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Benign bone tumors an approach
- 1. Dr . RafeevudhDharaja BENIGN BONE TUMORS – AN APPROACH الرحيم الرحمن هللا بسم
- 2. Bone-forming tumors • Osteoidosteoma • Osteoblastoma Cartilage-forming tumors • Osteochondroma • Chondroma • Chondroblastoma • Chondromyxoid fibroma Fibrous lesions • Fibrous dysplasia • Ossifying fibroma • Nonossifying fibroma Cystic and vascular lesions • Unicameral bone cyst • Aneurysmal bone cyst
- 3. CHARACTERISTICS OF BENIGN LESIONS •Well-defined or sclerotic border • Sharp zone of transition • Small size or multiple lesions • Confinement by natural barriers (eg, growth plate, cortex) • Lack of destruction of the cortex • Lack of extension into the soft tissue
- 4. APPROACH Age at Presentation •Primary bone tumours are rare below the age of 5 years and over the age of 40 years. • Osteoid osteoma • Osteobastoma • Enchondroma • Chondroblastoma- teenage • Chonromyxoid fibroma • ABC • FD – before 30 yrs •
- 5. • Solitary ormultiple? • Which bone affected? e.g. long bone vs. spine • Where is the lesion in the bone? • Well-defined margins? • Cortical destruction? • Bony reaction? • Central calcification? • Look also at the soft tissues. • Enchondromatosis • FD • Adamantinoma KEY QUESTIONS TO ASK OURSELVES • Posterior elements of spine - Osteoblatoma- • Metacarpals and phalanges - Enchonromatosis - • Calcaneum - Interosseous lipoma- • Humerus - Simple bone cyst • Tibial anterior cortex- adamantinoma
- 6. • Epiphysis –chondroblastoma • Metaphysis – CMF, ABC • Diaphsis – LCH, SBC • Medullary cavity – enchondroma CMF, FD • Surface of cortex - periosteal chondroma • Cortex- fibrous cortical defect
- 7. • Solitary ormultiple? • What type of bone, e.g. long bone vs. spine vs. ribs? • Where is the lesion in the bone? • Well-defined margins? • Cortical destruction? • Bony reaction? • Central calcification? • Look also at the soft tissues.
- 8. Lesion margin • Inbenign and low-grade malignant neoplasms, this margin is sharp - geographical; Type 1 Type 1A has a rim of sclerosis between the lesion and the host bone Type 1B is a very well defined lytic lesion but with no marginal sclerosis Type 1C has a slightly less sharp, nonsclerotic margin
- 9. Type 2 -moth-eaten destruction, characterised by multiple lucent areas measuring 2–5 mm in diameter separated by bone which has yet to be destroyed Moth-eaten- caused by the coalescence of multiple small lytic areas. Type 3 - is permeative destruction, which is the most aggressive pattern - composed of multiple coalescing small ill- defined lesions of 1 mm or less in diameter with a zone of transition of several centimetres AP radiograph of the ilium reveals the classic moth-hole appearance (arrows) of moth-eaten destruction AP radiograph of the ilium shows the typical diffuse, ill-defined appearance of permeative destruction.
- 10. • Solitary ormultiple? • What type of bone, e.g. long bone vs. spine vs. ribs? • Where is the lesion in the bone? • Well-defined margins? • Trabeculated? • What type of matrix ? • Cortical destruction? • Bony reaction? • Central calcification? • Look also at the soft tissues.
- 11. Matrix Mineralisation • Chondralcalcifications are typically linear, curvilinear, ring-like, punctate or nodular • Osseous mineralisation is cloudlike and poorly defined • Diffuse matrix mineralisation in benign fibrous tumours produces the characteristic ‘ground-glass’ appearance Typical punctate chondral calcification in grade 11 chondrosarcoma ‘cloud-like’ osseous mineralisation in patient with osteoblastic osteosarcoma Fibrous dysplasia showing typical ‘ground-glass’ mineralisation
- 12. • Solitary ormultiple? • What type of bone, e.g. long bone vs. spine vs. ribs? • Where is the lesion in the bone? • Well-defined margins? • Trabeculated? • Cortical destruction? • Bony reaction? • Central calcification? • Look also at the soft tissues. • GCT • CMF • ABC • NOF
- 13. GCT • Solitary ormultiple? • What type of bone, e.g. long bone vs. spine vs. ribs? • Where is the lesion in the bone? • Well-defined margins? • Cortical destruction? • Bony reaction? • Central calcification? • Look also at the soft tissues.
