Soap bubble appearance at lower end of radius, the treatment of choice is –
Correct Answer: Excision and bone grafting
Description: Ans- B Ref- Giant Cell Tumor Patrick O'Donnell Introduction A benign aggressive tumor typically found in the metaphysis of long bones Age & sex more common in females (unlike most bone tumors which show male predominance) ages 30-50 years Location distal femur > proximal tibia > distal radius > sacral ala 50% occur around knee (distal femur or proximal tibia) 10% in sacrum and vertebrae (sacral ala is most common site in axial skeleton) in the mobile spine, it usually occurs in the vertebral body distal radius is third most common location phalanges of the hand is also a very common location may arise in the apophysis (like chondroblastoma) Malignancy primary malignant giant cell tumor metastatic to lung in 2-5% hand lesions have greater chance of metastasis secondary malignant giant cell tumor occurs following radiation or multiple resections of giant cell tumor Symptoms Symptoms pain referable to involved joint Physical exam palpable mass decreased range of motion around affected joint Imaging Radiographs eccentric lytic epiphyseal/metaphyseal lesion that often extends into the distal epiphysis and borders subchondral bone "neo-cortex" is characteristic of benign aggressive lesions, and not unique to GCT Chest radiograph or chest CT pulmonary metatases occurs in 1-6% lung metastases are usually benign (histologically similar to primary bone tumor) Bone scan is very hot MRI shows clear demarcation on T1 image between fatty marrow and tumor Histology Characteristic cells Type I cell mononucleur stromal cell that resembles interstitial fibroblasts this is the neoplastic/tumor cell has features of mesenchymal stem cells Type II cell from monocyte/macrophage family recruited from peripheral blood precursors of giant cells Type III cell numerous giant cells are the hallmark of this lesion nuclei nuclei of giant cell appears same as stromal cells multiple nuclei (up to 50 per cell) similar characteristics as osteoclasts and resorb bone have same enzymes (tartrate resistant acid phosphatase, carbonic anhydrase II, cathepsin K, vacuolar ATPase) secondary ABC degeneration is not uncommon Molecular biology Type II and III cells have IGF-I and IGF-II activity 80% of patients with GCT have telomeric associations (tas) abnormality in half the cells RANK pathway is important denosumab acts on this pathway Treatment Nonoperative radiation alone indications only indicated for inoperable or multiply recurrent lesions outcomes leads to 15% malignant transformation medical management indications medical therapy can be used to augment or replace surgical management depending on the specific clinical scenario medications bisphosphonates osteclast inhibitors which may decrease the size of the defect in giant cell tumors denosumab monoclonal antibody against RANK-ligand recent clinical trials suggest denosumab can decrease the size of the bone defect in giant cell tumor 90% tumor necrosis shows dramatic sclerosis and reconstitution of cortical bone after treatment Operative extensive curettage and reconstruction (with adjuvant treatment) indications lesions amenable to currettage hand lesion treatment is controversial if no cortical breakthrough treat with curettage and cementing if significant cortical breakthrough consider intercalary resection (with free fibular graft) vs. amputation technique challenge of treatment is to remove lesion while preserving joint and providing support to subchondral joint extensive exterioration (removal of a large cortical window over the lesion) is required can fill lesion with bone cement or autograft/allograft bone outcomes 10-30% recurrence with curettage alone verses 3% with adjuvant treatment (phenol, hydrogen peroxide, argon beam, etc) amputation indications hand lesions with cortical breakthrough who are not amendable to intercalary resection Complications Malignant transformation (<1% prevalence) to high grade sarcoma (poor prognosis) latency 9 years for previous radiation treatment 19 years for spontaneous transformation Secondary ABC (<=14%) differentiate from primary ABC because of enhancing soft-tissue component in GCT (not present in primary ABC) Recurrence (15-25%) lucency at bone-cement interface diagnose with CT guided biopsy Pathologic fracture poorer outcome Postoperative infection increased risk with en bloc resection + endoprosthesis Differentials & Groups Brown tumor of hyperparathyroidism can look like GCT on radiographs except it occurs as multiple lesions and associated with serum calcium level abnormalities Chondroblastoma epiphyseal location may also demonstrate ABC formation has extensive surrounding soft tissue and marrow edema may have sclerotic margin and central calcification of chondroid matrix "ring and arcs" pattern Osteosarcoma telangiectatic OS giant cell-rich OS fibroblastic OS Chordoma (mimics GCT sacrum) occurs in midline
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