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CHONDROSARCOMA

Introduction

Chondrosarcoma is the most common malignant bone tumor in the hand. Although heterogeneous, the majority grow slowly and rarely metastasizes. There are two types of chondrosarcoma based on location within the bone: central (conventional) and peripheral. Peripheral chondrosarcomas have a better prognosis and usually affect younger patients. Chondrosarcomas may be categorized by histologic grade, which is an important predictor of tumor clinical behavior and patient prognosis, but it is challenging owing to a spectrum of lesion subtypes. These variants include clear cell (low grade), dedifferentiated (high grade), mesenchymal (highly malignant), and extraskeletal (rare). Chondrosarcomas also may be categorized as primary – arising de novo, or secondary – arising from a pre-existing lesion, such as an osteochondroma.  Chondrosarcoma in the hand is very rare.1,2,3

Related Anatomy

  • Chondrosarcomas are usually located in the metaphyses of long bones, especially in the pelvis,proximal femur, ribs, humerus, tibia, and scapula.
  • About 75% of all chondrosarcomas are conventional, characterized by the outgrowth of the tumor into the intramedullary cavity.
  • Peripheral chondrosarcomas often develop from a pre-existing enchondroma or osteochondroma and are located on the outside of the bone cortex.

Incidence

  • Chondrosarcomas account for ~3.6% of the annual incidence of all primary bone malignancies in the United States, after multiple myeloma and osteogenic sarcoma.
  • Incidence in the hand is 1.5–3.2%.
  • Chondrosarcomas are most common in men in the sixth to eighth decade of life.

Differential Diagnosis1,2

  • Chondroblastoma
  • Enchondroma
  • Enchondromastosis (multiple enchondromas, or Ollier disease)
  • Ostechondroma
  • Osteogenic sarcoma
  • Synovial chondroma 
  • Bone infarct
  • Sarcoidosis
  • Tuberculosis
ICD-10 Codes

  • CHONDROSARCOMA

    Diagnostic Guide Name

    CHONDROSARCOMA

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    CHONDROSARCOMA, MALIGNANT NEOPLASM, BONE    
    - SHORT BONES OF UPPER LIMB (FOREARM, WRIST, HAND, FINGERS, THUMB) C40.12C40.11 
    - OVERLAPPING SITES OF BONE AND ARTICULAR CARTILAGE OF LIMB C40.82C40.81 

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Clinical Photo - Chondrosarcoma Long Proximal Phalanx
  • Chondrosarcoma at arrow after open biopsy
    Chondrosarcoma at arrow after open biopsy
  • Chondrosarcoma before biopsy with disrupted cortex (arrow) and extension into soft tissues of finger. (double arrow)
    Chondrosarcoma before biopsy with disrupted cortex (arrow) and extension into soft tissues of finger. (double arrow)
Symptoms
Mass
Progressive pain (~70% of patients; pain can be absent even in advanced cases)
Limited range of motion (ROM)
Weakness of affected limb
Atrophy of affected limb
Typical History

The typical patient is male, aged 60–79 years. He will often have an enlarging mass with progressive pain that is constant, not relieved with rest, and interfers with sleep. The pain may be accompanied by weakness, atrophy, and limited range opf motion. Family history may or may not be present. 

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
Chondrosarcoma Imaging
  • Chondrosarcoma before biopsy with disrupted cortex (arrow) and extension into soft tissues of finger. (double arrow).
    Chondrosarcoma before biopsy with disrupted cortex (arrow) and extension into soft tissues of finger. (double arrow).
  • Bone scan of chondrosarcoma before biopsy with increased uptake in long finger.(arrow)
    Bone scan of chondrosarcoma before biopsy with increased uptake in long finger.(arrow)
  • Chondrosarcoma after open biopsy with bone cement to temporarily stabilize phalanx.
    Chondrosarcoma after open biopsy with bone cement to temporarily stabilize phalanx.
  • Chondrosarcoma after ray resection for en bloc excision
    Chondrosarcoma after ray resection for en bloc excision
  • MRI after ray resection with no signs of recurrence.
    MRI after ray resection with no signs of recurrence.
Treatment Options
Treatment Goals
  1. Control symptoms by removing mass
  2. Prevent recurrence
  3. Preserve hand function when possible
Conservative

None (surgery is needed)

Operative
  • Conventional: surgical resection with wide margins
  • Intralesional or debulking procedures are associated with a increased recurrence (Grade I lesions in an extremity may be managed with curettage)
  • Clear-cell type: wide local excision
  • Dedifferentiated, extraskeletal (rare), mesenchymal type: surgical resection

Note:  Chondrosarcomas are resisted to chemotherapy and radiation therapy. Radiation has been used for local control of microscopic disease or for palliation.

