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Duke Orthopaedics
presents
Wheeless' Textbook of Orthopaedics

Chondrosarcoma


- See: Bone Tumor Menu

- Discussion:
    - malignant cartilaginous tumor of bone (3rd most common malignant bone tumor / comprises 20% of malignant bone lesions);
    - it is less common and less aggressive than osteosarcoma;
    - staging:
            - histologic grading is best predictor of stage;
            - most often presents as a low grade - intracompartmental lesion;
            - grade-1: almost never metastasize and are difficult to differentiate from benign enchondroma
                    - on serial radiographs at monthly intervals grade 1 tumors will continue to grow where as enchondromas never grow;
           - they are slow to metastasize and may grow to immense proportions;
            - tumor tends to destroy bone and to extend into the soft tissues;
            - tumor may invade blood vessels and produce long intravascular tumor bungs  that may extend into heart and pulmonary arteries;
            - long-term survival rate of patients w/ treated chondrosarcoma is between 50-75%.
            - von Hippel-Lindau tumor suppressor gene (VHL), located in chromosome 3p25 may be involved in chondrosarcoma formation;
    - primary form occurs most often in men between 50-60 yrs of age (where as osteosarcoma occurs between 20-30 yrs of age);
    - location:
            - pelvis, proximal femur, & shoulder girdle (where as osteosarcoma occurs most often around the knee;
            - when chondrosarcoma occurs in the pelvis, it is often large, high grade, diagnosed late, and has a poor prognosis;
            - in report by Pring ME, et al. (2001), 64 patients with localized pelvic chondrosarcoma that had been surgically treated between 1975-96 were reviewed;
                   - there were 41 male and 23 female patients who had a mean age of 47 years;
                   - 33 of the 64 patients were first seen with grade-1 chondrosarcoma;
                   - 23, with grade-2; one, with grade-3; and seven, with grade-4 (dedifferentiated chondrosarcoma);
                   - 13 patients had a hemipelvectomy to achieve local tumor control, whereas 51 patients underwent a limb-salvage procedure;
                   - 12 (19%) had local recurrence, and eleven (17%) had distant metastases;
                   - at follow-up, 44 (69%) were alive w/o evidence of dz, 13 (20%) had died of dz, 6 (9%) had died of unrelated causes, and 1 (2%) was alive w/ dz;
                   - less than a wide surgical margin correlated with local recurrence (p = 0.014);
                   - high-grade tumors correlated with poor overall survival (p < 0.001);
                   - all patients who had a limb-salvage procedure were able to walk at the time of the final follow-up;
                   - ref: Chondrosarcoma of the Pelvis. A Review of Sixty-four Cases.
    - secondary chondrosarcoma:
            - occurs in about 25% of cases;
            - it is a malignant transformation from preexisting enchondroma or osteochondroma;