- 14. Periosteal Reaction • Solidperiosteal reaction - slow rate of growth but not necessarily a benign tumour- OO, chondroblastoma • Laminated periosteal reaction - subperiosteal extension of tumour, infection or haematoma. Lesions demonstrating periodic growth may show a multi-laminated pattern
- 15. • Solitary ormultiple? • What type of bone, e.g. long bone vs. spine vs. ribs? • Where is the lesion in the bone? • Well-defined margins? • Cortical destruction? • Bony reaction? • Central calcification/ lytic nidus • soft tissue reaction? • Osteoid osteoma • osteoblastoma • Chondroblastoma
- 16. PLAIN RADIOGRAPHS • Bestinitial modality • Should include views in 2 planes • 80 – 90 % of cases can be diagnosed • Advanced imaging should not be necessary for clearly benign lesions In other places FOR BONE LESIONS
- 17. Further imaging essentialif malignancy a possibility: • Bone scan will identify other lesions. • CT identifies bony architecture. • MRI gives invaluable soft tissue and bony information, particularly tumour extent.
- 18. Cartilage tumours Osteochondroma Chondroma Enchondroma Periostealchondromas Multiple chondromatosis Chondroblastoma Chondromyxoid fibroma
- 19. CARTILAGE TUMOURS Osteochondroma Age • Highestincidence is in the second decade. Location • Long bones are commonly affected, especially around the knee (~40%) • Commonest locations - distal femur, proximal humerus, proximal tibia and proximal femur. • The commonest flat bones affected are the ilium and scapula
- 20. • Continuity betweenthe medullary cavity of the lesion and that of the underlying bone is essential for the diagnosis • Cartilage cap is optimally demonstrated on axial proton density-weighted (PDW) or T2W fast spin-echo (FSE) MRI • It should not exceed 2-cm thickness in adults
- 21. • Outgrowth fromthe normal cortex, with which it is continuous • Pedunculated lesions have a long slim neck • Sessile lesions have a broad base from the bone of origin
- 22. Diaphyseal aclasis: • Hereditarymultiple exostoses,HME • Uncommon autosomal dominant disorder • Exostoses may be larger than the solitary variety • The metaphyses in this condition are also typically widened and dysplastic
- 23. Complications: • Bursa formation •Neurovascular compromise • Pseudoaneurysm
- 24. ENCHONDROMA • Intramedullary neoplasmcomprising lobules of benign hyaline cartilage • Enchondromas affect the tubular bones of the hands and feet in 40–65% of cases • Proximal phalanges (40–50%), • Followed by the metacarpals (15–30%) and • Middle phalanges (20–30%). Patients present due to • Increasing size • Pathological fracture • Or incidental finding
- 25. RADIOLOGICAL FEATURES • Mostenchondromas arise centrally in the phalanges and metacarpals • Well-circumscribed, lobular or oval lytic lesions, which may expand the cortex • Chondral-type mineralisationmay be present • Enchondroma protuberans- eccentrically placed enchondroma with associated extraosseous component covered by a thin shell of intact cortical bone Lobular, mildly expansile lesion with typical chondral matrix mineralisation Eccentrically placed enchondroma of the fourth toe proximal phalanx (arrowhead) with an associated extraosseous component covered by a thin cortical shell
- 26. Large enchondroma ora grade 1 chondrosarcoma?? • Lesion size above 5–6 cm and deep endosteal scalloping are suggestive of chondrosarcoma Low-grade chondral tumours- MRI features • Lobular margin with intermediate T1-weighted signal intensity • T2-weighted-hyperintensity without surrounding reactive oedema
- 27. ENCHONDROMATOSIS Multiple enchondromas, withexpansion of the proximal right femur
- 28. Maffucci’s Syndrome • Multipleenchondromas and soft-tissue haemangiomas
- 29. CHONDROBLASTOMA • Between theages of 5 and 25 years (mean age ~20 years). Location • Around knee • Proximal humerus • Proximal femur • Eccentrically in the epiphysis (40%), but with partial closure of the growth plate it usually extends into the metaphysis (55%) • Commonest tumour of the patella • Presents as monoarthropathy, since it is typically located in the epiphysis of a long bone,
- 30. Radiological Features • Lobularwith a fine sclerotic margin, measuring 1–4 cm in size • Matrix mineralisation is demonstrated in ~30% • Linear metaphyseal periosteal reaction is present in almost 60% of long bone cases
- 31. • MRI shows intermediateT1W SI • Variable SI on T2W images • Associated marrow and soft-tissue oedema and reactive joint effusion
- 32. • Rarely associatedwith metastases (especially to the lung) • Rare variant termed ‘aggressive’ (atypical) chondroblastoma, associated with cortical destruction and soft-tissue extension
- 33. CHONDROMYXOID FIBROMA • Between10 and 30 years of age (mean age 25 years). • Long bones -60% of cases, • 40% arising in the flat bones (ilium 10%) or • Small tubular bones of the hands and feet (17%). 25% of all chondromyxoid fibromas- upper third of the tibia
- 34. • Diagnosis –mainly depends on location • In the proximal tibia eccentric, lobular bubbly lesion with a sclerotic margin RADIOLOGICAL FEATURES Eccentric lytic lesion with a sclerotic margin and expansion of the anterior cortex.
