Osteosarcoma - bone malignancy Dr. Anu Aggrawal Musculoskeletal Oncosurgeon Delhi, India dranu.isic@gmail.com

1. Osteosarcoma
Presenter : Dr. Anu Aggrawal
Musculoskeletal Oncosurgeon
dranu.isic@gmail.com

2. Introduction
• High-grade malignant tumor of mesenchymal origin
• Mesenchymal cells producing osteoid and immature matrix
• 2ND most common primary malignant bone tumor (after MM)
• Most common non-haematological primary malignancy of bone
• ~80% have metastasis or micro-metastasis at diagnosis
• Advances in surgery- decreased number of amputations
• Prognosis improved from <30% to > 70% survival

3. History
• 1805- term ‘osteosarcoma’ : French surgeon Alexis Boyer
• 1847- natural history : Guillaume Dupuytren
• 1854- first histologic description of bone tumor : Hermann Lebert
• 1879- Amputation and radical resection : Samuel Gross
• 1909- features of osteosarcoma on X-rays : Ernest Codman
• Amputation was treatment of choice till 1970s
• With the advancement of chemotherapeutics- prognosis improved from 30% to 70%

4. Epidemiology
• Incidence : 2 to 3 /million/year
• Slightly male predominance (1.5 -1)
• Any age ( 75%- 12- 25 years)
• Bimodal age distribution
• Elderly : secondary osteosarcoma
• Rarely familial

5. Etiology
• Unknown true aetiology
• Multifactorial – genetic and environmental
• Mutations of tumor suppressor genes
• Retinoblastoma : RB1 gene
• Li-Fraumeni syn-drome: p53 tumor suppressor gene
• Rothman-Thomas syndrome: RECQL4 gene
• Ionozing radiation

6. Classification
Primary
• Intramedullary
• Conventional
• Telangiectatic
• Small cell
• Low-grade
• Juxtracortical
• Parosteal
• Periosteal
• High-grade surface
Secondary
Pagets disease
Radiation induced
Other bone lesions
Metastatic osteosarcomas

7. Conventional Osteosarcoma
• High-grade intramedullary tumor
• Metaphyseal regions of long bones
• 75% of all osteosarcomas
• 2nd or 3rd decade of life (15-25 years)
• Male: female :: 1.5-2.0:1

9. • Metaphyseal origin
• 10% - primary diaphyseal
• <1%- primary epiphyseal
• Metastasis : commonly lungs
• Skip lesions – 1-25%

10. Clinical features
• Pain and swelling- most frequent symptoms
• History of trauma
• 15-20%- pathological fracture
• Mild to moderate regional LN enlargement- ocassional

11. Serology
• ALP- elevated
• Normal after ablation
• Rises again in relapse cases
• LDH- less frequently increases

12. Imaging
• Intramedullary but breaches cortex
• Combination – radiolucency and osseous
• Soft tissue involvement- fluffy to cumulus cloud like radiodensities
• Peristeal reaction: (Codman triangle, lami-nated, hair-on-end, or
sunburst patterns)

13. CT
• Intraosseous and extra osseous extension
• Intratumoral and peritumoral densities

14. MRI
• Pre-operative choice of investigation for staging and evaluation

15. PET-CT
• Skip
• Metastasis

16. Advantages of multiplanar imaging
(1) Anatomic landmarks for the extent of marrow and soft tissue
involvement and their relationship to surrounding structures
(2) Invasion of the epiphysis
(3) Involvement of the joint or neurovascular structures
(4) Identification of skip metastases, and
(5) Identification of areas of viable tumor and mineralized matrix which
improve biopsy accuracy
Accurate assessment of the intra- and extraosseous extent of
osteosarcoma is critical in directing limb-salvage procedures

17. Treatment
Neoadjuvant chemotherapy
Wide resection surgery + resection of metastases
Adjuvant chemotherapy
Radiotherapy- in inoperable cases

