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Bone Tumors
Dr. Sudarshan Pandey
First year Resident
Department of Orthopaedics and Traumatology
Epidemiology
• Bone and soft tissue sarcoma – derived from mesenchymal origin
• Bone sarcoma – 0.2%
• Male > Female
• Age – male >85 years , female- 50-60 years
Epidemiology
Classification
• Benign lesion
• Non ossifying fibroma
• Aneurysmal bone cyst
• Intermediate (locally aggressive) lesion
• Osteoblastoma
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
World Health Organization (WHO) histological classification of tumours
Field theory of bone tumors
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
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
Investigations
• X-Rays
Epiphyseal Lesions
• Chondroblastoma (ages 10-25)
• Giant cell tumor (ages 20-40)
• Clear chondrosarcoma (rare)
15y/M
25y/M
• Ewing sarcoma (ages 5-25)
• Lymphoma (adult)
• Fibrous dysplasia (ages 5-30)
• Adamantinoma (consider in
the tibia)
• Histiocytosis (ages 5-30)
Diaphyseal Lesions
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
• Cortical
• Cortical osteoid osteoma
• Fibrous cortical defect
• Adamantinoma
• Osteofibrous dysplasia
• Pagets disease
• Juxtacortical
• Parosteal chondroma
• Surface osteosarcoma
• Osteochondroma
• Exostotic chondrosarcoma
Surface osteosarcoma
Radiographic features
• Lesion margin
• Periosteal reaction
• Mineralized matrix
Lodwick classification of lytic bone lesions
Low biological
activity/aggressiveness
High biological activity
/aggresiveness
Perisoteal reaction
• Non aggressive
• Continuous / solid
• Aggressive
• codman’s triangle
• Sun burst appearance / spiculated/hair on end
• Lamellated
Benign Aggressive
Mineralized matrix
• Osteoid
• Chondroid
• Fibrous
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
Multiple Lesions
• Histiocytosis
• Enchondroma
• Osteochondroma
• Fibrous dysplasia
• Multiple myeloma
• Metastases
• Hemangioma
• Infection
• Hyperparathyroidism
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
• 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
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
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
1. Latent—low biologic activity; well
marginated; often incidental findings (i.e.,
nonossifying fibroma)
2. Active—symptomatic; limited bone
destruction; may present with pathologic
fracture (i.e., aneurysmal bone cyst)
3. Aggressive—aggressive; bone
destruction/soft-tissue extension(i.e., giant
cell tumor)
TNM staging
Definition of regional lymph node (N)
TNM staging
Spine
Pelvis
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
AJCC System
Biopsy-Principles
• Referred 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
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
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
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
Types of Biopsy
Bone tumor Mimics
• Soft tissue hematoma
• Myositis Ossificans
• Stress fractures
• Tendon avulsion injuries
• Infection
• Osteopetrosis
• Osteopoikilosis
• Melorheostosis- dripping candle wax
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
Radiation therapy
• Blue cell tumor
• Multiple myeloma
• Lymphoma
• Ewings sarcoma
• Secondaries (Except – RCC)
• Reduce local recurrence of malignant soft tissue tumor
Chemotherapy
• Adjuvant
• Neoadjuvant
• Osteosarcoma
• Malignant fibrous histiocytoma
• Rhabdomyosarcoma
• Not useful in cartilaginous lesion
Principles of Surgery
• Amputation vs Limb salvage
1. Survival?
2. Morbidity ?
3. Salvaged limb vs prosthesis
4. Psychosocial conseuences
References
• Campbell’s Operative Orthopaedics -13th edition
• Apley and Solomon’s System of Orthopaedics and Trauma -10th
edition
• Millers Review of Orthopedics- 7th edition
Thank you

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Bone tumor final

  • 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
  • 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.
  • 8. Field theory of bone tumors
  • 9. 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
  • 10.
  • 11.
  • 12. 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
  • 14. Epiphyseal Lesions • Chondroblastoma (ages 10-25) • Giant cell tumor (ages 20-40) • Clear chondrosarcoma (rare) 15y/M 25y/M
  • 15. • Ewing sarcoma (ages 5-25) • Lymphoma (adult) • Fibrous dysplasia (ages 5-30) • Adamantinoma (consider in the tibia) • Histiocytosis (ages 5-30) Diaphyseal Lesions
  • 16. 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
  • 17. • Cortical • Cortical osteoid osteoma • Fibrous cortical defect • Adamantinoma • Osteofibrous dysplasia • Pagets disease • Juxtacortical • Parosteal chondroma • Surface osteosarcoma • Osteochondroma • Exostotic chondrosarcoma Surface osteosarcoma
  • 18. Radiographic features • Lesion margin • Periosteal reaction • Mineralized matrix
  • 19. Lodwick classification of lytic bone lesions Low biological activity/aggressiveness High biological activity /aggresiveness
  • 20.
  • 21. Perisoteal reaction • Non aggressive • Continuous / solid • Aggressive • codman’s triangle • Sun burst appearance / spiculated/hair on end • Lamellated Benign Aggressive
  • 22. Mineralized matrix • Osteoid • Chondroid • Fibrous
  • 23. 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
  • 24. Multiple Lesions • Histiocytosis • Enchondroma • Osteochondroma • Fibrous dysplasia • Multiple myeloma • Metastases • Hemangioma • Infection • Hyperparathyroidism
  • 25. 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
  • 26. • 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
  • 27. 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
  • 28. 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 1. Latent—low biologic activity; well marginated; often incidental findings (i.e., nonossifying fibroma) 2. Active—symptomatic; limited bone destruction; may present with pathologic fracture (i.e., aneurysmal bone cyst) 3. Aggressive—aggressive; bone destruction/soft-tissue extension(i.e., giant cell tumor)
  • 29. TNM staging Definition of regional lymph node (N)
  • 31. 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
  • 33. Biopsy-Principles • Referred 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
  • 34. 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
  • 35. 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
  • 36. 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
  • 38.
  • 39. Bone tumor Mimics • Soft tissue hematoma • Myositis Ossificans • Stress fractures • Tendon avulsion injuries • Infection • Osteopetrosis • Osteopoikilosis • Melorheostosis- dripping candle wax
  • 40. 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
  • 41. Radiation therapy • Blue cell tumor • Multiple myeloma • Lymphoma • Ewings sarcoma • Secondaries (Except – RCC) • Reduce local recurrence of malignant soft tissue tumor
  • 42. Chemotherapy • Adjuvant • Neoadjuvant • Osteosarcoma • Malignant fibrous histiocytoma • Rhabdomyosarcoma • Not useful in cartilaginous lesion
  • 43. Principles of Surgery • Amputation vs Limb salvage 1. Survival? 2. Morbidity ? 3. Salvaged limb vs prosthesis 4. Psychosocial conseuences
  • 44. References • Campbell’s Operative Orthopaedics -13th edition • Apley and Solomon’s System of Orthopaedics and Trauma -10th edition • Millers Review of Orthopedics- 7th edition

