Osteosarcoma

About
• Malignant tumor arise within bone & spreading rapidly outward to periosteum & surrounding soft
tissue
• 2nd most common tumor in orthopedic
• Highly malignant primary bone tumor

Most common
• Most common age group: Children, young adult (15 – 25 years old)
• Most common musculoskeletal tumor in children & young adult
• Most common site: Long bone metaphysis such as
» Lower end of femur (Most common)
» Upper end of tibia (Common)
» Upper end of humerus (Less common)
• Why osteosarcoma most common in metaphysis?
» Growth based on Johnson’s theory of field selection, proliferative primitive spindle cells lodged in
metaphysis

History
• Bone pain (75%) – 1st symptom, constant, boring in nature, worse at night, increase severity
• Swelling (lump) – 2nd symptoms (Most common 100%)
• History of trauma
• Pathological fracture – rare

Examination
• Swelling at metaphysis
• Local tenderness
• Palpable mass (late presentation)
• Skin over swelling is shiny + prominent veins
• Warm swelling
• Inflamed overlying tissue

Pathology
• Osteosarcoma: Malignant tumor of mesenchymal cells characterized by formation of osteoid or bone
by tumor cells

Gross morphology Histopathology

1. Osteoblastic type
• Greyish white, hard, gritty architecture
2. Chondroid type
• Opalescent, bluish grey
3. Fibroblastic type
• Typical fish flesh sarcomatous appearance
4. Telangiectatic
• Large area of tumor necrosis
• Blood filled spaces within tumor mass
Classification

Clinical Classification Histopathology classification

1. Primary Osteosarcoma 1. Osteoblastic
• Most common • High bone formation
• Age group: 15 – 25 years old 2. Chondroid
• No pre-malignant conditions • Malignant cell is cartilage cell
• More malignant than secondary osteosarcoma 3. Fibroblastic
2. Secondary Osteosarcoma • Malignant cell is fibroblast
• Less common 4. Telangiectatic or Osteolytic
• Age group: > 45 years old • Predominantly lytic tumor
• Pre malignant lesions: Paget’s disease, Multiple • Most agressive
endochromatosis, Fibrous dysplasia, Irradiation
to bone, Multiple osteochondroma
• Treatment: Same as primary osteosarcoma

Metastasis
• Hematogenous (through blood)
• 1st organ to metastasize: Lung
• Most common Bone metastasis – Vertebra
• Lymph node involvement: Uncommon
• Osteosarcoma rarely invade epiphyseal plate

Investigation

Investigation Reasons Expected indings

FBC Monitoring •  WBC
ESR Monitoring •  ESR
Serum Alkaline Useful for follow-up case; •  Serum Alkaline phosphatase
phosphatase (SAP) Indicator of recurrence or
metastasis
Bone X-ray Diagnosis • Area of irregular destruction in metaphysis
• New bone formation in tumor matrix
• Bone cortex is eroded
• Irregular periosteal reaction (elevation)– tumor
lift periosteum
• Codman’s triangle (typical)
• Sun-ray appearance (Pathognomonic) – due to
calcification along periosteal blood vessel
• Pathological fracture
Core needle biopsy Gold standard in diagnosis • Confirm diagnosis by gross morphology &
Open Biopsy histopathology by pathologist
FNAC • Core needle biopsy is ideal – because less
bleeding, minimum contamination
• Diagnostic: Presence of malignant osteod
Chest X-ray Metastasis • Multiple lung nodule (lung metastasize)
• Lung is the 1st site of metastasis
Chest CT-scan Metastasis • If Chest X-ray not clear
 • To detect micro metastasis
• More sensitive than Chest X-ray
• 10% patient have lung metastasis when
diagnosis is made
Bone scan Metastasis • To detect intra-medullary spread (Skip lesions)
MRI Metastasis • To detect soft tissue spread
• Best investigation for local staging
CT-scan Metastasis • To detect soft tissue spread

Confirm diagnosis

Open biopsy (Gold standard)


Clinical + Radiological (If biopsy unsure)

How to suspect osteosarcoma clinically?

• Young age group – 15 – 25 years old
• No joint involvement, but may have extra-articular restriction
• No constitutionals ymptoms

Treatment

“It is important to eradicate the primary lesion completely; the mortality rate after local recurrence is far
worse than following effective ablation at the first encounter” Apley concise orthopaedic

Stage & Treatment

Stage Description Treatment

IA Intracompartmental low grade Limb-saving surgery (Radical excision)
IB Extracompartmental low grade Amputation
II A Intracompartmental high grade Limb-saving surgery (Radical excision)
II B Extracompartmental high grade Amputation
(Most common stage 70%)
III Metastasis (Common stage 30%) Palliative ablation/ Chemo/ Radiotherapy

1. Local control (Surgical removal)

(1) Amputation (mainstay treatment) (2) Limb-saving surgery (Radical excision)

Site Level Commonly perform
Lower end femur • Mid thigh amputation For early diagnosis
(early lesion) After 8 – 12 weeks of pre-surgery (neo adjuvant)
• Hip disarticulation chemotherapy
Upper end femur • Hip disarticulation No skip lesions
(early lesion) Tumor is resectable
• Hindquarter 
amputation Perform radical excision
Upper end tibia • Mid thigh amputation 
Bone defect after surgery is filled with bone graft
 Upper end humerus • Forequarter or prosthesis
amputation 
Post-surgery Chemotherapy
(3) Radiotherapy
Indication:
• Tumor found at surgically inaccessible site
• Patient refuse surgery

2. Control distant macro or micro-metastasis

• Majority cases, micro-metastasis occur when diagnosis is made

(1) Neoadjuvant Chemotherapy (2) Immunotherapy

• Given pre or post-surgery
• High dose: Methotrexate, Citrovorum factor,
Endoxan, Cisplastinum
• Highly toxic, need to manage adverse effect
• Give for 8 – 12 weeks
• Not yet practice as its new treatment
• Principle: Portion of tumor (donor) is implanted
into sarcoma survivor (recipient) & the tumor
removed after 14 days. Sensitized lymphocytes
from survivor then infused into patient to kill the
cancer cells

Prognosis
• Without treatment, death occur within 2 years ~ usually within 6 months if there is micro-metastasis
• 20% survive with Surgery
• 70% survive with Surgery + Adjuvant Chemotherapy
• Worst prognosis: Telangiectatic osteosarcoma

Course of osteosarcoma if not treated

• It breaches the cortex and the soft tissues interior of muscles, nerves, vascular structures and distant
metastasis and not by lymphatics. This is because bone has no lymphatics.

Difference between Osteosarcoma & Ewing sarcoma

Osteosarcoma Ewing sarcoma

Location Appendicular skeleton 50% axial skeleton
50% appendicular skeleton
Constitutional symptoms Absent Present