Hematology and Oncology Book



Aka: Osteosarcoma, Osteogenic Sarcoma, Bone Sarcoma
  1. Epidemiology
    1. Bone Sarcoma is rare
      1. Incidence: 2500 U.S. cases diagnosed per year overall
      2. Of these, 1200 cases of Osteosarcoma are diagnosed
    2. Osteosarcoma is most common Bone Sarcoma and the most common Bone Cancer
      1. Third most common childhood malignancy
    3. Ages affected: 5-30 years (most common ages 10-20)
      1. Median Incidence age 12 years in girls and age 16 years in boys
      2. Rare after age 60 years
    4. Gender
      1. More common in males
    5. Race
      1. More common in black patients
  2. Risk Factors
    1. Genetic factors
      1. Li-Fraumeni Syndrome (p53 oncogene mutation)
      2. Retinoblastoma (Rb oncogene mutation)
      3. Rothmund-Thomson Syndrome (Autosomal Recessive)
    2. Other factors
      1. Radiation Therapy (precedes Osteosarcoma by 10-20 years)
      2. Orthopedic surgery with implanted metal prosthesis
      3. Paget Disease
  3. Pathophysiology
    1. High-grade mesenchymal tumor
      1. Primitive mesenchymal cells differentiate into Osteoblasts
      2. Osteoblasts generate malignant osteoid matrix
    2. Most common sites (60% of cases) of involvement
      1. Long bone metaphysis
        1. Distal femur
        2. Proximal tibia
        3. Proximal Humerus
      2. Other sites
        1. Pelvis
        2. Skull
    3. Most common sites of metastasis
      1. Skip lesions
        1. Regional metastases form via satellite growth
        2. May be transarticular across a joint or intraosseous along the same bone
      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. Metaphysis contains Growth Plate and Osteosarcomas are most common when bone growth is rapid
      3. 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
      4. Satellites (Tumor extension beyond pseudocapsule)
        1. Small tumor Nodules form outside pseudocapsule
        2. Microextension of tumor (satellites)
  4. 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)
  5. Signs
    1. Local tenderness, swelling, mass or deformity
    2. Patient limps
    3. Muscle atrophy
    4. Decreased joint range of motion
    5. Pathologic Fracture
  6. Differential diagnosis
    1. Ewing's Sarcoma
    2. Osteomyelitis
    3. Osteoblastoma
    4. Giant Cell Tumor
    5. Aneurysmal bone cyst
    6. Fibrous dysplasia
  7. Imaging
    1. Bone XRay
      1. Sunburst appearance (radiating calcifications)
      2. Dense sclerosis (small, irregular cloud-like densities) most commonly at metaphysis
      3. Lytic lesions (30% of cases)
      4. Pathologic Fractures
    2. Bone MRI
      1. Defines tumor involvement
        1. Intraosseous and extraosseous changes
        2. Soft tissue extension (75% of cases)
        3. Skip lesions
        4. 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. Chest XRay
        1. Up to 20% of patients present with lung metastases
      2. Bone scintigraphy
      3. Thallium scintigraphy
      4. Angiography
  8. 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]
  9. 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
  10. Management
    1. Surgery (preceded and followed by Chemotherapy)
      1. Limb salvage in most cases
        1. Amputation in some cases of large tumor, vital structure risk, tumor progression or poor response
      2. Tumor resection with clear margins and limb reconstruction
    2. Chemotherapy
      1. Phases
        1. Neoadjuvant (pre-operative Chemotherapy)
        2. Adjuvant (post-operative Chemotherapy)
      2. Common Agents used
        1. Cisplatin
        2. Doxorubicin (Adriamycin)
        3. Methotrexate (high dose)
        4. Additional agents may be used in combination (e.g. Etoposide, Isosfamide)
  11. Prognosis: Survival
    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
  12. Prognosis: Predictive factors (same as for Ewing's Sarcoma)
    1. Positive prognostic factors
      1. Localized extremity disease
      2. Clear margins on surgical resection
      3. Tumor necrosis response >90%
    2. Negative prognostic factors
      1. Incomplete surgical resection
      2. Older patient age at onset
      3. Poor Chemotherapy response
      4. Metastases present
      5. Axial skeleton primary tumors
      6. Tumor >8 cm in size
  13. References
    1. Abeloff (2000) Clinical Oncology, Churchill, p. 2170-97
    2. Canale (1998) Campbell's Orthopaedics, Mosby, p. 715-7
    3. Ferguson (2018) Am Fam Physician 98(4): 205-13 [PubMed]
    4. Wittig (2002) Am Fam Physician 65(6): 1123-32 [PubMed]

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
Italian Sarcoma osteogenico, Osteosarcoma NAS, Osteosarcoma
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
Derived from the NIH UMLS (Unified Medical Language System)

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
Derived from the NIH UMLS (Unified Medical Language System)

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