Editor's Notes

  1. Bone sarcoma – 0.2% of all new cancers diagnosed UK , Appleys
  2. Age-specific incidence rates (ASIR) for soft-tissue sarcomas and primary sarcomas of bone (a) Softtissue sarcomas in the UK 1996–2010; (b) primary bone sarcomas in the UK for the same period (National Cancer Intelligence Network (NICN) data). bone sarcomas demonstrate a bimodal distribution in both males and females, with peaks of incidence seen in both teenage/adolescent years and the elderly
  3. Benign – doesn’t invade and spread surrounding tissue, non destructive , surgical resection – curative Osteoblastoma – infiltrative and locally destructive growth pattern – en bloc resection – curative
  4. Higher grade tumor have > 25% chance of local recurrence and distant spread Low grade lesion< 25 % chance of local recurrence and mets
  5. IF tumors are analyzed, there is preferential sites of origin within each bone. A particular tumor of given cell type usually arises in the field where homologous normal cells are most active . Eg GCT
  6. Age , Sex (GCT: F>M), Race , Hereditary – Multiple hereditary exostosis AD
  7. Katanoda, K., Shibata, A., Matsuda, T., Hori, M., Nakata, K., Narita, Y., … Nishimoto, H. (2017). Childhood, adolescent and young adult cancer incidence in Japan in 2009–2011. Japanese Journal of Clinical Oncology, 47(8), 762–771
  8. CMF = Chondromyxoid fibroma SBC = Simple Bone Cyst EG = Eosinophilic Granuloma FD = Fibrous dysplasia HPT = Hyperparathyroidism with Brown tumor
  9. Pain – night pain referred pain Pathological fractures – 5-12% osteosarcoma , 21% in chondrosarcoma s/c # of femur in children, avulsion # of L/T in adults – bone tumors
  10. Very little trabecular bone in the diaphysis to provide an interface to allow the lesion to be seen
  11. Simple Bone Cyst /unicameral bone cyst
  12. If diaphyseal lesion is on radiograph cortex is involved , large medullary cavity diaphyseal lesion may be invisible d/t little trabecular bone in diaphysis to provide interface to allow lesion to be seen
  13. Osteoid osteoma
  14. 99mTcMDP – methyl diphosphonate
  15. aid in treatment decision making, provide some determination of prognosis, and allow meaningful comparisons of treatment methods.
  16. 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 Low grade : well differentiated , few mitoses and exibit moderate cytological atypia , risk of mets<25% High grade : high cell to matrix ratio A: well defined anatomical compartment (cortex, joint capsule , fascial septa)
  17. Tumor node metastasis staging- applied to primary sarcomas of bone
  18. AJCC System – based on prognostic variables Stage I- low grade , II high grade Skip metastasis – discontinuous lesion within the same bone Since the patient with non pulmonary metastasis from osteosarcoma and ewings sarcoma have worse prognosis than with pulmonary mets
  19. Raw area is covered with bone wax or methylmethacrylate cement to reduce bleeding , contamination from cut bone edge because they are extremely difficult or impossible to excise with the specimen single muscle compartment rather than contaminating an intermuscular plane
  20. round or oval to minimize stress concentration and prevent a subsequent fracture The hole should be plugged with methacrylate to limit hematoma formation. meticulous hemostasis ensured before closure, because a hematoma would be contaminated with tumor cells Drain should exit in line with the incision so that the drain track also can be easily excised en bloc with the tumor
  21. Complications : infection , bleeding/hematoma , pathological fracture , tumor contamination and seeding FNAC : 90% accurate in dx malignancy Core needle biopsy : accuracy 84-98% Excisional biopsy : <3cm , sc mass , unlikely malignant – osteoid osteoma , osteochondroma Painful lesion in proximal fibula and distal ulna
  22. Large opacification on medial thigh separate from femur Osteopetrosis – Marble bone disease- confused with sclerotic metastates from breasr and prostate ca , Pagets disease Osteopoikilosis- AD sclerosing bone dysplasia formation of multiple bone island Melorheostosis- mesenchymal dysplasia – widening and sclerosing of cortices in sclerotomal distribution
  23. Adjuvant chemotherapy refers to chemotherapy administered postoperatively to treat presumed micrometastases. Neoadjuvant chemotherapy refers to chemotherapy administered before surgical resection of the primary tumor