Bone tumor final

Bone tumor Overview

1. Bone Tumors Dr. Sudarshan Pandey First year Resident Department of Orthopaedics and Traumatology

2. Epidemiology Bone and soft tissue sarcoma – derived from mesenchymal origin Bone sarcoma – 0.2% Male > Female Age – male >85 years , female- 50-60 years

3. Epidemiology

4. Classification Benign lesion Non ossifying fibroma Aneurysmal bone cyst Intermediate (locally aggressive) lesion Osteoblastoma

5. Classifications Intermediate (rarely metastasizing) lesion Risk of spread < 2% , often non fatal Giant cell tumor of bone Malignant tumors Aggressiveness defined by histological grade Low grade tumor- metastatic rate: 2-10% chordoma, parosteal osteosarcoma High grade tumor – metastatic rate : 20- 100 % Osteosarcoma, Ewing's sarcoma

6. World Health Organization (WHO) histological classification of tumours

7. Field theory of bone tumors

8. General approach History- patient information, Pain , Mass Examination Investigation Imaging ( X rays , CT , MRI , Radionuclide scanning) Systemic evaluation ( in case of secondaries ) PSA, Bence - Jones protein, Serum Calcium, ALP ESR , CRP Biopsy – gold standard

9. Clinical Presentation Asymptomatic Pain Swelling/mass All superficial soft tissue lesions measuring >5cm and all deep seated lesions should be considered a sarcoma until proven otherwise Pathological fractures Systemic findings

10. Investigations X-Rays

11. Epiphyseal Lesions Chondroblastoma (ages 10-25) Giant cell tumor (ages 20-40) Clear chondrosarcoma (rare) 15y/M 25y/M

12. Ewing sarcoma (ages 5-25) Lymphoma (adult) Fibrous dysplasia (ages 5-30) Adamantinoma (consider in the tibia) Histiocytosis (ages 5-30) Diaphyseal Lesions

13. Location in Bone Axial Central – Enchondroma , Unicameral Bone Cyst Conventional osteosarcoma Ewing sarcoma Myeloma, lymphoma Eccentric- Aneurysmal bone cyst(ABC) GCT, Non Ossifying fibroma(NOF) Eosinophilic granuloma Enchondroma , fibrous dysplasia

14. Cortical Cortical osteoid osteoma Fibrous cortical defect Adamantinoma Osteofibrous dysplasia Pagets disease Juxtacortical Parosteal chondroma Surface osteosarcoma Osteochondroma Exostotic chondrosarcoma Surface osteosarcoma

15. Radiographic features Lesion margin Periosteal reaction Mineralized matrix

16. Lodwick classification of lytic bone lesions Low biological activity/aggressiveness High biological activity / aggresiveness

17. Perisoteal reaction Non aggressive Continuous / solid Aggressive codman’s triangle Sun burst appearance / spiculated /hair on end Lamellated Benign Aggressive

18. Mineralized matrix Osteoid Chondroid Fibrous

19. Lesions of the Spine Older than 40 Years Metastases Vertebral body, pelvis Proximal femur, proximal humerus skull Multiple myeloma Hemangioma Chordoma (in sacrum) Younger than 30 Years Vertebral body Histiocytosis Hemangioma Posterior elements Osteoid osteoma Osteoblastoma Aneurysmal bone cyst

20. Multiple Lesions Histiocytosis Enchondroma Osteochondroma Fibrous dysplasia Multiple myeloma Metastases Hemangioma Infection Hyperparathyroidism

21. Sclerotic metastasis Prostatic Ca Breast Ca Transitional Cell Ca Carcinoid Mucinous Adeno Ca Lytic bone metastasis Lung Ca Renal Cell Ca Thyroid Ca Adrenal gland Ca Uterine Ca Melanoma