18. History
• 10% survival rate
• 80% with pulmonary metastases at diagnosis
• 1931: Sir Stanford Cade a British Surgeon Radiotherapist
• Advocated radiotherapy to treat primary bone tumors
• Metastases observed for 6-9 months
• No metastases- elective amputation

19. • 1969: post-operative chemotherapy
• 1978: pre and post-op CT

20. Current treatment
• NACT , wide excision surgery and adjuvant CT
• Surgery is essential for local control
• Amputation (3%) and LSS (8.2%)

21. Prognosis
• 10 years survival (non-metastatic OS)
• Without CT: 15%
• With CT: 70%
• Local recurrence
• Post amputation: 2-3%
• Pre-op CT and conservative surgery: 5-7%
• Metastases – MC lungs
• First 2-3 years

22. TELANGIECTATIC OSTEOSARCOMA
• Described by Paget in 1854
• 1903: malignant bone aneurysm – Gaylord
• 1922: Ewing
• Classified distinct histologic variant
• Characterized by a malignant, osteoid-forming sarcoma of bone with large blood-
filled vascular channels.
• WHO: purely lytic destructive process without matrix mineralization

23. • Rapidly aggressive course
• Sex, age and localizations- same as conventional
• Imaging: purely lytic lesion
• Ill-defined margins
• Extensive cortex disruptions
• Scarce periosteal reaction
• CT and MRI: multicystic tissue with fluids levels

25. • Treatment and prognosis similar to conventional OS
• Course is usually very rapid

26. Small cell Osteosarcoma
• 1% of high grade osteosarcoma (rare)
• Subtype of conventional OS composed of small , round blue cells
• Male = Female
• Age, site and clinical features – similar to convetional OS
• Metaphyseal with frequent extension to epiphysis
• 15% - diaphysis
• Prognosis – poor regardless of treatment

28. Low Grade Intraosseous Osteosarcoma
• 1–2% of all osteosarcomas
• Well-differentiated or sclerosing osteosarcoma
• Third decade of life
• M=F
• Non-specific symptoms or asymptomatic
• Site and localization – similar to conventional OS
• Imaging: well defined, sclerotic rim, prominent internal trabeculations
• Intralesional resection – high local recurrence
• Wide resection- excellent results
• 15% incompletely treated- high grade transformations

29. Parosteal Osteosarcoma
• 65% of juxtacortical OS (5% of all OS)
• Origin: outer layer of periosteum
• Abundant mineralized bone production
• Low grade of anaplasia
• Slow indolent course
• F>M
• 3rd -4th decade of life

30. • Metaphyseal region
• MC: posterior distal femur
• c/f: Palpable mass with bony hard consistency
• Mechanical limitation of joint movement
• Imaging: round-lobulated mass
• Ossified juxta cortical
• Radiolucent cleavage between mass and bone’
• Invasion into medullary cavity

31. • Surgery with wide margins
• CT indicated in higher grades
• Excellent prognosis with wide resection

32. Periosteal Osteosarcoma
• Chondroblastic OS
• Intermediate grade
• Origin: periosteum
• Diaphysis
• M>F
• 2nd decade
• MC- tibia , femur
• Symptom: palpable mass

33. • Imaging: periosteal fusiform solid mass
• Well defined borders
• Cortex scalloping
• No intra-medullary extension
• Perpendicular periosteal reaction extending into the soft tissue
• Chondroblastic: subtle mineralization

34. Treatment
• LSS with wide local excision
• No role of CT
• Good prognosis after adequate surgical removal

35. High grade surface Osteosarcoma
• Peripheral conventional OS
• Rare ( 10% of Juxtacortical OS)
• Origin: periosteum or parosteal tissues
• MC: femur , Humerus and fibula – Diaphysis
• Imaging: similar to periosteal OS with minimal involvement if underlying
cortex
• Treatment, pathology and prognosis – similar to conventional OS

37. Intracortical Osteosarcoma
• Very rare
• High grade
• Shaft of long bones
• Radiolucent defect confined within the cortex
• Well defined tumor margin with thickened
surrounding cortex

40. Thank you