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]
An image depicting a hemangioma can be seen below.

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.
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, redpurple 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, 9]

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.
[10]
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.

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.

Epidemiology
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.[11] Watson and McCarthy estimated that intramuscular hemangiomas
accounted for 0.8% of all benign vascular tumors.[12] 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 childhood and adolescence,
with few reports of the disease in infants.[13]
Osseous hemangiomas
Hemangiomas of bone accounted for approximately 1% of primary bone tumors of which
biopsies were taken in Dahlin and Mirra's series.[14] 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 10-12% of autopsy specimens and have a slight predominance in females (ratio
2:1).[15]
Hemangiomatosis
Hemangiomatosis and skeletal-extraskeletal angiomatosis are rare conditions.
Gorham disease
Gorham disease (ie, massive osteolysis, disappearing bone disease) is very rare (see image
below). The disease typically occurs in the second or third decade of life with most patients
younger than 40 years.

Radiograph of a patient with Gorham disease

showing dissolution of bone.

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 factorbeta,
transforming growth factorbeta) have been implicated in the development of hemangiomas.[16]
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.[17]
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.

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
thought to be related to excess vascularity of the involved bone. The exact pathogenesis of this
process is unknown at this time. Newer theories involve hormone and cytokine stimulation of
osteoclast function with heightened sensitivity of osteoclast precursors to interleukin-6 leading to
increased bone resorption locally.[18]

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, progressive onset of
pain, limited range of motion, limping, and even mechanical symptoms suggesting intra-articular
derangement.[19, 13] A palpable, spongy, compressible mass may be present, and it may decrease in
size with elevation of the extremity. The synovium is particularly hypertrophic and causes
recurrent hemarthrosis. The knee is by far the most common joint involved, where the
presentation may be confused with meniscal or ligamentous pathology.[20] Both localized and
diffuse forms exist.
Osseous hemangiomas

Hemangiomas of bone are often incidental findings and the vast majority are asymptomatic but
may cause pain and swelling. Those in the skull may be associated with swelling, erythema,
tenderness, or facial deformity. Although rare, representing only 1-2% of lesions, a locally
aggressive subtype of vertebral hemangiomas is now well reported. These locally aggressive
subtypes may cause problems ranging from back pain to nerve root or cord compression and
fractures. These locally aggressive vertebral hemangiomas can mimic other pathologies such as
primary bony malignancy or metastatic disease and therefore must be accurately diagnosed.[21, 22]
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. Skeletalextraskeletal 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 localized aching pain, swelling, progressive deformity,
weakness, and/or muscle atrophy. Patients are often asymptomatic during the osteolytic process
until a pathologic fracture occurs or a mass develops. Hemangiomas and vascular malformations
can also be present in as many as 60% of patients. [18] The disease is rarely suspected prior to
radiographic evaluation. Patients are usually 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.

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

Symptomatic or locally aggressive vertebral hemangiomas are usually treated conservatively

when symptoms are limited to mild-to-moderate pain, whereas neurologic deficit and intractable
pain are widely accepted in the literature as common indications for surgical intervention. [23] See
Treatment for more details.
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. No standard treatment is currently available for Gorham disease. Regression of lesions
or stabilization of disease has been reported with steroids, radiation, surgery, bisphosphonates,

zoledronic acid, and interferon-.[18] Because of the unpredictable natural history and the
potentially devastating effects of progressive disease, treatment 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.