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Malignant bone tumors

Last updated: November 20, 2020

Summarytoggle arrow icon

Malignant bone tumors can be classified as primary (arising from abnormal bone or cartilage cells) or secondary (bone metastases of other tumors). The most common primary bone tumors are osteosarcomas, Ewing sarcomas, and chondrosarcomas. These tumors differ with regard to primary localization, radiographic characteristics, and the patient age at which they usually develop. Progressive, localized pain that worsens at night or with physical activity is common and usually accompanied by swelling. In Ewing sarcoma and osteosarcoma, symptoms often first manifest after an injury. Diagnosis is confirmed by imaging and biopsy. Depending on the type and stage of the tumor, chemotherapy, radiation therapy, and/or definitive surgical resection may be required. Bone tumors may also occur secondary to metastases of other primary cancers. The metastases are commonly detected in the spine and pelvis and usually arise from the lung, breast, and prostate cancer. Treatment focuses on the underlying malignancy and additional management of pain and prevention of fractures related to the metastases.

Description

Clinical features

  • Frequently first manifests with localized pain (progressive, worsens at night), hyperthermia, and swelling after trauma to the bone (tissue mass that is tender to palpation and accompanied by erythema)
  • B symptoms are common.

Diagnostics

Ew, did you feed on 22 onions?”: Ewing sarcoma, femur region, chromosome 22, onion skin appearance.

Treatment [4]

Prognosis

  • Extremely aggressive, early metastases
  • Usually responsive to chemotherapy
  • Five-year survival rate of ∼ 80% for localized disease [5]

References:[6]

Description

Clinical features

Diagnostics [7]

Remember to wear your SOCK (Sunburst, Osteosarcoma, Codman, Knee region).

Treatment

Osteosarcomas are usually resistant to radiation therapy.

Prognosis

  • Aggressive course
  • Primary osteosarcoma: five-year survival rate of ∼ 70% (usually responsive to treatment)
  • Secondary osteosarcoma: poor prognosis (less responsive to treatment)

References:[8]

Description

Clinical features

Diagnostics [10]

Treatment

Prognosis [12]

  • Five-year survival rate of 50–85% (depending on the histological grading)
  • Late recurrences are possible.
  • Regular follow-ups for 10 years are required.

References:[14]

Imaging

General approach

Radiographic signs of malignant bone tumors [16]

  • Margins of the lesion: The more poorly defined the margins of the lesion are, the more rapid is the tumor growth. [15]
    • Type I: geographic
    • Type II: moth-eaten appearance
    • Type III: permeative
  • Periosteal reactions: reactive periosteal bone synthesis as a result of bone destruction by a malignant process

Types of periosteal reactions

  • Continuous periosteal reactions
    • Solid periosteal reaction
      • Increased formation of new bone, with or without cortical destruction
      • Indicates slow tumor growth
    • Lamellated periosteal reaction
    • Spiculated periosteal reaction
      • Spicules (new bone formations) that grow along Sharpey fibers (collagen fibers that anchor the periosteum to the compact bone)
      • Indicates more aggressive tumor growth compared to the solid and lamellated types
      • Hair-on-end appearance: spicules that extend perpendicular to the bone surface
      • Sunburst appearance: divergent spicules that resemble a sunburst
  • Interrupted periosteal reactions
    • Occur if continuous periosteal reactions are themselves disturbed by tumor growth
    • Indicate the presence of a particularly aggressive malignant process
    • Codman triangle: develops as a result of the destruction and elevation of singular or multiple periosteal lamellae

Biopsy

Laboratory tests

Overview of primary malignant bone tumors
Age group Main localization Radiographic characteristics Treatment Five-year survival rate
Ewing sarcoma
  • Surgery
  • Polychemotherapy
  • Radiation
  • ∼ 70%
Osteosarcoma
  • Surgery
  • Polychemotherapy
  • ∼ 70%
Chondrosarcoma
  • Adults
  • Moth-eaten appearance
  • Endosteal scalloping
  • Intralesional calcifications
  • Surgery
  • 50–60%

Differential diagnosis of bone pain in children

Benign bone conditions

Growing pains [17][18]

  • Etiology: unknown
  • Epidemiology
    • Affects up to 35% of all children
    • Most commonly seen at 3–12 years of age
  • Clinical features
    • Episodic, bilateral pain that affects predominantly the lower extremities (shins, calves, thighs, popliteal fossa)
    • Pain typically occurs late in the day or during the night children awakening from sleep
    • Pain is not present during the day or activities.
    • May be mild to severe and lasts for a few minutes to several hours; usually resolves by morning
  • Diagnostics
    • The diagnosis is clinical.
    • Imaging shows no abnormalities and is not indicated.
  • Treatment
  • Prognosis
    • Usually good
    • Most cases resolve spontaneously by late childhood.

Description

Clinical features

Diagnostics

Approach

  • Imaging is generally only performed in patients with primary malignancies who have clinical signs or laboratory findings (e.g., elevated alkaline phosphatase, hypercalcemia) indicating metastatic bone disease.
  • Biopsy is performed to confirm the diagnosis when imaging is insufficient or the patient has no known history of cancer.

Laboratory

  • Elevated serum calcium

Radiographic imaging [20]

Biopsy

  • Preferably taken from a soft tissue mass at the afflicted site, otherwise from an accessible bone where biopsy does not cause lasting impairment of motility or stability
  • In the case of an isolated lesion, it should be presumed to be a primary tumor unless proven otherwise.

Treatment

The choice of treatment generally depends on the underlying primary malignancy and stage of the disease. [22]

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