Bone Tumors STG 2024 Student PDF

Bone Tumors: Systematic Approach SEAN T. GRAMBART DPM FACFAS D, D.ABFAS 1 Objectives Demonstrate knowledge of the basic radiographic changes that accompany benign and malignant bone tumors. Recognize the basic characteristics of the more common bone tumors Demonstrate knowledge of the diagnostic ste...

Bone Tumors: Systematic Approach SEAN T. GRAMBART DPM FACFAS D, D.ABFAS 1 Objectives Demonstrate knowledge of the basic radiographic changes that accompany benign and malignant bone tumors. Recognize the basic characteristics of the more common bone tumors Demonstrate knowledge of the diagnostic steps of bone tumors 2 Primary Goal… Increase understanding of these abnormalities rather than to provide lists for memorization It is better to: Recognize the predilection of different lesions for certain locations and age groups Understand how to analyze the radiographic appearances of these lesions than it is to memorize long lists Aggressive versus Non-Aggressive 3 Clinical Presentation Pain All bone tumors that affect cortical bone carry a risk of pathologic fracture Referral Incidental Finding 4 Imaging Radiographs The most important tool Detection CT Scan Sometimes needed in complex anatomical areas MRI Bone Scans 5 Radiographic Assessment Principles of Assessment None of these rules are absolute, and there are some exceptions At least 50% of trabecular bone needs to be destroyed before the tumor becomes detectable on radiographs Ultimate Goals Short Differential Diagnosis List Identify those tumors that need further management rather than arriving at a specific diagnosis Aggressive versus Non-Aggressive 6 Radiographic Assessment: Detection The bone tumor itself is not visible on radiographs in most instances unless it is mineralized Cortical and surface tumors become obvious earlier than medullary tumors Tumors in the epiphyses and metaphysis are easier to detect than those in the diaphysis Tumor diagnosis can be delayed when located in flat bones, axial skeleton, and the ribs 7 Most Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 8 Most Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 9 AGE Age is the most important clinical factor in differentiating possible bone tumors Differential diagnosis in patients > 30 years must always include metastases and myeloma 10 AGE ABC = Aneurysmal bone cyst CMF = Chondromyxoid fibroma EG = Eosinophilic Granuloma GCT = Giant cell tumour FD = Fibrous dysplasia HPT = Hyperparathyroidism with Brown tumor NOF = Non Ossifying Fibroma SBC = Simple Bone Cyst 11 Most Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 12 LOCATION Specific Bones 13 LOCATION Transverse Plane Central Eccentric GCT, Osteosarcoma, Chondromyxoid Fibroma Cortical Enchondroma Non-Ossifying Fibroma, Osteoid Osteoma Parosteal Osteochondroma, Parosteal Osteosarcoma 14 LOCATION Longitudinal Plane 15 Most Radiographic Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 16 Radiographic Assessment: Zone of Transition The margin of the tumor The transition between normal bone and tumoral tissue as seen on radiographs The most reliable indicator in determining whether an osteolytic lesion is benign or malignant 17 Narrow Zone of Transition Fine point pen /Well-defined Less-Aggressive 18 Wide Zone of Transition Ill-Defined Borders Aggressive 19 Radiographic Assessment: Pattern of Bone Destruction Geographic Non-Geographic Aggressiveness 20 Radiographic Assessment: Pattern of Bone Destruction Geographic IA Well-defined lucency with a sclerotic rim 21 Radiographic Assessment: Pattern of Bone Destruction Geographic IB Well-defined lucency without a sclerotic rim 22 Radiographic Assessment: Pattern of Bone Destruction Geographic IC Ill-defined lytic lesion 23 Radiographic Assessment: Pattern of Bone Destruction Aggressiveness 24 Radiographic Assessment: Pattern of Bone Destruction