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Aneurysmal Bone Cyst

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DEFINITION AND PATHOGENESIS

  • Benign lesion with cyst-like walls filled with blood
  • Vascular anomaly may be begun by trauma or tumor

IMPORTANCE

  • Can be mistaken for a malignancy due to fast rate and destructive nature of early growth as well as exuberant cellularity
  • May be associated with other lesions, particularly
  • Fibrous dysplasia
  • GCT
  • Chondroblastoma
  • Osteoblastoma
  • Osteosarcoma
  • NOF

CLINICAL FEATURES

  • Most occur prior to age 20, rarely after age 50
  • Mean age for pelvic lesions 15.5 (2-44)
  • Symptoms may be of short duration, often <3mos, with pain ? swelling
  • Average duration of sxs for pelvic lesions 7 months
  • 95% of pelvic lesions present with pain (thigh, groin, LBP, buttocks, leg), 40% with ? hip ROM, 10% with mass (Ö rectal exam), neurologic signs/sxs (67% of sacral lesions) including urinary retention, ? anal tone
  • M:F = 1:1.16 from compiled data
  • Neurological sxs
  • Hemothorax froma a T4 ABC has been reported
  • Has been reported after fx or trauma (up to 23%)
  • Has been reported causing proptosis and epiphora in an ethmoid location
  • Labyrinthine fistula reported from ABC within fibrous dysplasia

RADIOLOGIC FEATURES

  • Expansile, often involving long bones, pelvis, posterior spinal elements, and may become massive in size
  • Extension across the physis is rarely reported
    • Deformity and limb length discrepancy reported
  • ?40-44% of spinal lesions involve multiple vertebrae
  • Anterior extension and "vertebral plana" may occur
  • Three (not always distinct) phases:
    • Early, incipient, or initial phase: lytic area may be small or large, geographic or poorly circumscribed
    • Midphase or proliferation stage: extreme cortical destruction, "blowout", Codman's triangles, very worrisome aggressive features, including an incomplete rim of periosteal bone, and the "finger-in-the-balloon" sign
    • Late, equilibrium, or inactivation phase: bone's reaction to the lesion has caught up, with rim of periosteum
  • Campanacci radiographic classification system
    • Type 1: the cyst is within the center of the bone with little or no cortical expansion
    • Type 2: the cyst is within the entirety of the involved segment of bone with severe cortical expansion
    • Type 3: the cyst is eccentrically located within the bone with little or no cortical expansion
    • Type 4: the cyst is supbperiosteal with erosion of the cortex (least common)
    • Type 5: the cyst expands into the soft tissues with periosteal elevation, cortical destruction, and cancel-lous osteolysis
  • ± pathological fx (up to 10% reported)
  • Uncommonly (~10%) with intralesional densities
  • Cortical thinning and trabeculation
  • Bone scan: peripheral uptake
  • Angiography: hypovascular lesions with localized regions of hypervascularity
  • Fluid-fluid levels on MRI (85%) when blood within the cysts layer out below serum in the cysts and septations
  • Has been reported in monozygotic twins
  • Unusually may cross physeal plate
  • Unusual location

GROSS PATHOLOGY

  • Bloody!, as a sponge or a honeycomb filled with (unclotted) blood and fibrous cyst-like walls

HISTOLOGIC AND MOLECULAR FEATURES

  • Fibrous cyst-like walls (not always seen because curettage fragments the flimsy lining)
  • Blood-filled spaces (may be washed out of most cavities by formalin)
  • Fibrous stroma with moderate to exuberant cellularity, esp in the early phase
  • May have intramural calcified chondroid (up to 30%) or slivers of osteoid or woven bone
  • Osteoclast-like giant cells scattered in the walls in variable numbers
  • 'Pseudosarcomatous' nature of rare lesions
  • ABCs harbor translocation of TRE17/USP6** Transcriptionally upregulated
    • TRE17 encodes ubiquitin-specific protease (USP)
    • TRE17 encodes a TBC domain that mediates binding to Arf6 GTPase
    • TRE17 induces expression of MMP-9 and MMP-10
      • MMP-9 transcription induced through activation of nuclear factor-kappaB
        • Mediated (in part) by GTPase RhoA and its effector kinase, ROCK
      • Requires USP but not Arf6 binding
      • Induces formation of tumors with ABC features
    • SS18 rearrangement has also been described in addition to USP6

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Juxtacortical chondroma in periosteal lesions without periosteal rim, but with a sclerotic cortical margin
  • Osteosarcoma, particularly telangiectatic and fibroblastic variants
  • GCT
  • Osteoblastoma
  • Fibrous dysplasia
  • Chondroblastoma with ABC changes
  • CMF with ABC changes
  • Osteomyelitis

DISEASE COURSE AND TREATMENT

  • Never metastasizes, although LR (0-59% reported) may occur (esp skeletally immature pts)
  • Median time to LR = 17 mos, rare > 2 yrs
  • LR after curettage/burring/cementation ?5-17%
  • LR after curettage/bone graft ?20-50% (only one had burring)
  • LR after curettage and adjunctive cryotheraphy ~4-18%, adjunctive phenolization ~12-20%
    • Cryotherapy LR of 5% reported successfully txd with 2nd curettage and cryotherapy
  • (Wide cortical saucerization with curettage, cryotherapy or phenolization recommended tx)
  • LR after marginal excision reported 0%
  • Spontaneous healing has been reported (rare)
  • Usually growth arrest occurs when epiphysis is involved (and has been reported without direct physeal involve-ment)
  • Juxtaphyseal lesions may be treated with curettage and expectation of normal physeal growth reported
  • Subperiosteal resection with reconstitution of fibula has been reported
  • Spinal lesions (?20% of locations) may compress nerve roots or spinal cord
  • En bloc excision for small tubular bones of the hand described with 8% LR
  • Cavitational ultrasonic surgical aspirator (Cavitron) has been used to fascilitate resection
  • 10% LR within 10 yrs, 2nd LR in 16.7% within 9 yrs
  • Preoperative selective arterial embolization, complete curettage, bone grafting, ? fusion
  • XRT has been used in spinal lesions, but is contraindicated now because of postradiation sarcoma
  • Embolization alone after diagnostic bx can be curative, hastening maturation of the lesion
  • Percutaneous repetitive sclerotherapy** Polidocanol is used
    • Used successfully in pelvic and spine lesions
  • Percutaneous CT-guided injection of DBM mixed with bone marrow concentrate reported in sacral lesions
  • Percutaneous embolization(s) with alcohlic solution of Zein reported successful unless marked venous drainage apparent
  • Postoperative fx in up to 10% reported

SPECIAL CONSIDERATIONS


SOLID ABC VARIANT

  • Rarely reported in the spine
  • Lesion may have little or no areas of blood filled spaces, with areas of exuberant spindle cells
  • Osteoid or chondroid may mislead to a dx of OSA
  • Reparative nature of the lesion indicated by mature areas of hypocellularity
  • Osteoclast-like giant cells and lymphocytes may be seen

EXTRAOSSEOUS ABC

CONGENITAL ABC

METACHRONOUS/MULTIPLE ABC

METASTATIC ABC

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