II. Epidemiology

  1. Bone Sarcoma is rare
    1. Incidence: 2500 U.S. cases diagnosed per year
  2. Osteosarcoma is most common Bone Sarcoma
    1. Third most common childhood malignancy
  3. Ages affected: 5-30 years (most common ages 10-20)

III. Pathophysiology

  1. High-grade mesenchymal tumor
  2. Most common sites of involvement
    1. Distal femur
    2. Proximal tibia
    3. Proximal Humerus
  3. Most common sites of metastasis
    1. Skip lesions
      1. Regional metastases form via satellite growth
      2. May be transarticular or intraosseous
    2. Systemic metastases
      1. Lungs (most common)
      2. Distant bone (occurs after lung metastases)
  4. Growth
    1. Grows outward radially forming ball-like mass
    2. Reactive Zone (Pseudocapsule composed of muscle)
      1. Forms when Tumor extends beyond bone cortex
      2. Compresses surrounding muscles
      3. Muscle appears as pseudocapsule to tumor
    3. Satellites (Tumor extension beyond pseudocapsule)
      1. Small tumor Nodules form outside pseudocapsule
      2. Microextension of tumor (satellites)

IV. Symptoms

  1. Dull aching unilateral bone pain for several months
    1. Patient often presents for sudden worsening
    2. Referred pain is common in children
  2. Night pain may awaken patient from sleep
    1. Distinguishing feature from benign causes
  3. May be misdiagnosed associated with minor injury
    1. Growing Pains (e.g. Osgood Schlatter)
    2. Recent Knee Injury or strain
  4. Uncommon features
    1. Fever
    2. Night Sweats
    3. Weight loss
    4. Lymphadenopathy (think Osteomyelitis if present)

V. Signs

  1. Local tenderness, swelling, mass or deformity
  2. Patient limps
  3. Muscle atrophy
  4. Decreased joint range of motion
  5. Pathologic Fracture

VI. Differential diagnosis

  1. Ewing's Sarcoma
  2. Osteomyelitis
  3. Osteoblastoma
  4. Giant Cell Tumor
  5. Aneurysmal bone cyst
  6. Fibrous dysplasia

VII. Radiology

  1. Bone XRay
    1. Sclerosis (small, irregular cloud-like densities)
    2. Lytic lesions
    3. Pathologic Fractures
  2. Bone MRI
    1. Defines tumor involvement
      1. Intraosseous and extraosseous changes
      2. Skip lesions
      3. Neurovascular involvement
    2. Used preoperatively
      1. Establishes surgical margins (2-3 cm from tumor)
      2. Establishes resectability
  3. Bone CT with contrast
    1. Preferred over MRI if significant edema and necrosis
    2. Defines neurovascular structures via IV contrast
  4. Other measures used in defining tumor involvement
    1. Bone scintigraphy
    2. Thallium scintigraphy
    3. Angiography

VIII. Staging (Enneking system)

  1. Stage I: Low-grade tumor
  2. Stage IA: Intracompartmental
  3. Stage IB: Extracompartmental
  4. Stage II: High grade tumor
  5. Stage IIA: Intracompartmental
  6. Stage IIB: Extracompartmental
  7. Stage III: Metastatic disease from either grade
  8. Enneking (1980) Clin Orthop 153:106-20 [PubMed]

IX. Monitoring

  1. Timing
    1. First 2 years after treatment: Every 3 months
    2. Two to 5 years after treatment: Every 6 months
    3. Five or more years after treatment: Annual
  2. Protocol
    1. Serial Physical exams
      1. Palpate extremity for masses
      2. Assess for prosthetic failure or Infection
    2. Serial XRay of involved limb
    3. Serial CT Chest
      1. Baseline, at time of diagnosis
      2. Repeat every 6 months to 2 years
  3. Additional testing
    1. Bone scan annually for first 2 years

X. Management

  1. Surgery
    1. Limb salvage in most cases (amputation in some)
    2. Tumor resection and limb reconstruction
  2. Chemotherapy
    1. Phases
      1. Induction (pre-operative Chemotherapy)
      2. Adjuvant (post-operative Chemotherapy)
    2. Common Agents used
      1. Cisplatin
      2. Methotrexate (high does)

XI. Prognosis

  1. Eligible for limb sparing surgery in U.S.: 90-95%
    1. Contrast with 100% amputation rate before 1970
  2. Long-term survival with localized disease: 60-80%
    1. Contrast with 80% mortality rate before 1970

XII. References

  1. Abeloff (2000) Clinical Oncology, Churchill, p. 2170-97
  2. Canale (1998) Campbell's Orthopaedics, Mosby, p. 715-7
  3. Wittig (2002) Am Fam Physician 65(6): 1123-32 [PubMed]

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Ontology: Osteosarcoma (C0029463)