- Variants of Chondrosarcoma: (based on histology & location);
    - dedifferentiated (high grade)
         - rare type of high grade chondrosarcoma which arises from a low grade chondrosarcoma;
         - a high-grade spindle-cell sarcoma coexists with a lower-grade chondroid tumour;
         - histologically there will be areas c/w MFH, osteosarcoma, or fibrosarcoma;
         - high risk for metatastasis, only 5% of patients will survive more than 5 years;
         - references:
                - Dedifferentiated chondrosarcoma
                - Dedifferentiated chondrosarcoma. A report of the clinicopathological features and treatment of seventy-eight cases.
                - Experience in the treatment of dedifferentiated chondrosarcoma.   
    - extraosseous chondrosarcoma:
               - Extra-osseous chondrosarcoma. Report of five cases and review of the literature.
    - clear cell (intermediate grade)
         - rare, slow growing, locally recurrent tumor easily confused w/ chondroblastoma but malignant;
               - tumor is especially rare in a child or adolescent;
               - may invade epiphysis;
               - most common in proximal femur (over 50%) followed by proximal humerus;
         - microscopically, sheets of cartilaginous cells in a lobular arrangement are mixed with scattered giant cells;
         - this radiolucent lesion is often misdiagnosed and undertreated;
         - references:
               - Chondrosarcoma: clear-cell variant. A report of sixteen cases.
               - Year Book: Clear Cell Chondrosarcoma of Bone: Observations in 47 Cases.
               - Radiologic case study. Clear cell chondrosarcoma.
               - Clear cell chondrosarcoma
               - Clear cell chondrosarcoma of bone: long time follow-up of 18 cases 
               - Year Book: Clear-Cell Chondrosarcoma: A Report of Ten Cases and Review of the Literature.
    - atypical enchondroma (juxtacortical chondroma)
         - histologic exam of a low grade chondrosarcoma may show nodular cartilage tissue with mostly isomorphic tumor cells;
               - tissue samples show cellularity than is seen w/ enchondroma;
               - differentiating between this malignant tumor growth and an enchondroma can be extremely difficult;
               - an aysmptomatic - well circumscribed calcified lesion which has not changed in size is most consistent w/ an enchondroma;
               - more peripherally located lesions are also more likely to represent an enchondroma;
         - references:
               - Low risk of recurrence of enchondroma and low grade chondrosarcoma in extremities. 80 patients followed for 2-25 years.


- Clinical Findings:
    - persistent, dull, aching pain;
    - found in an older age group, peaking at age 45 years;
    - majority of chondrosarcomas occur about the hip and pelvis.

- Histology:       
    - grade 1:
          - may be identical to benign enchondroma;
          - where as a low grade malignant appearing cartilagenous tumor of extremities may actually be an enchondroma, a centrally occurring malignant appearing
                  enchondroma in an adult is more likely represents a chondrosarcoma;
    - grade 2:- increased cellularity but rarely have mitotic figures and are more locally aggressive, with a 15% to 20% rate of metastasis;
    - grade 3: (dedifferentiated and mesenchymal types) mitotic figures with increased cellularity, leading to a 70% rate of metastasis;

- Radiographic Studies:
    - subtle, radiolucent, permeative lesion;
    - lytic lesions that often erode the cortex with or without cortical thickening
    - may have hazy or speckled calcifications w/ either a diffuse "salt & pepper" pattern or a more discrete "pop corn" pattern;
            - pattern of calcification is usually diagnostic of  cartilaginous tumor;
 
          

- Treatment:
    - tumor does not respond to x-ray therapy nor to currently available antineoplastic drugs;
    - low grade tumors:
         - rarely metastasize;
         - rarely recurs after wide limb salvaging excision;
         - the involved bone is resected along w/ a small cuff of surrounding muscle;
    - high grade tumors
         - have higher rate of recurrence after limb salvage (requires amputation)
         - are prone to pulmonary metastases.



A New Histologic Approach to the Differentiation of Enchondroma and Chondrosarcoma of the Bones: A Clinicopathologic Analysis of 51 Cases.

Amputations and Artificial Limbs--General Orthopaedics: Tumors: Periosteal Chondroma: A Report of Ten Cases and Review of the Literature.

S-100 protein in human cartilage lesions.

Replantation of an autoclaved autogenous segment of bone for treatment of chondrosarcoma. Long-term follow up.

Chondrosarcoma in Maffucci's syndrome.

Chondrosarcoma of the spine

The biology of human chondrosarcoma. I. Description of the cases, grading, and biochemical analyses.

Partial or complete resection of the hemipelvis. An alternative to hindquarter amputation for periacetabular chondrosarcoma of the pelvis.

Extra-articular synovial chondromatosis.

Association between ratio of matrix metalloproteinase-1 to tissue inhibitor of metalloproteinase-1 and local recurrance, metastasis, and survival in human chondrosarcoma.

Chondrosarcoma of the Hand: is a Wide Surgical Resection Necessary? 

Recent advances in the basic science of chondrosarcoma

Surgical treatment and outcome of conventional pelvic chondrosarcoma



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Thursday, June 7, 2012 6:48 pm