Anuerysmal bone cyst

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Aneurysmal Bone Cyst (ABC)

Definition

An aneurysmal bone cyst is a locally destructive, blood-filled, reactive lesion of bone - not a true neoplasm. It consists of multiloculated, hemorrhagic cystic spaces within an expanding shell of bone.

Epidemiology

  • Primarily affects patients younger than 20 years (1st-2nd decades)
  • Slight female predominance
  • Any bone can be involved

Common Locations

SiteNotes
Proximal humerusMost common long bone site
Distal femurFrequent
Proximal tibiaFrequent
Spine (posterior elements)15-20% of cases; may extend into vertebral body
Flat bonesLess common
The lesion arises eccentrically in the metaphysis and does not cross the growth plate.

Pathogenesis

Two mechanisms are recognized:
1. Primary (de novo): Spindle-shaped cells show rearrangements of chromosome 17p13, causing fusion of USP6 gene coding sequences to regulatory elements of COL1A1 (most common) or other genes. This leads to USP6 overexpression - a deubiquitinating enzyme that upregulates NF-kB, which in turn increases matrix metalloprotease expression, causing cystic bone resorption.
2. Secondary (up to 30% of cases): ABC-like changes arise within other bone tumors, including:
  • Giant cell tumors
  • Chondroblastomas
  • Osteoblastomas
  • Fibrous dysplasia
  • Nonossifying fibromas
  • Chondromyxoid fibromas
A local circulatory disturbance causing increased venous pressure and hemorrhage has also been proposed.

Morphology

Radiography

  • Eccentric, expansile, lytic, metaphyseal lesion with well-defined margins
  • Thin shell of overlying cortical bone (periosteum elevated but intact)
  • May occasionally have a permeative appearance mimicking malignancy

CT

  • Delineates complex anatomy (spine, pelvis)
  • Identifies the thin cortical rim
  • Shows multiloculated architecture

MRI (most informative)

  • Fluid-fluid levels - the hallmark finding (arrow in image below)
  • Double-density fluid levels + intralesional septations distinguish ABC from unicameral bone cyst
  • Multiloculated cavities clearly demonstrated

Bone Scan

  • Diffuse or peripheral tracer uptake with central area of decreased uptake ("doughnut sign")
ABC - MRI fluid-fluid levels (A) and gross hemorrhagic sponge-like appearance of bisected proximal fibula (B)
Fig. 19.26 - Robbins & Kumar Basic Pathology: (A) Axial MRI showing characteristic fluid-fluid levels (arrow). (B) Gross appearance - hemorrhagic, sponge-like bisected proximal fibula.

Gross

  • Multiple blood-filled cystic spaces separated by thin tan-white septa
  • Surrounded by a thin layer of bone covered by raised periosteum

Microscopy

  • Cystic spaces lack an endothelial lining
  • Septa contain:
    • Plump uniform fibroblasts and histiocytes
    • Multinucleate osteoclast-like giant cells
    • Hemosiderin-laden macrophages
    • Reactive woven bone
    • Chronic inflammatory cells
Histologic appearance of ABC - H&E showing fibroblasts, giant cells, and hemorrhagic stroma
Fig. 27.18 - Campbell's Operative Orthopaedics: Histologic appearance showing compressed fibroblasts, histiocytes, giant cells, and hemosiderin-laden macrophages.

Clinical Features

  • Localized pain and swelling (present for weeks to months)
  • Limitation of motion
  • Rapid growth can mimic a malignancy clinically
  • Spinal lesions may cause neurologic deficits or radicular pain
  • Although benign, ABCs are locally aggressive

Differential Diagnosis

The radiographic and clinical appearance overlaps with:
  • Giant cell tumor - most important mimic (especially in adults)
  • Enchondroma
  • Unicameral (simple) bone cyst - distinguished by centrally located unilocular cyst with clear fluid
  • Telangiectatic osteosarcoma - most dangerous mimic; biopsy mandatory

Solid Variant

A solid variant of ABC exists, frequently referred to as giant cell reparative granuloma, which lacks the classic blood-filled spaces.

Treatment

SettingPreferred Treatment
Standard long bone lesionExtended curettage + bone graft substitute
Expendable bonesMarginal resection
Spine/pelvisPreoperative arterial embolization to minimize blood loss, then curettage
Inaccessible lesionsPercutaneous sclerotherapy (fluoroscopic guidance)
High surgical riskDenosumab (anti-RANKL monoclonal antibody)
Historical/rarely usedLow-dose radiation (risk of malignant transformation precludes routine use)
Key surgical note: Tourniquet control is advised due to heavy bleeding potential.

Recurrence

  • After curettage: 10-20% recurrence rate (Robbins: up to 50% in some series)
  • Risk factors for recurrence:
    • Age younger than 15 years
    • Centrally located cysts
    • Incomplete removal of cystic cavity contents
  • Recurrent cysts can be retreated with the same approach

Summary Comparison: ABC vs Unicameral Bone Cyst

FeatureABCUnicameral BC
Age1st-2nd decade1st-2nd decade
SexSlight female predominanceMale:female 2:1
LocationEccentric, metaphysealCentral, metaphyseal
Common sitesProximal humerus, distal femur, tibia, spineProximal humerus, proximal femur
MRIFluid-fluid levels, double-density, septaSimple cyst, no septa
FluidBlood-filledStraw-colored fluid
PresentationPainUsually silent; pathologic fracture
TreatmentExtended curettageObservation/aspiration/injection

Sources:
  • Robbins & Kumar Basic Pathology, p. 787 (Robbins Pathology)
  • Campbell's Operative Orthopaedics 15th Ed 2026, pp. 1172, 4548-4549
  • Grainger & Allison's Diagnostic Radiology, p. 1959
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