Moth-Eaten Area of destruction with ragged borders More rapid growth 25 Radiographic Assessment: Pattern of Bone Destruction Permeative Ill-defined lesion with “worm-hole” appearance Spreads through the medullary canal Aggressive malignancy 26 Most Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 27 Radiographic Assessment: Matrix Matrix is the dominant internal extracellular substance of a lesion Most tumors have an osteolytic appearance or NO MATRIX 28 Radiographic Assessment: Matrix Clear Matrix Lesions that are clear or mostly clear 29 Radiographic Assessment: Matrix Patterns of Cartilage Matrix Stippled Flocculent 30 Ring and Arc Enchondroma Chondrosarcoma 31 Radiographic Assessment: Matrix Patterns of Bone Producing Matrix 32 33 Radiographic Assessment: Matrix Patterns of Fibrous Producing Matrix Ground Glass 34 Most Radiographic Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 35 Radiographic Assessment: Cortical Appearance (Periosteum) The periosteum will react to an irritation Tumor Trauma Infection 36 37 Radiographic Assessment: Cortical Appearance (Periosteum) 38 Radiographic Assessment: Cortical Appearance (Periosteum) 39 Radiographic Assessment: Cortical Appearance (Periosteum) 40 41 42 43 44 Radiographic Assessment: Cortical Appearance (Periosteum) 45 Most Important Factors: ALTMCS What is the age of the patient? What is the location of the lesion? How does the transition zone appear? Is there production of a matrix? How does the cortex look? Is there any swelling? 46 Radiographic Assessment: Swelling 47 Aggressive versus Non-Aggressive 48 49 Fibrous Dysplasia Non-neoplastic developmental anomaly of the skeleton characterized by replacement of bone interior by fibro-osseous tissue and occasionally accompanied by brown skin macules and endocrine disorders (triad known as Albright’s syndrome) 2nd to 3rd decades, M>F, 75% have single lesion Ground Glass Appearance 50 Osteoid osteoma F, pain not as severe as osteoid osteoma Large (>2cm) lesion, eccentrically located in metaphysis or shaft, without surrounding reactive bone sclerosis as seen in osteoid osteoma Rarely transforms to osteogenic sarcoma 52 Giant Cell Tumor Known as osteoclastoma in UK Aggressive tumor composed of well vascularized stroma with oval tumor cells and multinucleated tumor giant cells uniformly distributed throughout the lesion Third decade and older, F>M Large, eccentric, oval, radiolucent, destructive, lesion centered in the epiphysis (base of first, heads of 2-5 metatarsals) producing cortical erosion and thinning, and expanding the bone contours without a border of periosteal new bone and sclerosis “Massive in metaphysis” Treatment: Curettage and bone graft; recurrence rate as high as 50% En-bloc excision and graft; recurrence rate 10-15% May metastasize to lungs or transform to fibrosarcoma or osteogenic sarcoma 53 Aneurysmal Bone Cyst Arteriovenous or other vascular anomaly resulting from a local circulatory disturbance in bone 5-20 y/o with presenting symptoms of swelling or pain Eccentrically located in the metaphysis and often with a characteristic subperiosteal “blow-out” appearance On MRI-Mosaic glass on MRI 54 Chondroblastoma Uncommon tumor derived from chondroblasts 10-25 y/o Origin in epiphyseal plate and extends into the epiphysis and may involve the metaphysis-well demarcated, expansile, oval lesion, with a thin sclerotic bony margin-may have amorphous, spotty calcification “chicken-wire matrix” Also consider possibility of osteoblastoma in this age group when viewed on x-ray 55 Chondromyxoid fibroma Originates from cartilage-forming connective tissue characterized by chondroid tissue and intracellular mucin-like substance F Ovoid in shape with long axis parallel to bone with a sclerotic margin and lobulated contour All cartilage tumors has increased signal on T2 and decreased signal on T1 56 Hyperparathyroidism