Osteosarcoma: Difference between revisions
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==Background== | ==Background<ref>Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.</ref>== | ||
[[File:Predilection sites of osteosarcoma2.png|thumb|Osteosarcoma occurrence sites]] | |||
*Most common primary pediatric bone tumor | *Most common primary pediatric bone tumor | ||
**3% of all childhood cancers | **3% of all childhood cancers | ||
*Around two-thirds of those diagnosed with non-metastatic disease under the age of 40 will be long-term survivors or cured of the disease with appropriate treatment | *Around two-thirds of those diagnosed with non-metastatic disease under the age of 40 will be long-term survivors or cured of the disease with appropriate treatment | ||
*Survival is less than 20 | *Survival is less than 20% with metastatic disease | ||
**One-fifth will have metastases at presentation | |||
**Most common metastatic site is lung followed by bone | |||
*Bimodal as it peaks in early adolescence and over the age of 65 | *Bimodal as it peaks in early adolescence and over the age of 65 | ||
==Pathophysiology== | ==Pathophysiology== | ||
*Overproduction of osteoid and immature bone by malignant osteoblasts | *Overproduction of osteoid and immature bone by malignant osteoblasts | ||
==Clinical Features== | ==Clinical Features== | ||
*Often an adolescent during a growth spurt | |||
*Bone pain for several months that worsens with activity and is more painful at night | |||
*Absence of constitutional symptoms (e.g., fevers, weight loss, night sweats, and decreased appetite are typically absent) | |||
*Large soft tissue mass on exam that is tender to palpation | |||
*Occurs at metaphysis of long bones | |||
**Most commonly at the distal femur followed by the proximal tibia | |||
*Less frequently presents as pathological fracture | |||
*Risk factors include prior cancer treatment, [[Paget disease]] of bone, benign bone disease, Hereditary Retinoblastoma or Li-Fraumeni syndrome | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Bone tumors and their mimics DDX}} | |||
==Evaluation== | ==Evaluation== | ||
*X-ray | |||
**"Sunburst" pattern on X-ray | |||
***This pattern describes a lytic lesion with periosteal reaction and cortical disruption at or near the metaphysis | |||
[[File:Figure1A_Osteosarcoma.jpg|thumb|(A) Sunburst appearance of Osteosarcoma.]][[File:Figure1B_Osteosarcoma.jpg|thumb|(B) Ultrasound of same patient in (A) showing cortical destruction and boney mass.]] | |||
**Codman triangle | |||
***New subperiosteal bone formed when the periosteum is raised away from the bone | |||
*Elevated alkaline phosphatase | |||
*Elevated LDH | |||
**High LDH associated with poor outcome | |||
*Elevated ESR | |||
*MRI and biopsy for definitive diagnosis | |||
==Management== | ==Management== | ||
*Pain control | |||
*Limb sparing surgery or amputation, if needed | |||
**Depends on location and extent of the primary tumor | |||
*Neoadjuvant chemotherapy | |||
**Response is a major prognostic factor | |||
*MAP therapy (methotrexate, doxorubicin, cisplatin) often regimen of choice for non-metastatic disease | |||
**No standard approach if metastatic | |||
*Typically, radiation resistant | |||
**If at base of skull or sacrum can consider intensity-modulated radiation therapy | |||
==Disposition== | ==Disposition== | ||
*Home if patient can get expedited follow up | |||
*Admission and work-up if suspected delay in outpatient care | |||
==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Orthopedics]] | |||
[[Category:Heme/Onc]] | |||
Latest revision as of 21:02, 3 February 2020
Background[1]
- Most common primary pediatric bone tumor
- 3% of all childhood cancers
- Around two-thirds of those diagnosed with non-metastatic disease under the age of 40 will be long-term survivors or cured of the disease with appropriate treatment
- Survival is less than 20% with metastatic disease
- One-fifth will have metastases at presentation
- Most common metastatic site is lung followed by bone
- Bimodal as it peaks in early adolescence and over the age of 65
Pathophysiology
- Overproduction of osteoid and immature bone by malignant osteoblasts
Clinical Features
- Often an adolescent during a growth spurt
- Bone pain for several months that worsens with activity and is more painful at night
- Absence of constitutional symptoms (e.g., fevers, weight loss, night sweats, and decreased appetite are typically absent)
- Large soft tissue mass on exam that is tender to palpation
- Occurs at metaphysis of long bones
- Most commonly at the distal femur followed by the proximal tibia
- Less frequently presents as pathological fracture
- Risk factors include prior cancer treatment, Paget disease of bone, benign bone disease, Hereditary Retinoblastoma or Li-Fraumeni syndrome
Differential Diagnosis
Bone tumors and their mimics
Malignant
- Multiple myeloma
- Chondrosarcoma
- Paget disease
- Osteosarcoma
- Adamantinoma
- Chordoma
- Primary bone lymphoma
- Fibrosarcoma
- Myosarcoma
Benign
- Giant cell tumor
- Chrondroblastoma
- Enchondroma
- Langerhans cell histiocytosis of bone
- Osteoblastoma
- Osteochondroma
- Osteoid osteoma
Other
Evaluation
- X-ray
- "Sunburst" pattern on X-ray
- This pattern describes a lytic lesion with periosteal reaction and cortical disruption at or near the metaphysis
- "Sunburst" pattern on X-ray
- Codman triangle
- New subperiosteal bone formed when the periosteum is raised away from the bone
- Codman triangle
- Elevated alkaline phosphatase
- Elevated LDH
- High LDH associated with poor outcome
- Elevated ESR
- MRI and biopsy for definitive diagnosis
Management
- Pain control
- Limb sparing surgery or amputation, if needed
- Depends on location and extent of the primary tumor
- Neoadjuvant chemotherapy
- Response is a major prognostic factor
- MAP therapy (methotrexate, doxorubicin, cisplatin) often regimen of choice for non-metastatic disease
- No standard approach if metastatic
- Typically, radiation resistant
- If at base of skull or sacrum can consider intensity-modulated radiation therapy
Disposition
- Home if patient can get expedited follow up
- Admission and work-up if suspected delay in outpatient care
References
- ↑ Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.