Spot diagnosis? Identify the bone tumor


 Ans:


Discussion: 
    - a common benign but locally aggressive lesion of unknown etiology; 
    - occurs chiefly in men between 20-50 yrs (after epiphyseal closure); 
    - tumor is expansile lytic lesion that involves the epiphysis & metaphysis; 
    - tumor may enlarge to occupy most of epiphysis & adjacent metaphysis; 
    - tumor may erode & penetrate subchondral bone, articular cartilage, & cruciate ligaments; 
    - location: 
         - epiphysis of distal femur, proximal tibia, & distal radius; 
         - other sites: fibula, sacrum, proximal humerus, & distal tibia; 
         - can occur in bones of pelvis, particularly ilium near SI joint and sacrum; 
         - spine: 
              - usually located in vertebral body; 
              - radiolucent lesion in vertebral body of a young patient is likely to be GCT;  - staging: 
         - stage I: 
              - benign latent giant cell tumors; 
              - no local agressive activity; 
         - stage II: 
              - benign active GCT; 
              - imaging studies demonstrate alteration of the cortical bone structure; 
         - stage III: 
              - locally aggressive tumors; 
              - imaging studies demonstrate a lytic lesion surrounding medullary and cortical bone; 
              - there may be indication of tumor penetration through the cortex into the soft tissues; 
    - prognosis: 
         - tumors w/ malignant stroma tend to behave in malignant fashion; 
         - lesions that appear benign may metastasize; 
         - 5% of pts will have pulmonary metastases (2% risk of benign pulmonary metastasis in all cases and 6% risk in recurrent cases); 
         - tumor is considered benign if pulmonary lesions are histologically benign; 
                 - pulmonary lesions may be cured w/ surgical resection; 
         - GCT involving the distal radius may be more aggressive than in other locations; 
    - differential dx: 
         - non ossifying fibroma; 
         - aneurysmal bone cyst; 
         - histoplasma
                 - Histoplasma Osteomyelitis Simulating Giant-Cell Tumor of the Distal Part of the Radius: A Case Report.

         - hyperparathyroidism
                 - may produce brown tumors that are radiographically & histologically similar to giant cell tumor of bone, w/ the exception 
                          that brown tumors tend to be diaphyseal in location; 
                 - unlike brown tumors, serum Ca is normal in GCT; 


- Clinical Manifestations: 
    - pts report deep, persistent intraosseous pain that mimics an internal derangement of the knee. 
    - pathologic frx or reactive knee effusioin is initial symptom in about 1/3 of pts; 


- Radiographic Features:     
     - well-defined lytic lesion that involves the metaphysis and epiphysis (typical of a giant cell tumor);

- CT Scan: 
    - helps determine is extact amount of cortical destruction and helps determine the optimal location of the cortical window; 

- Bone Scans: 
    - bone scans may show decreased radioisotope uptake in the center of lesion (doughnut sign); (also found w/ ABC); 

- MRI: 
    - help determine determine extent of tumor destruction; 
    - may be indicated when the tumor has eroded thru the cortex and allows determination of whether concomitant neurovascular 
              structures are involved; 
    - may help evaluate subchondral penetration; 

         


- Histologic Studies; 


- Treatment: 
    - due to proximity to articlar cartilage, excision of GCT of bone is difficult; 
    - because intralesional excision of GCT tends to leave tumor cells behind, past attempts of excision had been asociatted w/ high 
           recurrance rate (40%); 
           - GCT involving the distal radius may be more agressive that GCT in other locations; 
    - prior pathologic fracture should be allowed to heal before surgery is attempted; 
    - ref: Giant Cell Tumor With Pathologic Fracture: Should We Curette or Resect?


- Stage 1 or 2 Lesions: 
    - intra-lesional excision is treatment of choice; 
          - excision is facilitated by making a large cortical window; 
          - window must be large enough to allow complete access to every corner of the intra-osseous lesion; 
          - high speed burr is used to complete the excision, with care taken to remove 5 mm of normal bone surrounding the lesion; 
          - at completion of procedure (which usually involves placement of cement or bone graft in the cavity), window is replaced w/ 
                    bone graft or w/ bone graft substitute; 
    - note that in areas such as the distal ulna or fibula, en bloc excision is preferred over currettage because there is minimal functional 
          consequences of en bloc exision;       
    - motorized burr and cautery are used to complete and excision the excision; 
          - when motorized burr is used to debride the subchondral bone, the joint cartilage should be irrigated w/ chilled saline; 
          - Blackley, et al (1999), the authors followed 59 patients who underwent curettage and bone grafting of GCT lesions; 
                - 12% had a local recurrence (and all but one of these patients responded to revision currettage and/or soft tissue resection); 
                - low recurrence rate was due to meticulous debridement of GCT tissue which included use of a high speed burr; 
                - ref: Treatment of giant-cell tumors of long bones with curettage and bone-grafting.
    - adjunctive measures
          - use of phenol, polymethacrylate, and liquid nitrogen; 
                - phenol can be extremely toxic, and if used at all, its concentration should be limited to 5%; 
          - these work by increasing the zone of necrosis at periphery of excision; 
          - cement: 
                - heat generated from the polymerization reaction may kill tumor cells 0.5 mm in cortical bone and 2 mm in cancellous bone; 
                - as reported by O'Donnell, et al (1994) the use of cement did not appear to improve the results as compared to historic 
                         controls with a 25% recurrence rate w/ long term follow up; 
                - barium impregnated cement allows for accurant determination of local recurrance; 
                - cement provides mechanical support following curretage and does not appear to affect cartilage when placed in the 
                           subchondral region; 
                - when cement is applied beneath subchondral cartilage, joint should be irrigatted w/ chilled saline, inorder
                           to protect  cartilage; 
                 - Recurrence of giant-cell tumors of the long bones after curettage and packing with cement.
          - liquid nitrogren: 
                     - may cause excessive tissue necrosis, extending into normal tissue; 
          - bone grafting:
                     - Supplemental Bone Grafting in Giant Cell Tumor of the Extremity Reduces Nononcologic Complications


- Recurrent or Stage 3 Lesions: 
    - this category includes frx w/ major pathologic frx, articular or cortical penetration by the tumor; 
    - en bloc excision w/ wide margin along w/ appropriate reconstruction; 
    - en bloc excision involves removal of one side the joint, necessitating a major limb reconstructive procedure (often w/ allografts); 
    - chemotherapy:
           - Denosumab, a Potential Alternative to the Surgical Treatment of Distal Radius Giant Cell Tumor of Bone: Case Report 
    - radiation therapy 
           - traditionally XRT is avoided because of the possibility of malignant degeneration of the tumor; 
           - in the report by Chakravarti, et al JBJS 1999, 20 patients underwent a single course of megavoltage radiation (40-70 gray 
                  administered at 1.8 to 2.0 gray per fraction with an average total duration of treatment of 5-7 weeks) 
                  - after a median duration of follow-up of 9.3 years, the tumor had not progressed in seventeen of the twenty patients; 
                  - no radiation-induced tumors were observed in our series; 
                  - authors note that the results of XRT were comparable to published efficacy of surgical resection; 
           - references: 
                  - Megavoltage Radiation Therapy for Axial and Inoperable Giant-Cell Tumor of Bone.  
    - pulmonary metastasis
           - 5% of pts will have pulmonary metastases; 
           - tumor is considered benign if pulmonary lesions are histologically benign; 
           - pulmonary lesions may be cured w/ surgical resection; 
           - references: 

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