Brown tumors-hemosiderin deposition in bone matrix that is being rapidly turned-over w/o adequate calcification 3rd to 5th decades, females 3:1 over males Subperiosteal bone resorption is hallmark, especially in phalanges-generalized loss of bone density follows, with endosteal resorptionfinally, lightly trabeculated, expanding bone lesions appear (“brown tumors”) Hypercalcemia exists, so soft tissues, especially blood vessels show calcification 57 Non-ossifying Fibroma Starts as a fibrous cortical defect (FCD) in metaphyseal area of bone caused by periosteal fibroblastic proliferation FCD is found in 30-40% of children and is asymptomatic If FCD persists and enlarges by fibroblastic proliferation into the medullary cavity it is termed a nonossifying fibroma Soap-bubble appearance 58 Enchondroma Composed of mature hyaline cartilage in the medullary cavity 3rd to 6th decades of life Central, or slightly eccentric, wellcircumscribed or “bubbly” radiolucent lesion, finely or densely stippled with calcification End of bone with speckled tone Very common in phalanges and metatarsals May transform to chondrosarcoma 59 Osteochondroma Aberrant proliferation of epiphyseal cartilage cells and resulting endochondral ossification, and ceases growth at the time of skeletal maturation F Bony projection arising from the surface of the bone, but whose cortex and medullary canal is continuous with the cortex and medullary canal of the bone from which it is arising, usually a metatarsal Has a cap of hyaline cartilage and projects away from the epiphysis from which it arises 60 Bone Island Area of sclerotic bone due to increased osteblastic activity Not a tumor, but very common in foot, especially calcaneus 61 Eosinophilic Granuloma Destructive histiocytic lesions of bones F Oval, radiolucent lesion located centrally in diaphysis or metaphysis, and surrounded in later phases by border of sclerosis-resembles osteomyelitis Endosteal scalloping Can be associated with extraskeletal histiocytic deposition with can lead to fatality (Hand-Schuller-Christian disease and Letterer-Siwe disease) 62 Solitary bone cyst (Unicameral bone cyst) Cause unknown-may be due to skeletal trauma and intraosseous hemorrhage 3-19 y/o, M>F Central, symmetrical lesion surrounded by a thin rim of bone and located in metaphysis, but not crossing epiphyseal plate-appears to move away from plate or apophysis as growth occurs in plate Almost exclusively in the calcaneus, under the middle facet Best bet is middle facet Fallen fragment sign Piece of bone on the margin of the tumor which breaks off and deposits on the gravity dependent edge of tumor 63 Ewing’s Sarcoma Cellular origin is neuroectodermal cells Second decade; M>F, 2:1 Mottled moth-eaten destructive lesion in diaphysis with periosteal reactive new bone producing “onion-skin” appearance, and often accompanied by a soft tissue mass 1/3 in hindfoot, 2/3 in forefoot-pain, swelling, warmth, fever, increased ESR (mimics osteomyelitis) Highly aggressive-patients without metastasis have survival rate of 88%; those with non-pulmonary and pulmonary metastasis had 10% and 0% survival rate 64 65 Osteogenic Sarcoma (Osteosarcoma) Proliferating malignant spindle-cell stroma produces immature bone F, 2:1 (primary OSA) Matrix and periosteum exhibit all malignant changes previously described OSA develops in places of active growth, such as long bones of knee (primary OSA, 80 % of cases) or at a site of Paget’s disease in older individual (secondary OSA, 20%) Alk Phos markedly increased Treatment: amputation of extremity, with five year survival rate of 20% (death due to pulmonary mets) Current treatment: pre-op chemotherapy, local “en bloc” excision, immediate placing of a prosthesis, and post-op methotrexate-five year survival is 80% 66 Osteogenic Sarcoma (Osteosarcoma) Proliferating malignant spindle-cell stroma produces