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 Epiphyseal lesion Treatment is USUALLY currettage and bone grafting(1) Giant Cell Tumor • • Chondroblastoma • • Aneurysmal Bone cyst • Osteoblastoma • Chondromyoid fibroma (CMF) • ASSUMPTIONS: (1) assuming no impending fracture IBank Location Xray Xray CT B. Scan MRI MRI Histo(1) Case A knee -recurrence Case B knee Case C distal radius Case D hand Case B knee Case B ankle Case A humerus (1) - histology does not always correspond to clinical case
QUESTIONS 1 of 37 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ06.73) A 41-year-old male presents with 6 months of wrist pain and swelling. He is otherwise healthy and denies trauma. Clincal photograph, radiographs, MRI, and histology are shown in Figures A through E. What is the most likely diagnosis? Review Topic QID: 184 FIGURES: A B C D E 1 Aneurysmal bone cyst 4% (42/1078) 2 Giant cell tumor 89% (958/1078) 3 Telangectatic osteosarcoma 4% (46/1078) 4 High-grade osteosarcoma 3% (27/1078) 5 Osteomyelitis 0% (2/1078) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 2 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ07.197) A 40-year-old female presents with dull pain in her knee that has been increasing in severity over the past 6 months. Figures A through D show representative radiographs, MRI, and biopsy section. What is the most appropriate treatment? Review Topic QID: 858 FIGURES: A B C D 1 Observation 1% (11/1012) 2 Bone marrow biopsy 2% (20/1012) 3 Intra-lesional curettage with local adjuvant therapy 85% (863/1012) 4 Neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy 8% (77/1012) 5 Neoadjuvant radiotherapy and surgical excision 3% (34/1012) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (OBQ06.114) A 21-year-old man presents with a lytic lesion in his distal femur. A chest radiograph reveals a lung nodule. Which of the following tumors most likely to metastasize to lung ? Review Topic QID: 300 1 Non-ossifying fibroma 1% (16/2026) 2 Giant cell tumor 93% (1894/2026) 3 Aneurysmal bone cyst 1% (22/2026) 4 Osteoid Osteoma 1% (28/2026) 5 Hemangioma 3% (60/2026) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (OBQ09.50) Which of the following radiographs demonstrates a tumor that would be best treated by curettage with a power burr and packing with cement? Review Topic QID: 2863 FIGURES: A B C D E 1 Figure A 5% (87/1829) 2 Figure B 2% (45/1829) 3 Figure C 1% (12/1829) 4 Figure D 0% (5/1829) 5 Figure E 91% (1673/1829) ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ07.186) Giant cell tumors of bone can be locally aggressive and result in significant bone destruction. Which of the following is responsible for this type of bone destruction? Review Topic QID: 847 1 Multinuclear giant cells 14% (156/1121) 2 Osteocytic stromal cells 3% (34/1121) 3 Tumor cell activation of osteoclasts 76% (852/1121) 4 Tumor cell inactivation of osteoblasts 1% (13/1121) 5 Osteoprotegrin activation of osteoclasts 5% (61/1121) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (OBQ10.204) A 29-year-old female complains of increasing severity back pain for the past 4 months. Radiograph, CT scan, T1 and T2 MRI, and biopsy specimen are shown in Figures A through E. What is the most likely diagnosis? Review Topic QID: 3297 FIGURES: A B C D E 1 Osteosarcoma 4% (110/2493) 2 Ewing's sarcoma 2% (41/2493) 3 Lymphoma 2% (38/2493) 4 Giant cell tumor 58% (1458/2493) 5 Chordoma 34% (838/2493) ML 4 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (OBQ05.203) All of the following locations are common sites for giant cell tumor of bone to occur EXCEPT? Review Topic QID: 1089 1 Posterior elements of the spine 67% (437/649) 2 Distal femur 2% (12/649) 3 Sacrum 20% (132/649) 4 Distal radius 6% (42/649) 5 Proximal tibia 4% (23/649) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 1 (OBQ05.240) Giant cell tumors of bone can occur in many different areas throughout the body. All of the following are common locations for giant cell tumors of bone EXCEPT? Review Topic QID: 1126 1 Sacrum 14% (62/440) 2 Distal radius 2% (11/440) 3 Tibial diaphysis 69% (305/440) 4 Distal femur 3% (12/440) 5 Phalanges of the hand 11% (47/440) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (OBQ08.252) A 33-year-old female reports lateral knee pain and slight fullness. She underwent a bone scan which showed increased activity isolated to the proximal fibula. Radiographs and histology are shown in Figures A and B. What is the most likely diagnosis? Review Topic QID: 638 FIGURES: A B 1 Parosteal osteosarcoma 1% (19/1548) 2 Eosinophilic granuloma 13% (203/1548) 3 Chondroblastoma 7% (111/1548) 4 Giant cell tumor 78% (1203/1548) 5 Multiple myeloma 0% (7/1548) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 4 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
The Role of Denosumab in the Modern Treatment of Giant Cell Tumor of Bone Pathology - Pathology Rounds this a new review and update on the role of denusumab in GCT treatment. It...
Recurrent Lytic Lesion of the Proximal Femur (C2779) Pathology - Giant Cell Tumor HPI - 36 year old female previously presented with a history of left hip pain 3 years ago with a lytic lesion in the left intertrochantric area. She was operated on with a fixed angle plate, bone graft and biopsy. 8 months ago removal of implant was done and attached is X-Ray post removal and result of histopathology. 1 month ago, patient presented with progressive left hip pain and X-Ray is shown with a recurrent lytic lesion with extension to the femoral neck. What is the best option of treatment? 3/10/2017 100 2 6 Recurrent giant cell tumor at distal femur with pathological fracture (C2749) Pathology - Giant Cell Tumor HPI - Patient presents to the emergency room with acute left knee pain after low energy trauma. Would you perform a needle biopsy in this patient prior to surgical intervention to confirm the diagnosis? 1/12/2017 4916 16 8 LYTIC LESION PROXIMAL PHALANX THUMB (C2268) Pathology - Giant Cell Tumor HPI - Pain on loading right thumb fort 02 ys duration. Gradually worsening, aggravated for 02 months Likely diagnosis 6/23/2015 180 0 2 See More See Less