British Journal of Radiology (2006) 79, e64-e66
© 2006 British Institute of Radiology
doi: 10.1259/bjr/53131368
Intraosseous haemangioma of the proximal femur: imaging findings
A Chawla, DMRD1,
M Singrakhia, MS2,
M Maheshwari, MD1,
N Modi, MS2 and
H Parmar, MD1
Departments of 1Radiology and 2Orthopaedics, King Edward VII Memorial Hospital, Parel, Mumbai - 400012, India
Correspondence: Dr Mohit Maheshwari, 10, Ava Road, #09-06 Ava Towers, Singapore 329949
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Abstract
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Haemangiomas of bone are uncommon lesions, accounting for approximately 1% of all primary bone tumours. The most frequent sites of involvement are the calvaria and the vertebral column. When haemangiomas involve long tubular bones, they are usually found in the diaphysis or metadiaphysis. Juxta-articular or epiphyseal location for a long bone haemangioma is rare. We present the imaging findings in a case of a histopathologically proven juxta-articular intraosseous haemangioma of the proximal femur. We believe ours is the first report of a haemangioma involving the proximal end of the femur.
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Case report
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A 38-year-old man presented with gradually increasing right hip pain over 4 months. There was no evidence of previous trauma or fever. There was no history of other medical or surgical illness. On examination, there was restricted hip joint movements and tenderness. Baseline haematological examination was normal. A plain radiograph of the hip showed a 4 cm x 3 cm osteolytic lesion involving the right femoral head, extending into the femoral neck. The lesion had a narrow zone of transition with an unremarkable matrix with focal expansion on its lateral aspect (Figure 1
). The soft tissues, contralateral hip joint and remaining pelvis were normal. MRI was performed on a 0.2 Tesla open MR system (Magnetom Open Viva; Siemens Medical Systems, Erlangen, Germany), with images obtained in axial, coronal and sagittal planes. Spin echo (SE) images were obtained with repetition time/time to echo/number of acquisition (TR/TE/NA) of 528/26/2 for T1 weighted images (WI) and 2840/102/3 for T2 WI with 4 mm slice thickness and 1 mm intersection gap. On T1 WI, the lesion was isointense (to muscle) (Figure 2a). It was markedly hyperintense on T2 WI and short tau inversion recovery (STIR) images (Figure 2b). There were no fluid–fluid levels within the lesion. Minimal joint effusion and bone marrow oedema in the right acetabulum were also noted (Figure 2b). A bone scan revealed a solitary focus of mild increased uptake in the right femoral head. Based on the patient's age and imaging features, a differential diagnosis of a giant cell tumour (GCT), aneurysmal bone cyst (ABC) and plasmacytoma were considered.
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Figure 1. Frontal radiograph of both hip joints demonstrating an osteolytic lesion in the head of the right femur with a narrow zone of transition. The lesion is causing minor expansion laterally.
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Figure 2. (a) T1 weighted imaging axial image at the level of the femoral head shows an isointense (to muscle) lobulated mass lesion involving the femoral head. (b) Short tau inversion recovery (STIR) coronal image at the level of the femoral head shows this lesion to be markedly hyperintense. The right acetabulum also shows bone marrow oedema.
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While excision biopsy could have been performed for this lesion, we decided to perform excision of the tumour followed by an uncemented total hip arthoplasty. On gross examination, the mass appeared as a vascular irregular growth arising from the superolateral quadrant of the femoral head. Frozen section pathology showed regular patterns of mononuclear cells with multiple vessels, suggestive of a benign bone tumour. Histopathology revealed multiple thin-walled vascular spaces with endothelial lining of cells and stuffed with red blood cells. Specks of calcification and border sclerosis were also noted (Figure 3). There was no evidence of malignancy. The final diagnosis was of a haemangioma of the head of femur. On 6 month follow-up, the patient was asymptomatic with no radiological evidence of recurrence.
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Figure 3. Histopathology of the excised specimen shows collections of fine thin walled vessels stuffed with red blood cells. Specks of calcification and borders of sclerosis are also seen.
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Discussion
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Osseous haemangioma accounts for less than 1% of all primary bone tumours [1–3] and most frequently involve the bones of the axial skeleton. The peripheral, long, tubular bones and the ribs are the least affected sites [1, 2, 5]. Haemangiomas of the tubular bones can be broadly divided as medullary, periosteal and intracortical. The medullary type is further subdivided into diaphyseal (48%), metadiaphyseal (30%), metaphyseal (12%), metaepiphyseal (4%), epimetadiaphyseal (3%) and epiphyseal (1%) [6]. Epiphyseal or juxtarticular location is rare with only two cases reported in the literature; neither of these involved the femur [4, 5].
