Aneurysmal bone cyst
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Aneurysmal bone cyst (ABC) accounts for 1% to 2% of all primary bone tumors. The incidence of aneurysmal bone cyst is approximately 0.14 per 100,000 individuals worldwide. Adolescents and children are most affected by aneurysmal bone cyst. The age distribution of aneurysmal bone cyst is between 1.5-25 years. The mean age of the patients with aneurysmal bone cyst is 16 years. Women are more commonly affected than men, with a 1:0.84 ratio. In 1942, Jaffe and Lichtenstein first described aneurysmal bone cyst. Aneurysmal bone cyst can be classified based on mechanism of origin into primary and secondary. The true causes of aneurysmal bone cyst are not known. ABCs usually present in the long bones such as the humerus, femur, tibia and fibula. About 25% of ABC's are seen in posterior element of spine. ABC's typically occur in the metaphysis of the long bones. The hallmark of ABC is Pain, swelling and pathological fracture. The mainstay of treatment for ABC is surgery in form of curettage and bone grafting.
- In 1942, Jaffe and Lichtenstein first described aneurysmal bone cyst.
- In 1940, Ewing propoosed that aneurysmal bone cyst was caused by increased venous pressure.
- In 1956, Cruz and Coley also supported Ewing's hypothesis.
- In 1956, Barnes hypothesized that aneurysmal bone cyst was due to an abnormal response to injury.
- In 1956, Otterdoom after a thorough pathological study, concluded that aneurysmal bone cyst was an angioma of bone.
Mechanism of Origin
Primary Aneurysmal Bone Cyst(ABC)
- When ABC arises de novo, it is called primary ABC.
- About 70% of ABC's are primary in origin.
Secondary Aneurysmal Bone Cyst(ABC)
- When ABC coexist with other bone lesions, it is termed as secondary ABC's.
- About 30% of ABC's are secondary in origin.
- Commonly associated bone lesions include:
Enneking (MSTS) Staging System
- The Enneking surgical staging system (also known as the MSTS system) for benign musculoskeletal tumors based on radiographic characteristics of the tumor host margin.
- It is widely accepted and routinely used classification.
|1||Latent: Well demarcated borders|
|2||Active: Indistinct borders|
|3||Aggressive: Indistinct borders|
- The exact pathogenesis of aneurysmal bone cyst is not fully understood.
- Various theories have been proposed concerning the pathogenesis of aneurysmal bone cyst:
- Local alteration in hemodynamics resulting in markedly increased venous pressure and development of a dilated vascular bed within the involved bone.
- Primary ABCs demonstrate a t(16;17)(q22;p13) fusion of the TRE17/CDH11-USP6 oncogene leading to increased cellular cadherin-11 activity which arrests osteoblastic maturation in a more primitive state.
- ABCs usually present in the long bones such as the humerus, femur, tibia; and fibula.
- About 25% of ABC's are seen in posterior element of spine.
- ABC's typically occur in the metaphysis of the long bones.
- The true causes of aneurysmal bone cyst(ABC) are not known.
- Commonly proposed theories are as follows:
Differentiating Aneurysmal Bone Cyst from Other Diseases
Aneurysmal bone cyst must be differentiated from following bone disorders:
|Disease||Bubbly lytic lesion on x-ray||Lakes of Blood on histology||Diagnosis||Treatment is curretage and bone grafting|
|Aneurysmal bone cyst||+||+||Radiology and biopsy||+|
|Unicameral bone cyst||+||-||Radiology and biopsy||-|
|Non ossifying fibroma||+||-||Radiology and biopsy||-|
|Giant cell tumor||-||-||Radiology and Biopsy||+|
|Chondromyxoid Fibroma||-||-||Radiology and biopsy||+|
|Osteoblastoma||-||-||Radiology and biopsy||+|
|Telangiectatic osteosarcoma||-||+||Radiology and biopsy||-|
Epidemiology and Demographics
- ABC accounts for 1% to 2% of all primary bone tumors.
- The incidence of aneurysmal bone cyst is approximately 0.14 per 100,000 individuals worldwide.
- Adolescents and children are most affected by aneurysmal bone cyst.
- The age distribution of aneurysmal bone cyst is between 1.5-25 years.
- The mean age of the patients with aneurysmal bone cyst is 16 years.
- Women are more commonly affected than men, with a 1:0.84 ratio.
- There is no racial predilection to aneurysmal bone cyst.
There is insufficient evidence to recommend routine screening for aneurysmal bone cyst.
Natural History, Complications, and Prognosis
- Common complications of aneurysmal bone cyst include:
- Prognosis is generally excellent for aneurysmal bone cyst.
- Factors that influence the outcome of the aneurysmal bone cyst include:
Diagnostic Study of Choice
- Biopsy is the diagnostic study of choice for the diagnosis of aneurysmal bone cyst.
- Gross appearance of aneurysmal bone cyst include:
- Blood-soaked sponge.
- A thin subperiosteal shell of new bone surrounds the structure
- Cystic blood-filled cavities.
- The tissue within shows brownish intertwining septa.
- The stroma contains proliferative fibroblasts, spindle cells, areas of osteoid formation, and an uneven distribution of multinucleated giant cells that tend to surround the fluid-filled cavities in a pigs at the trough formation.
- HIstological appearance includes:
History and Symptoms
- The majority of patients with aneurysmal bone cyst have a positive history of:
- Common physical examination findings of aneurysmal bone cyst include:
There are no diagnostic laboratory findings associated with aneurysmal bone cyst.
- An x-ray may be helpful in the diagnosis of aneurysmal bone cyst.
- Findings on an x-ray suggestive of aneurysmal bone cyst include include:
- Radiologically aneurysmal bone cyst can be stratified into 4 phases:
- Initial phase 1 – Small, lytic lesion without evidence of lifting off of the periosteum.
- Phase 2 - Rapidly enlarging demonstrating a characteristic blowout appearance.
- Phase 3 - Growth slows or stops after some sort of intervention.
- Phase 4 - Healing with progressive ossification and calcification of the cavity.
Echocardiography or Ultrasound
- CT scan confirms the x-ray findings.
- In addition, CT demonstrates internal septation such as calcified rim, giving an eggshell appearance, which may be completely or partially intact.
- Fluid-fluid levels can also be seen.
- Magnetic resonance imaging (MRI) are similar to those from CT scan.
- However, MRI can more specifically reveal blood within the lesion as well as extension into the soft tissues.
Other Imaging Findings
- Increased uptake is observed around the lesion of aneurysmal bone cyst.
- Findings often demonstrate a halo effect of increased radionuclide uptake surrounding an area of little uptake.
Other Diagnostic Studies
- Angiography demonstrates a hypervascular area around the aneurysmal bone cyst.
- An intense diffuse area of persistent contrast accumulation may be visualized without main afferent or efferent vessels observed.
- It can be helpful to plan selective arterial embolization as the primary treatment or as a preoperative method to help control intraoperative blood loss.
Aggressive curettage with adjuvant and bone grafting
- Symptomatic aneurysmal bone cyst without acute fracture
- Local recurrence in up to 25% and more common in children with open ephyses.
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