immature bone F, 2:1 (primary OSA) Matrix and periosteum exhibit all malignant changes previously described OSA develops in places of active growth, such as long bones of knee (primary OSA, 80 % of cases) or at a site of Paget’s disease in older individual (secondary OSA, 20%) Alk Phos markedly increased Treatment: amputation of extremity, with five year survival rate of 20% (death due to pulmonary mets) Current treatment: pre-op chemotherapy, local “en bloc” excision, immediate placing of a prosthesis, and post-op methotrexate-five year survival is 80% 67 Chondrosarcoma Basic neoplastic component is cartilage, without tumor osteoid or bone being formed by the sarcomatous stroma 40+, M>F Large lesion in interior of diaphysis or metaphysis characterized by “bubbly” radiolucencies, granular areas of calcification, “scalloped” regions of destruction of inner cortex, bone enlargement, focal periosteal reaction, possible extension into soft tissue Arises de novo in 75% of cases, by malignant transformation of an enchondroma, or by sarcomatous change in the cartilage cap of an osteochondroma Most common malignant BONE tumor in foot and ankle Treatment: major amputation; five year survival rate is 40% 68 Chondrosarcoma Basic neoplastic component is cartilage, without tumor osteoid or bone being formed by the sarcomatous stroma 40+, M>F Large lesion in interior of diaphysis or metaphysis characterized by “bubbly” radiolucencies, granular areas of calcification, “scalloped” regions of destruction of inner cortex, bone enlargement, focal periosteal reaction, possible extension into soft tissue Arises de novo in 75% of cases, by malignant transformation of an enchondroma, or by sarcomatous change in the cartilage cap of an osteochondroma Most common malignant BONE tumor in foot and ankle Treatment: major amputation; five year survival rate is 40% 69 Metastasis/Myeloma Most probable cause of osteolytic metastases in a child is neuroblastoma In an adult male, CA of lung In an adult female, CA of breast CA of kidney and thyroid Myeloma is most common primary malignant tumor of bone in the body Malignant tumor of plasma cells with lytic bone lesions, monoclonal gammopathy, bone marrow failure, recurrent infections, hypercalcemia, amyloidosis, nephrocalcinosis > 40 y/o 70 71 72 Case Study 73 The Acronym Game 74 Solitary Lytic Lesions of Bone Radiolucent-type lesions Acronym: FOGMACHINES The “go-to” acronym since most lesions are of this type All these lesions start out totally radiolucent, but over time those derived from cartilage or bone will look less radiolucent or more sclerotic as they produce calcifications and bone Sex and age are not reliable demographic features to help diagnose-however, age is very reliable in diagnosing malignancies or metastases and is the only reliable demographic feature you should use 75 Solitary Lytic Lesions of Bone F Fibrous dysplasia O Osteoid osteoma/osteoblastoma G Giant cell tumor M Metastasis, myeloma A Aneurysmal bone cyst C Chondroblastoma, chondromyxoid fibroma H Hyperparathyroidism, hemangioma I Infection N Non-ossifying fibroma E Eosinophilic granuloma, enchondroma S Solitary bone cyst, sarcoidosis 76 “Bubbly lesions of bone” “NFC GAMES” Nonossifying fibroma Fibrous dysplasia Chondrosarcoma Giant cell tumor Aneurysmal bone cyst Metastatic CA Enchondroma/Ewing’s sarcoma Solitary bone cyst 77 Multiple Lucent Bone Lesions H Hyperparathyroidism/hemangioma I Infection F Fibrous dysplasia E Enchondroma/eosinophilic granuloma M Metastasis/myeloma 78 Lytic Lesions in Metaphysis C A G E “Cage On Mud” Chondroblastoma Aneurysmal bone cyst Giant cell tumor Enchondroma, Ewing’s sarcoma O Osteoblastoma, osteosarcoma, osteomyelitis N Non-ossifying fibroma M Malignant fibrous histiocytoma (fibrosarcoma) U Unicameral bone cyst D Desmoid tumor 79 Malignant Lytic Bone Lesions Best classified by age: