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OSTEOSARCOMA

Introduction

Osteosarcoma, also called osteogenic sarcoma, is a extremely rare type of cancer that can develop in the metacarpals and phalanges of the hand. Primary bone tumors are exceptionally unusual in the hand, but among these hand lesions, osteosarcoma is the most common. It also is one of the most common malignancies after cancer treatment in children. Osteosarcoma of the hand usually presents with swelling and pain. Osteosarcoma of the hand has no preference for right or left hand; no preference for male or female and it occurs in all age groups. The metacarpals and phalanges are equally involved.8 Osteosarcoma is highly invasive, and 85% of patients with osteosarcoma have metastases. Teenagers are the primary affected age group among typical patients, which include those aged 10–30 years. But osteosarcoma can occur at any age; about 10% occurs in people >60 years old. The diagnosis is often delayed in youth, because symptoms of pain and swelling are common in active people. Recent reports have highlighted radiation and extraskeletal osteosarcomas of the hand. These very rare lesions typically occur in older individuals.1-8

Pathophysiology

  • Most osteosarcomas are not caused by inherited mutations, but rather are the result of genetic changes during life.
  • Radiation therapy can damage DNA, but other changes are not understood.
  • There are no known lifestyle or environmental causes of osteosarcoma.
  • Histopathologic grading
    • Low grade: slowest-growing; look like normal bone and have few dividing cells

Subtypes:

  • Parosteal
  • Intramedullary or intraosseous well differentiated and are the  most uncommon
  • Periosteal are high grade: fastest-growing lesions; do not look like normal bone, and dividing cells proliferate
  • Osteoblastic*
  • Chondroblastic*
  • Fibroblastic*
  • Mixed
  • Small cell
  • Telangiectatic
  • High-grade surface (juxtacortical high grade)
  • Pagetoid
  • Extra-skeletal
  • Post-radiation

*Most common among high-grade osteosarcomas

Related Anatomy

  • Most osteosarcomas develop around the knee (50%); the proximal humerus is the next most common site.
  • Malignant bone tumors are rare in the hand but osterosarcoma is the most common bone cancer that occurs in the hand
  • Osteosarcoma can develop in any bone, including the pelvis/hips, shoulder, and jaw.
  • If the cancer recurs, it is usually in the lungs.

Incidence and Related Conditions

  • Approximately 800 new cases of osteosarcoma are diagnosed annually in the United States.
  • The overall incidence rate is 4% in the 0- to 14-year age range and 5% in the 0- to 19-year age range, per million persons.
  • Among childhood cancers, osteosarcoma is ranked eighth in general incidence (2.4%).
  • Patients with the following are at increased risk of developing osteosarcoma: bone disorders including Paget disease; inherited cancer syndromes including retinoblastoma, Li-Fraumeni syndrome, and Rothmund-Thomson syndrome.

Differential Diagnosis

  • Cancerous bone tumors
    • Chondrosarcoma
    • Chordoma
    • Fibrosarcoma
    • Malignant fibrous histiocytoma
    • Malignant giant cell tumor of bone
  • Benign bone tumors
    • Chondromas
    • Osteochondromas
    • Osteomas
ICD-10 Codes

  • OSTEOSARCOMA

    Diagnostic Guide Name

    OSTEOSARCOMA

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

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    OSTEOSARCOMA, MALIGNANT, SHORT BONES, UPPER LIMBS C40.12C40.11 

    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
Osteosarcoma of the Left Hand
  • Osteosarcoma of the left fifth metacarpal with dorsal and ulnar swelling (arrows)
    Osteosarcoma of the left fifth metacarpal with dorsal and ulnar swelling (arrows)
  • Osteosarcoma of the left fifth metacarpal with ulnar swelling (arrow)
    Osteosarcoma of the left fifth metacarpal with ulnar swelling (arrow)
Symptoms
Bone pain, most common symptom
Pathologic fracture - (Bone weaken secondary to the tumor and fractures from minimal trauma)
Erythema, swelling, lump or mass, depending on tumor location
Typical History

The typical patient is most likely to be a male teenager, who will present with complaints of pain and swelling, and a nodule or mass (possibly non-tender) will be evident. Because the diagnosis of osteosarcoma is often delayed due to expected, occasional pain and swelling in this active age group, the physician should maintain a high degree of suspicion until the possibility of bone cancer is ruled out. There may be a history of previous treatment with radiation therapy for another cancer. 

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
Osteosarcoma of the Left Hand
  • X-ray of the left fifth metacarpal osteosarcoma
    X-ray of the left fifth metacarpal osteosarcoma
Treatment Options
Operative
  • Surgery
    • For low-grade osteosarcoma, wide excision alone
    • For high-grade osteosarcoma, wide excision plus high-dose combination chemotherapy
    • Amputation may be appropriate for extensive hand tumor or recurrence
  • External beam radiotherapy:
    • Used when complete resection is not possible
Complications
  • General:
    • Metastatic lesions
    • Heart or lung problems (due to certain chemo drugs or radiation therapy)
    • Hearing loss (due to certain chemo drugs)
    • Slowed or decreased growth and development
    • Changes in sexual development and fertility
    • Learning problems in younger children
    • Development of secondary cancers
  • Chemotherapy: severe side effects
  • Resection:  reactions to anesthesia, excess bleeding, blood clots, and infections
  • Limb salvage surgery: due to complexity, can lead to more complications than amputation
    • Infections
    • Loose or broken grafts or rods (implant failure)
    • Nonunion/fracture of arthrodesis reconstruction
    • Need for more surgery because of developmental growth
    • Intensive physical therapy critical to success
    • Eventual amputation
Outcomes
  • Localized osteosarcoma: 5-year survival rate is 60–80%
  • Metastatic osteosarcoma: 5-year survival rate is 15–30%  
Key Educational Points
  • Survival rates of patients with osteosarcoma are not adversely affected by limb salvage, instead of amputation.
  • Because these cancers are so rare, the available data that can be used to assist the hand surgeon is limited and planning treatment for these patients with malignant bone tumors of the hand is difficult.
  • No data exist to help determine the optimal surgical treatment for post-radiation osteosarcoma in the hand.
  • The risk of developing post-radiation sarcoma is dose dependent. 
  • Hand osteosarcoma seems to have a slightly better prognosis than osteosarcoma in other anatomic locations.8
References

New Articles

  1. Healy C, Kahn LB, Kenan S. Subcutaneous extraskeletal osteosarcoma of the forearm: a case report and review of the literature. Skeletal Radiol 2016;45(9):1307-11. PMID: 27357312
  2. Puhaindran ME, Hamilton K, Schlumbohm S, et al. Radiation-induced osteosarcoma of the hand: case report. J Hand Surg Am 2014;39(6):1151-54. PMID: 24799140
  3. Puhaindra ME, Hamilton K, Schlumbolm S, Rich M, Mitchell D, Steensma M.  Radiation - induced osteosarcoma of the hand: case report.  J Hand Surg Am 2014; 39(6): 1151-1154.
  4. Dunbar RM, Sumarriva GE, Duncan SFM, Reith JD, Meyer MS.  Extraskeletal osteosarcoma of the hand.  J Hand Surg Am 2018: 43(5): 490.ei-e4.
  5. Mohler CG, Jensen C, Steyers CM, Landas SK, Renfrew DL. Osteosarcoma of the hand: case report and review of the literature. J and Surg Am 1994; 19A: 287-289.
  6. Mulligan ME, Lewis DR, Resnick CS, Kumar D, Levine AM.  Small cell osteosarcoma of the ulna: a case report and review of the literature. J Hand Surg Am 1999; 24A(2): 417-420
  7. Hatano H, Morita T, Kobayashi H, Iwabachi Y.  Osteosarcoma of the distal sradius treated with segmental forearm resection, hand replanttion, and subsequent limb lengthening: case report.  J Hand Surg Am 2014; 39(6): 1155-1159.
  8. Carroll RE.  Osteogenic sarcoma in the hand.  JBJS 1957; 39A(2): 325-331.

Reviews

  1. Casey DL, van de Rijn M, Riley G, et al. Extraskeletal osteosarcoma of the hand: the role of marginal excision and adjuvant radiation therapy. Hand (NY) 2015;10(4):602-6. PMID: 26568711
  2. Salunkee AA, Chen Y, Tan JH, et al. Does a pathological fracture affect the prognosis in patients with osteosarcoma of the extremities?: a systematic review and meta-analysis. Bone Joint J 2014;96-B(10):1396-403. PMID: 25274928

Classics

  1. Fleegler EJ, Marks KE, Sebek BA, et al. Osteosarcoma of the hand. Hand 1980;12(3):316-22. PMID: 6934158
  2. Drompp BW. Bilateral osteosarcoma in the phalanges of the hand. W solitary case report. J Bone Joint Surg Am 1961;43-A:199-204. PMID: 13724456
  3. Carroll RE.  Osteogenic sarcoma in the hand.  JBJS 1957; 39A(2): 325-331.
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