22. Computed tomography Shows accurately intraosseous and extraosseous extension Great for cortical bone evaluation Helps in staging MRI Defines local extent of lesion , tissue characterization Useful in assessing soft tissue tumor and cartilaginous tumor Radionuclide scanning 99mTcMDP – non specific reactive changes – reveal site of small tumor (osteoid osteoma) Detecting skip lesions , evidence of metastatic disease

23. Definition of histopathological parameters in the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grading system (HPF = high-power field)

24. Enneking staging system Based on: Tumor grade Metastasis Confinement in compartment A compartment, for the purposes of this system, is defined as an enclosed tissue space, such as a bone, a joint space or a muscle group confined by its fascial envelope

25. Enneking staging system Malignant Stage Grade Site Metastasis IA Low Grade Intra- compartmental No metastasis IB Low Grade Extra- compartmental No metastasis IIA High Grade Intra- compartmental No metastasis IIB High Grade Extra- compartmental No metastasis III Any Any Metastases Benign Latent—low biologic activity; well marginated ; often incidental findings (i.e., nonossifying fibroma) Active—symptomatic ; limited bone destruction; may present with pathologic fracture (i.e., aneurysmal bone cyst) Aggressive—aggressive; bone destruction/soft-tissue extension(i.e ., giant cell tumor)

26. TNM staging Definition of regional lymph node (N)

27. TNM staging Spine Pelvis

28. TNM stages Stage 1A Low-grade , small, no metastases Stage 1B Low-grade , large, no metastases Stage 2A Intermediate- or high- grade , small, no metastases Stage 2B Intermediate-grade , large, no metastases Stage 3 High-grade , large, no metastases Stage 4 Any with metastases

29. AJCC System

30. Biopsy-Principles R eferred to the institution where definitive treatment will take place . Should be done after clinical, lab and radiographic examinations Planned placement of biopsy incision biopsy track should be considered contaminated with tumor cells biopsy track needs to be excised en bloc with the tumor The surgeon performing the biopsy should be familiar with incisions for limb salvage surgery and standard and nonstandard amputation flaps

31. Biopsy- Principles If a tourniquet is used, the limb elevated before inflation but should not be exsanguinated by compression to prevent “squeezing” the tumor’s cells into the systemic circulation Transverse incisions should be avoided The deep incision should go through single muscle compartment Avoid major neurovascular structure Soft tissue extension of bone lesion should be sampled

32. Biopsy- Principles hole in the bone should be round or oval Frozen section should be sent intraoperatively to ensure that diagnostic tissue has been obtained meticulous hemostasis ensured before closure Drain should exit in line with the incision Wound should be closed tightly in layers

33. Biopsy- Principles Sample should be sent for microbiology as well as histology The pathologist reporting biopsy must have an appropriate level of experience If risk of fracture following biopsy, bone must be splinted

34. Types of Biopsy

35. Bone tumor Mimics Soft tissue hematoma Myositis Ossificans Stress fractures Tendon avulsion injuries Infection Osteopetrosis Osteopoikilosis Melorheostosis - dripping candle wax

36. Management Primary Goal with primary malignancy- make patient disease free Goal of treatment of patient with metastatic carcinoma to bone: Minimize pain Preserve function Optimal treatment of tumor : Surgery Radiation therapy Chemotherapy

37. Radiation therapy Blue cell tumor Multiple myeloma Lymphoma Ewings sarcoma Secondaries (Except – RCC) Reduce local recurrence of malignant soft tissue tumor

38. Chemotherapy Adjuvant Neoadjuvant Osteosarcoma Malignant fibrous histiocytoma Rhabdomyosarcoma Not useful in cartilaginous lesion

39. Principles of Surgery Amputation vs Limb salvage Survival? Morbidity ? Salvaged limb vs prosthesis Psychosocial conseuences

40. References Campbell’s Operative Orthopaedics -13 th edition Apley and Solomon’s System of Orthopaedics and Trauma -10 th edition Millers Review of Orthopedics- 7 th edition

41. Thank you