diffuse type.
Osseous hemangiomas
Hemangiomas of bone frequently are asymptomatic and may never require any treatment. Indications for treatment are based on symptoms.
Hemangiomatosis
Both osseous hemangiomatosis and skeletal-extraskeletal angiomatosis often become symptomatic during childhood, with pain and diffuse swelling. Perhaps even more significantly, extraskeletal manifestations of hemangiomatosis can lead to hepatic dysfunction and cardiac complications. Because of the extensive nature of the disease, chemotherapy has been used with some success.
Gorham disease
The natural history of Gorham disease is poorly defined. Extent and pace of bone loss are variable. However, because of the unpredictable natural history and the potentially devastating effects of progressive disease, treatment with steroids and/or radiation therapy generally should be instituted upon diagnosis.
Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is a potentially life-threatening coagulopathy that is related to platelet trapping in a large cavernous hemangioma. Approximately 30% of patients who develop this complication die from hemorrhage or infection. Surgical resection of the hemangioma often is difficult. Consequently, steroids, radiation therapy, interferon alfa-2a, and pentoxifylline have been used in attempts at treatment.
Tumor-induced osteomalacia
Tumor-induced osteomalacia results in diffuse osteopenia with marked hypophosphatemia, low serum calcium, and increased serum alkaline phosphatase. Because osteomalacia generally resolves with excision of the tumor, surgical treatment usually is indicated.
Contraindications
Contraindications to surgery are lack of symptoms, failure to attempt nonoperative measures, and threat to life or limb should surgery be performed.
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Further Reading
Keywords
hemangioma, benign vascular tumor, senile hemangioma, cherry hemangioma, strawberry nevus, visceral hemangioma, intramuscular hemangioma, hemangioma of the bone, hemangiomatosis, skeletal-extraskeletal angiomatosis, vertebral hemangioma, Kasabach-Merritt syndrome, tumor-induced osteomalacia, Gorham disease, disappearing bone disease, osteolysis, hemangiomatous disease, enchondromatosis, Maffucci syndrome, synovial hemangioma, osseous hemangioma