Definition (NCI) A usually aggressive malignant bone-forming mesenchymal tumor, predominantly affecting adolescents and young adults. It usually involves bones and less frequently extraosseous sites. It often involves the long bones (particularly distal femur, proximal tibia, and proximal humerus). Pain with or without a palpable mass is the most frequent clinical symptom. It may spread to other anatomic sites, particularly the lungs.
Definition (NCI_CDISC) A malignant neoplasm usually arising from bone.
Definition (MSH) A sarcoma originating in bone-forming cells, affecting the ends of long bones. It is the most common and most malignant of sarcomas of the bones, and occurs chiefly among 10- to 25-year-old youths. (From Stedman, 25th ed)
Definition (CSP) malignant primary cancer of bone composed of a connective tissue stroma with evidence of malignant osteoid, bone and/or cartilage formation.
Concepts Neoplastic Process (T191)
MSH D012516
ICD10 M9180/3
SnomedCT 21708004, 189878003, 408387006, 307576001
English Osteogenic Sarcomas, Sarcoma, Osteogenic, Sarcomas, Osteogenic, OSRC, OSTEOGENIC SARCOMA, osteochondrosarcoma, Osteosarcomas, OSTEOSARCOMA, [M]Osteosarcoma NOS, osteoid sarcoma, Osteosarcoma, no ICD-O subtype, Osteosarcoma [Disease/Finding], Tumor, Osteosarcoma, Tumors, Osteosarcoma, Osteosarcoma Tumors, Osteosarcoma Tumor, Osteosarcoma, osteosarcomas, bone sarcomas, bone sarcoma, Osteogenic Sarcoma, Sarcoma osteogenic, Osteosarcoma NOS, Osteosarcoma (disorder), Osteosarcoma - disorder (disorder), Osteosarcoma, no International Classification of Diseases for Oncology subtype, Osteosarcoma, no International Classification of Diseases for Oncology subtype (morphologic abnormality), Osteosarcoma, no ICD-O subtype (morphologic abnormality), OSTEOSARCOMA, MALIGNANT, Osteogenic sarcoma, Osteochondrosarcoma, Osteosarcoma - disorder, osteogenic sarcoma, osteosarcoma, sarcoma, osteogenic, Osteogenic sarcoma, NOS, Osteosarcoma, NOS
Swedish Osteosarkom
Czech osteosarkom, sarkomy kostí, sarkom kosti, Osteogenní sarkom, Osteosarkom NOS, Osteosarkom
Finnish Osteosarkooma
Russian OSTEOSARKOMA, SARKOMA OSTEOGENNAIA, ОСТЕОСАРКОМА, САРКОМА ОСТЕОГЕННАЯ
Italian Sarcoma osteogenico, Osteosarcoma NAS, Osteosarcoma
Croatian OSTEOSARKOM
Spanish osteosarcoma, morfología: osteosarcoma, subtipo no clasificado en CIE - O, osteosarcoma, no clasificado como subtipo en CIE-O, morfología: osteosarcoma, subtipo no clasificado en CIE - O (anomalía morfológica), Sarcoma osteogénico, Osteosarcoma NEOM, osteosarcoma, no clasificado como subtipo en la Clasificación Internacional de Enfermedades para Oncología, osteocondrosarcoma, osteosarcoma, no clasificado como subtipo en la Clasificación Internacional de Enfermedades para Oncología (anomalía morfológica), osteosarcoma, no clasificado como subtipo en CIE-O (anomalía morfológica), (M)osteosarcoma, sarcoma osteógeno, Osteosarcoma, Sarcoma Osteogénico
Polish Mięsak kościotwórczy, Mięsak kostnopochodny
Japanese 骨肉腫, 骨形成性肉腫, 骨肉腫NOS, コツニクシュNOS, コツニクシュ, ホネニクシュ, コツゲンセイニクシュ, 骨原性肉腫, 肉腫-骨原性
Norwegian Osteosarkom
Dutch osteosarcoom, osteosarcoom NAO, osteogeen sarcoom, sarcoom osteogeen, Osteosarcoom, Sarcoom, osteo-, Sarcoom, osteogeen
French Ostéosarcome SAI, Ostéosarcome, Sarcome ostéogène, Sarcome ostéogénique
German Osteosarkom NNB, Sarkom osteogen, osteogenes Sarkom, Osteosarkom, Sarkom, osteogenes
Hungarian Osteogen sarcoma, Osteosarcoma, Csonttermelő sarcoma, Osteosarcoma k.m.n.
Portuguese Sarcoma osteogénico, Osteossarcoma, Osteossarcoma NE, Osteosarcoma, Sarcoma Osteogênico

Ontology: Bone Sarcoma (C1704327)

Definition (NCI) A malignant mesenchymal tumor arising from the bone.
Concepts Neoplastic Process (T191)
SnomedCT 448710000
English SARCOMA BONE, Bone Sarcoma, sarcoma of bone, sarcoma of bone (diagnosis), bone sarcoma, Bone sarcoma NOS, Sarcoma bone, Sarcoma of bone (disorder), Sarcoma of bone, Bone sarcomas, Bone sarcoma, Osseous Sarcoma, Sarcoma of Bone, Sarcoma of the Bone, Skeletal Sarcoma
Italian Sarcoma dell'osso, Sarcomi dell'osso, Sarcoma dell'osso NAS, Sarcoma osseo
Dutch botsarcoom NAO, sarcoom bot, botsarcomata, botsarcoom
German Sarkom Knochen, Knochensarkom NNB, KNOCHENSARKOM, Knochensarkome, Knochensarkom
Portuguese Sarcoma ósseo NE, SARCOMA OSSEO, Sarcoma ósseo, Sarcomas ósseos
Spanish Sarcoma óseo NEOM, SARCOMA OSEO, sarcoma de hueso, sarcoma de hueso (trastorno), Sarcoma óseo, Sarcomas óseos
French SARCOME OSSEUX, Sarcome osseux SAI, Sarcome osseux, Sarcomes osseux
Czech Kostní sarkom, Sarkom kosti NOS, Sarkom kosti, Sarkomy kosti
Hungarian Csont sarcoma k.m.n., Csontsarcoma, Csont sarcomák, Csont sarcoma
Japanese 骨の肉腫, コツノニクシュ, コツノニクシュNOS, 骨の肉腫NOS