Haemangiomas involving the axial skeleton are commonly asymptomatic [3] and may remain undetected for long time. They are often discovered incidentally during a radiographic study [3], whereas haemangiomas of the appendicular skeleton are often symptomatic. In one series, peripheral haemangioma were symptomatic in 91% of cases, out of which 37% had pain, 7% had swelling and 7% had restriction of function due to pathological fracture [6]. The rate of pathological fracture depends upon the type of bone involved and whether this bone is weight-bearing. In our case the tumour was located in the weight-bearing posterolateral aspect of the femoral head and was at risk of pathological fracture.
The histopathological diagnosis of a haemangioma is not difficult in an en bloc specimen. However, diagnosis from biopsy or tissue curettage is challenging to the pathologist. This is due to the destructive nature of these procedures, disrupting thin-walled blood vessels and resulting in histological sections showing non-diagnostic empty spaces with scattered bone trabeculae. This causes hindrance in the pre-operative diagnosis of haemangioma by any invasive procedure [6]. Histologically, haemangiomas can be classified as cavernous, capillary, venous or mixed, depending on the type of vascular involvement [6]. Cavernous haemangioma is the most common type in the peripheral bones and accounts for up to 50% of all cases reported [6]. They are predominant in the medullary and the intracortical portion of the bone and are not yet reported in the subarticular epiphyseal bone. Pure capillary haemangioma accounts for 10% of all these types as reported in the literature [7, 8]. The other varieties are rare.
Intraosseous haemangiomas may have a variety of radiological appearances. Due to the lack of consistent features and rarity, it is often difficult to radiologically diagnose these lesions pre-operatively [6]. On radiographs, haemangiomas may show a coarse loculated, sunburst, moth-eaten, and soap bubble appearance due to expansive proliferation of engorged vessels and thickened, remodelled bone trabeculae [7]. An expansile osteolytic appearance is their least common presentation [9]. On MRI, haemangiomas show a variable appearance and may demonstrate low, intermediate or high signal intensity on T1 WI. Haemangiomas may sometimes appear hyperintense on T1 WI because of their fat content. They are usually hyperintense on T2 WI or STIR [10]. Hyperintensity seen on T2 WI is due to free water in stagnant blood within the haemangioma. On STIR images, these lesions appear markedly intense due to suppression of normal bone marrow. Haemangiomas can show marked to minimal or no enhancement after contrast administration [11]. In our case, the lesion was hypointense on T1 WI and markedly hyperintense on T2 WI and STIR images.
The differential diagnosis for a long bone haemangioma in juxta-articular location includes GCT, ABC and plasmacytoma. Other rare possibilities are metastasis and a brown tumour. GCTs predominately arise in long tubular bones [12], with their epicentre in the epiphysis. The radiographic signs of GCT include a geographical radiolucency without internal mineralization, thinning of the cortex and eccentric location in the epiphysis of long bones. ABCs are common in a younger age group with 80% occurring in patients under 20 years of age [10]. Most lesions are metaphyseal; however, they can extend to the epiphyseal end after the growth plate is closed [13]. On MRI, ABCs tend to be well circumscribed but heterogeneous. They often show fluid–fluid levels [14, 15]. Plasmacytomas have a typical radiographic appearance of a geographical radiolucent lesion, which is highly expansile with a soap bubble internal architecture [13]. Metastases are commonly multiple, with solitary metastatic lesions occurring in only 10% of patients [16]. An osteolytic appearance is the most common radiographic feature, present in 75% of all metastatic lesions [13]. Brown tumours are usually central, slightly expansile lightly septated lesions, mimicking destructive neoplasms. They are associated with other features of hyperparathyroidism [13]. In our case the lesion was solitary, with no clinical suspicion suggesting metastasis or a brown tumour.
In conclusion, haemangiomas can present with bizarre imaging appearances and should be included in the differential diagnosis of an osteolytic and expansile lesion of the epiphysis, especially in asymptomatic or mildly symptomatic patients. However, the diagnosis can only be confirmed by histopathological examination. If correctly diagnosed, such tumours in a non-weight bearing region can be managed conservatively, as with spinal haemangiomas [17]. However, symptomatic or aggressive lesions should be treated as a GCT or an ABC.
Received for publication May 10, 2004.
Revision received May 9, 2005.
Accepted for publication September 2, 2005.
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