- 35. Osteogenic tumours Osteoidosteoma Osteoblastoma
- 36. OSTEOGENIC TUMOURS Osteoid Osteoma(OO) • Characteristic clinical picture of night pain relieved by aspirin • Second and third decades of life • Occurs anywhere in appendicular skeleton • Most common in the appendicular skeleton, with over 50% located in the diaphysis or metaphysis of the tibia or femur
- 37. • The characteristicfeature of OO is the nidus, which may appear lytic, sclerotic or most commonly of mixed density depending upon the degree of central mineralisation. • Nidus measures up to 15 mm in diameter and is commonly surrounded by a region of reactive medullary sclerosis and solid periosteal reaction.
- 38. • CT demonstratesthe classical features of a round or oval soft- tissue density nidus, which commonly shows central dense mineralisation • Vascular groove sign - thin, serpentine channels in the thickened bone surrounding the nidus
- 39. MR- heterogenously low– intermediateSI on both T1W and T2W images enhancing strongly with contrast Edema-like marrow and soft- tissue SI is almost invariably seen adjacent to the nidus.
- 40. • Intra-articular lesionsare most commonly seen in the hip • Present with local osteopenia due to disuse • Soft-tissue oedema-like changes and a joint effusion
- 41. • In theankle and foot - subperiosteal region of the talar neck is a classical site
- 42. OSTEOBLASTOMA • Histological similaritiesto OO and is differentiated primarily by its size, greater than 1.5 cm in diameter. • More aggressive growth pattern with potential for extraosseous extension, and does not resolve spontaneously. • Under the age of 30 years • Common location is the posterior column of the spine • Tumors in the spine may be difficult to identify on plain radiographs • Humerus is the commonest location in the appendicular skeleton • The lesion arises in the medullary cavity
- 43. RADIOLOGICAL FEATURES • Predominantlylytic lesions measuring over 2 cm in diameter • Larger lesions showing a greater degree of matrix mineralisation • CT – occult calcification, which can be punctate, nodular or generalised • Larger lesions may result in bone expansion • MRI - low–intermediate SI lesion with associated reactive changes as seen with OO, but of a lesser intensity Large mixed lytic-sclerotic lesion in the medullary cavity with associated periosteal thickening
- 44. Fibrogenic tumours Desmoplasticfibroma Fibrohistiocytic tumour Benign fibrous hystiocytoma Fibrous cortical defect Non ossifying fibroma
- 45. FIBROGENIC TUMOURS Desmoplastic Fibroma •Rare, locally aggressive benign neoplasm • Similar histological features to soft-tissue fibromatosis. • Between 10 and 30 years of age (mean age 21 years) • Usually arises in the metaphyseal region of long bones (femur, humerus, tibia and radius constitute 56% of cases), the mandible (26%) and ilium (14%) • Rarely associated with fibrous dysplasia
- 46. RADIOLOGICAL FEATURES • Mostlesions are metadiaphyseal and arise as either subperiosteal or intraosseous tumours. • Many are large at presentation (over 5 cm in diameter) • Two patterns are seen: an ill-defined moth-eaten or permeative lesion an expanding, trabeculated lesion
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- 48. FIBROHISTIOCYTIC TUMOURS • Fibrouscortical defect, • Non-ossifying fibroma and • Benign fibrous histiocytoma Identical histological appearances Different in their clinical and radiological features.
- 49. • As anincidental finding, in the distal femoral and proximal tibial metaphyses • Cortically based lytic lesion commonly with a thin sclerotic margin • Consolidates/fades with time FIBROUS CORTICAL DEFECT
- 50. • Commonly identified incidentally •May present with pathological fracture when large enough • In the second decade of life • Majority involve the lower limbs, particularly the tibia and distal end of the femur NON-OSSIFYING FIBROMA Lobular lesion (arrows) expanding the distal fibular metadiaphysis
- 51. Axial PDW FSEand coronal T2W FSE MR images showing a lobular lesion (arrows) containing prominent areas of hypointensity due to its fibrous nature.
