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Giant cell tumor of bone

Giant cell tumor of bone is a locally aggressive bone tumor that typically affects young adults near the epiphysis of long bones. It appears lytic and expansile on imaging. Treatment has shifted from intralesional curettage, which has a high recurrence rate, to more extensive curettage with adjuvants like PMMA to fill the defect. For more advanced cases, en bloc resection is preferred but can require reconstruction. Close follow up is important due to the risk of local recurrence and rare pulmonary metastasis.

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Giant Cell Tumor of Bone
Definition • 10 bone neoplasm • First described Cooper 1818 • Generally benign but locally aggressive • Potential for : – Recurrence – Pulmonary metastasis – Frank malignancy
• Osteolytic tumour arising from epiphysis • Common in young adults • Though it is benign it is locally malignant
Epidemiology • 5-10% 10 bone tumors • 20% benign bone tumors • F : M 1.5 : 1 • 70-80% age 20-40yrs
SITES • Most common location –distal femur followed closely by the proximal tibia • In the distal radius(3rd most common location) these are frequently more aggressive
Presentation • Swelling with skin over the swelling stretched • Pain x wks. – mos(usually not a presenting feature • Mass • Pathologic # • Neuro deficit (spine / sacrum) • Incidental
• EGG SHELL CRACKING sensation may be present • Limitation of joint movement and pathological fractures –usually a late feature
Radiology • Lytic lesion near epiphysis • Eccentric or central • Narrow zone transition B/W tmr and surrounding tissue • Cortical thinning • expansile • No sclerotic margin • No periosteal bone formation
• Thin septa of bone traverse the interior producing soap bubble appearance • Joint extension usually not a feature • Cortex disrupted in late stages • Intra articular extension is rare as subchondral bone usually remains intact
Other modalities • CT – Integrity cortical rim • MRI _extent of lesion within the bone and soft tissue – Assess subchondral breakthrough – Lesion dark on T1 and bright on T2 wt • Bone Scan – Suspect multicentri loci – Uses very low radioactive material (disphosphonate) to see spread to other bone
CAMPANACCI’S GRADING • GRADE 1 :CYSTIC LESION • GRADE 2:CORTEX THIN BUT NOT PERFORATED • GRADE 3:CORTEX PERFORATED WITH EXTENSION INTO SOFT TISSUE
ENEKING’S STAGING • STAGE 1: LATENT-NO CHARACTERISTIC GROWTH OR PROGRESSIVE CHANGE,RESOLVE SPONTANEOUSLY • STAGE 2:ACTIVE-LESION DEFORM THE HOST BONE BUT REMAINS INSIDE BONE • STAGE3:AGGRESSIVE-TUMOUR EXTEND BEYOND THE BONE
Histology • Fibrohistiocytic origin • Multinucleated giant cells (40-60 nuclei per cell) in a sea of mononuclear stroma – Round / ovoid / spindle • Indistinct cell membrane • Mitoses • Appearance of spindle cells-malignant potential
• GCT USUALLY ARE SOLITARY LESIONS;HOWEVER 1%-2% MAY BE SYNCHRONOUSLY OR METACHRONOUSLY MULTICENTRIC
PULMONARY METASTASIS • OVERALL MORTALITY:15% • PATIENT WITH RECURRENT LESIONS OR PRIMARY LESIONS THAT APPEAR AGGRESSIVE RADIOGRAPHICALLY ARE AT HIGHER RISK • MALIGNANT GCT <5% CASES
TREATMENT • Traditionally: – Intralesional curettage / resection & bone graft – Recurrence 35-42% • En Bloc resection – Recurrence ~10% – Multiple complications • Adjuvant
• HISTORICALLY TRT CONSISTED OF SIMPLE CURETTAGE • BUT RECURRENCE RATES > 50% • FOR DEFECTS AFTER RESECTION OR CURETTAGE,EITHER ALLOGRAFT OR BONE CEMENT USED AS FILLING AGENTS EXTENDED CURETTAGE –USE OF A POWER BURR TO ENLARGE THE CAVITY 1-2 CM IN ALL DIRECTIONS IS NOW CONSIDERED STANDARD
Curettings
Adjuvant Tx • PMMA, Liquid N2, Phenol, CO2 laser, Electrocautery – Local extension of margin – Kill residual foci and remaining tumour cell • ASSOCIATED WITH PATHOLOGIC FRACTURES,WOUND HEALING PROBLEMS
BONE GRAFT ADVANTAGE: • RESTORING NORMAL BIOMECHANICS TO JOINT SURFACE • PREVENT FUTURE DEGENERATIVE JOINT DISEASES • RESTORING BONE STOCK
• DISADVANTAGES JOINT MUST BE PROTECTED FOR AN EXTENDED PERIOD OF TIME TO PREVENT A PATHOLOGICAL FRACTURES TUMOUR RECURRENCE IS DIFFICULT TO DISTINGUISH FROM GRAFT RESORPTION
• THE ABOVE DISADVANTAGES OVERCOME BY USE OF BONE CEMENT • PROVIDES IMMEDIATE STABILITY-HENCE QUICKER REHABILITATION • EASIER DETECTION OF RECURRENCE SEEN AS EXPANDING RADIOLUCENCY ADJ TO CEMENT • KILLS RESIDUAL TMR CELLS THRUPOLYMERISATION
Enbloc Resection • Expendable bones – Prox fibula / Distal ulna • Eroded cortex and extended into soft tissue • Recurrence • Pathologic # • Joint involvement
• INITIAL PROCEDURE OF CHOICE AND HERE 2CM OF NORMAL BONE IS ALSO EXCISED • DEFECTS ARE FILLED WITH CANCELLOUS BONE GRAFTS,FREEZE DRIED ALLOGRAFT OR PROSTHESIS
• AROUND THE KNEE,A HEMICONDYLAR OSTEOARTICULAR ALLOGRAFT RECONSTUCTION OR A ROTATING HINGE ENDOPROSTHESIS MAY BE NECESSARY • FOR AGGRESSIVE LESION OF DISTAL RADIUS,PRIMARY RESECTION AND RECONSTRUCTION WITH A PROXIMAL FIBULAR AUTOGRAFT INDICATED
• FOR LESIONS IN EXPENDABLE BONES(DISTAL ULNA OR PROXIMAL FIBULA)PRIMARY RESECTION WITHOUT RECONSTRUCTION INDICATED • FOR INOPERABLE LESIONS IN SPINE OR PELVIS,RADIATION MAY BE USED
EXCISION AND RECONSTRUCTION • FOR GCT AFFECTING LOWER END OF FEMUROR UPPER END OF TIBIA • AFTER EN BLOCK EXCISION RECONSTRUCTION CAN BE DONE BY 1.TURN-O-PLASTY TECHNIQUE 2.ARTHRODESIS 3.ARTHROPLASTY
RECURRENCE OF LESIONS • MOST LOCAL RECURRENCES AND PULMONARY METASTASES OCCUR WITHIN 3YRS OR EVEN UPTO 20 YRS • PATIENT SHOULD HAVE RADIOGRAPH OF THE PRIMARY TUMOUR SITE AND THE CHEST AT 3MONTHS INTERVAL FOR 1YR 6MONTHS INTERVAL FOR NEXT 2 YRS AND ANNUALLY THEREAFTER
• TREATMENT IS SAME AS FOR PRIMARY LESIONS. • AFTER BIOPSY SHOWS THAT TUMOUR IS STILL BENIGN,REPEAT CURETTAGE OR RESECTION IS PERFORMED
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