Aneurysmal Bone Cyst

Definition

  • A benign cystic lesion of bone that expands the cortex.
  • It is the only bone lesion that derives its name from its X-ray appearance rather than its histology.
  • Term first used by Jaffe & Lichtenstein in 1942

Classification

  • primary (arise de novo)
  • secondary (arise in pre-existing tumours, frequently GCTs)
    • More than 50% of ABCs arise from preexisting lesions

Incidence

  • 2.5% of all primary bone tumours.
  • Half as frequent as giant cell tumours
  • M=F
  • 80% in the first two decades of life
  • In contrast, only 15% of patients with GCT are in the first two decades of life

Aetiology

  • Unknown
  • Trauma may be related
  • nearly 1/2 are seen to occur in conjunction with another benign tumor and may represent a breakdown in the body’s reaction to the other tumor

Localization

  • Can involve any bone.
    • 1. Proximal end of long bones
      • In long bones involves the metaphysis but can cross the growth plate.
    • 2. Spine
      • In the spine involve the posterior elements (Differential Diagnosis here is osteoblastoma).
  • ABC is the commonest benign tumour of the clavicle

History

  • Pain
    • usually mild and intermittent
    • may be Rapid ↑ in pain
  • Swelling
    • Swelling will tend to increase until the lesion is treated
  • Rarely, a pathologic fracture
  • Neurological
    • If the lesion is located in the vertebral column, it may cause signs and symptoms of spinal cord compression (leg weakness, bowel or bladder dysfunction).

Examination

  • May be a mass or neurological symptoms if the spinal cord is compressed

Investigations

Xray

  • Expansile, multilocular balloon disrupting the adjacent bone & elevating the periosteum
    • Most commonly, an area of lucency situated eccentrically in the medullary cavity of a long bone.
    • Less commonly, may be situated centrally within the medulla.
    • Much less commonly, may arise in the cortex or periosteum.
  • No matrix mineralization is present in the lesion
  • Most ABCs are completely lytic
  • ABCs may cross joints & involve several bones
    • particularly in the spine where several adjacent vertebrae & ribs may be involved.
  • lesion tends to involve the cortex & may destroy it completely, when it may bulge out into the soft tissue where it usually forms a thin rim of calcification.
  • margins can be poorly or well defined; in half of cases the X-ray appearances suggest a benign process, & in a small number of cases they may suggest malignancy.
  • Periosteal new bone formation causing a buttress effect is characteristic.
  • There is no matrix calcification

CT & MRI

  • internal septae & fluid levels with a layering effect.
  • Fluid levels (T2 weighted scans)
  • Finger in balloon sign
    • preservation of a cortical cuff extending for a short distance into the expanded area of destructive blowout

Pathology

Gross pathology

  • Red brown granular material (haemosiderin deposition)
  • “a hole containing blood”
  • blood in the lesion is not clotted
  • no endothelial lining
  • pressure in an ABC may be elevated to arteriolar levels

Histology

  • Essential feature is cavernomatous spaces,
  • with walls that lack the normal features of blood vessels, such as muscle or elastic lamina
  • Thin strands of bone are often present in the fibrous tissues of the walls
  • septae almost invariably contain giant cells; this helps to distinguish from unicamerla bone cyst
  • Solid areas contain spindle cells that are loosely arranged
  • Chondroid like zones of calcification in solid portions of septae are commonly found & are relatively specific

Behaviour

  • ABCs are usually aggressive lesions associated with major bone destruction, pathological fractures & local recurrence
  • Spontaneous malignant transformation not recorded in the Mayo files but occurs very occasionally

Differential diagnosis (histology)

  • Giant cell tumour
  • Giant cell reparative granuloma
  • Low-grade osteosarcoma
  • Telangiectatic osteosarcoma – probably most difficult Differential Diagnosis
    • This disease is uncommon & rarely involves the vertebrae or small bones of the hands or feet
  • Renal cell carcinoma metastasis

Associated Conditions

May occur in other benign bone tumors or processes

  • Giant cell tumor
  • Chondroblastoma
  • Osteoblastoma
  • Fibrous dysplasia
  • NOF

Treatment

  • Intralesional curettage & bone grafting & chemical cauterization of cyst walls
    • treatment of choice
  • Lesions in expendable bones are excised
  • Preoperative Embolization
    • If the lesion has a large soft tissue component or is large (>5-6cm) should consider preoperative embolization
    • Especially pelvis, sacrum, vertebral bodies
  • Even incomplete resection may be followed by regression of the lesion
  • High recurrence rate
    • Recurrence tends to occur within 6-18 months. It is very rare after 2 years.
    • Recurrence rate is higher in spinal tumours
  • Radiotherapy
    • has no role unless surgery is impossible.
    • It used to be used adjuvantly but no longer is used to minimize the risk of sarcomatous transformation

Prognosis

  • 95% of patients cured
  • ↑ rate of recurrence in younger patients
  • Children with open physes are much more prone to local recurrence (up to 50%)