Treatment Photos and Diagrams
Chondrosarcoma for ray resection for en bloc excision
  • Chondrosarcoma with dorsal excision marked for ray resection.
    Chondrosarcoma with dorsal excision marked for ray resection.
  • Chondrosarcoma with palmar excision marked for ray resection
    Chondrosarcoma with palmar excision marked for ray resection
  • Dissection of dorsum for ray resection
    Dissection of dorsum for ray resection
  • Dissection of palm for ray resection with digital nerve exposed for transection (arrow)
    Dissection of palm for ray resection with digital nerve exposed for transection (arrow)
  • Drill holes in the metacarpal proximally prior to osteotomy (arrow)
    Drill holes in the metacarpal proximally prior to osteotomy (arrow)
  • Osteotomy of metacarpal shaft proximally
    Osteotomy of metacarpal shaft proximally
  • Ray amputated except for small amount of interosseous muscle and intrametacarpal ligament. (arrow)
    Ray amputated except for small amount of interosseous muscle and intrametacarpal ligament. (arrow)
  • Amputated ray - Note tumor not exposed
    Amputated ray - Note tumor not exposed
  • Hand after ray amputation before closure
    Hand after ray amputation before closure
  • Hand after ray amputation after closure
    Hand after ray amputation after closure
Complications

SURGICAL COMPLICATIONS

  • Recurrence
  • Infection
  • Decreased range of motion, pinch and grip
Outcomes
  • Low-grade chondrosarcoma of the hand treated with amputation rarely recur.
  • In general, poorer outcomes in the hand are associated with inadequate resection or delayed surgical intervention.
  • Conventional: depends on grade and location of lesion; prognosis for patients with low-grade, central chondrosarcoma is good, with a 5- and 10-year survival rate of >80%.
  • Clear-cell: prognosis is better than for patients with other types
  • Mesenchymal: 5-year survival rate is 60%; 10-year survival rate is 25%
  • Dedifferentiated chondrosarcoma is quickly lethal; the tumor metastasizes in 90% of patients, and the 5-year survival rate is 0–18%.
Key Educational Points
  • Chondrosarcoma of the hand can appear histologically aggressive but usually have a benign clinic course.
  • The age at diagnosis often does not coincide with the time when symptoms first appear.
  • Early research into molecular therapeutic targets focuses on the role of basic fibroblast growth factor (BFGF) in lymphangiogenesis and chondrosarcoma metastasis.4
  • Biopsy
    • Controversial due to high potential for sampling error; chondrosarcomas are extremely heterogeneous
    • Open biopsy is preferred when the diagnosis unclear, to minimize sampling error.
    • Closed (core-needle) biopsy with real-time imaging may be ideal for confirmation.
  • Differentiating benign, active lesions from low-grade, malignant lesions is difficult, and clinical and radiographic evidence must accompany the histologic data.
  • Metastatic disease and death secondary to chondrosarcoma of the hand is extremely rare.
  • Treatment of low-grade hand chondrosarcoma is controversial. Lesions contained within a bone have been successfully treated by curettage and bone grafting but recurrence is higher than with wide resections (amputations).  However amputations decrease hand function.
  • Malignant transformation of enchondroma and osteochondroma to chondrosarcoma occur with an increased frequency in Ollier disease.1,2,3
References

Cited Articles

  1. Muramatsu K, Kawakami Y, Tani Y, Taguchi T.  Malignant transformation of multiple enchondromas in the hand: case report.  J Hand Surg 2011: 36A:304-307. PMID: 21186085
  2. Palmieri TJ. Chondrosarcoma of the hand. J Hand Surg Am 1984;9:332-8. PMID: 67258891.
  3. Pino JG, Calderon SAL, Chebib I, Jupiter JB.  Intralesional versus wide resection of low-grade chondroma of the hand.  J Hand Surg 2016;41(4):541-549. PMID: 26803570
  4. Tzeng HE, Chang AC, Tsai CH, et al. Basic fibroblast growth factor promotes VEGF-C-dependent lymphangiogenesis via inhibition of miR-381 in human chondrosarcoma cells. Oncotarget 2016;7(25):38566-78.PMID: 27229532

Reviews

  1. American Association of Osteopathic Surgeons. Chondrosarcoma. 2007. Accessed November 1, 2016 at http://orthoinfo.aaos.org/topic.cfm?topic=A00395
  2. Gelderblom H, Hogendoorn PC, Dijkstra SD, et al. The clinical approach towards chondrosarcoma. Oncologist2008;13:320-9. PMID: 18378543
  3. Nota SP, Braun Y, Schwab JH, et al. The identification of prognostic factors and survival statistics of conventional central chondrosarcoma. Sarcoma 2015;2015:623746. PMID: 26633939

Case Report

  1. Muramatsu K, Kawakami Y, Tani Y, Taguchi T. Malignant transformation of multiple enchondromas in the hand: case report. J Hand Surg Am 2011;36:304–7. PMID: 21186085

Classics

  1. Lichtenstein L, Jaffe HL. Chondrosarcoma of bone. Am J Pathol 1943;19(4):553-89. PMID: 19970709
  2. Morton JJ, Mider GB. Chondrosarcoma. Ann Surg 1947;126(6):895-929. PMID: 17859044
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