- 52. Jaffe–Campanacci syndrome • Multiple(usually unilateral) NOFs with café-au-lait spots Radiological Features • Metaphyseal or diametaphyseal and essentially intracortical • Lobular appearance is classical • Lesion usually enlarging into the medullary cavity. • Long axis in the line of the bone • When NOF arises in a slim bone such as the fibula, it crosses the shaft readily and its characteristic intracortical origin is less obvious • Low–intermediate SI on T1W and PDW images and enhances with contrast
- 53. BENIGN FIBROUS HISTIOCYTOMA • Uncommonlesion occurring in an older age group • Between 20 and 50 years, with a mean age in the third decade Radiological Features • Lesion resembles a giant cell tumour • Eccentric subarticular location, but with a well- defined sclerotic margin indicating slower growth
- 54. Giant cell tumourGiant cell tumour Vascular tumours Haemangioma Smooth muscle tumours Leiomyoma Lipogenic tumours Lipoma Neural tumours Neurilemmoma
- 55. GIANT CELL TUMOUR •Aggressive benign neoplasm • Between 18 and 45 years of age • Always occurs in a subarticular or subcortical region • Adjacent to a fused apophysis of a long bone - with the knee (distal femur/proximal tibia—55%), distal radius (10%) and proximal humerus (6%) being the commonest sites.
- 56. RADIOLOGICAL FEATURES • Subarticular,eccentric, lytic lesion with a geographic, non- sclerotic margin • Usually measures 5–7 cm in size. • Apparent trabeculation and cortical expansion are common features • Cortical destruction with extraosseous extension may occur in up to 50% Distal radial subarticular lytic lesion (arrows) with internal trabeculation Eccentric, subarticular lytic lesion of the proximal tibia with a poorly defined margin (arrows) and destruction of the lateral cortex.
- 57. • Iso- orhypointense on T1W Images heterogeneous hyperintensity on STIR • Areas of hyperintensity on T1W indicate the presence of subacute haemorrhage • Profound hypointensity on T2W images due to the deposition of haemosiderin from chronic recurrent haemorrhage Sagittal T1W
- 58. Important DDs • Lyticosteosarcoma • In older patients, malignant fibrous histiocytoma or • Subarticular lytic metastasis, particularly from a primary renal tumour.
- 59. VASCULAR TUMOURS Haemangioma Haemangiomas areclassified histologically as • Capillary, • Cavernous, • Arteriovenous or • Venous • Osseous capillary haemangiomas most commonly affect the vertebral body • Osseous cavernous haemangiomas affect the skull vault • Involvement of the appendicular skeleton is relatively rare
- 60. RADIOLOGICAL FEATURES • Fineor coarse vertical trabeculation is seen involving the epiphyses and metaphyses of long bones, with the direction of the linear striations running along the axis of the bone • Bone expansion and extraosseous extension are also recognised features • Scintigraphy typically shows triple-phase uptake due to the vascular nature of the lesion. Expansile lytic lesion (arrows) containing coarse trabeculation.
- 61. • CT demonstratesthe thickened trabeculae as dense ‘dots’ within a fatty matrix • MRI - intermediate SI on T1W and hyperintense on T2W Poorly defined lytic lesion containing multiple, dense thickened trabeculae
- 62. • Intraosseous Lipoma •Parosteal Lipoma Intraosseous Lipoma • Arises in the medulla and produces expansion, • Predilection for the calcaneus and femur • CT and MRI demostrates fatty nature of the matrix LIPOGENIC TUMOURS well-defined lytic lesion (arrows) in the calcaneal body
- 63. Parosteal Lipoma • Rarelesion- most frequently encountered around the proximal radius • May result in pressure erosion of the bone and the formation of circumferential juxtacortical new bone. Axial CT of the elbow showing a fatty mass (arrows) arising in association with a parosteal bony lesion (arrowhead) from the proximal radius
- 64. Miscellaneous lesions Aneurysmalbone cyst Bone cyst Fibrous dysplasia Osteofibrous dysplasia Langerhans cell histiocytosis (eosinophilic granuloma) Erdheim–Chester disease Chest wall hamartoma
- 65. ANEURYSMAL BONE CYST •Between 5 and 20 years of age • Long bones (>50% of cases) and spine (20% of cases) are most common • Involvement of flat bones is most common in the pelvis. • Secondary ABC change can develop in non-ossifying fibroma, chondroblastoma, giant cell tumour, fibrous dysplasia, osteoblastoma and osteosarcoma.
- 66. RADIOLOGICAL FEATURES • Classicallesion – purely lytic, expansile intramedullary lesion in the metaphysis of a long bone extending to the growth plate which may be centrally or, more commonly eccentrically placed • A thin ‘egg-shell’ covering of expanded cortex is often identified Mildly expansile lytic lesion with a thin sclerotic margin (arrows) located eccentrically within the proximal tibial metaphysis Expansile lesion with a thin sclerotic margin (arrows) arising from the surface of the proximal
- 67. • Heterogeneous intermediate SIon T1W images with internal hypointense internal septa may be seen, which may enhance following administration of gadolinium contrast medium • T2 or PDW images almost invariably demonstrate multiple FFLs • Most important differential diagnosis of ABC is telangiectatic osteosarcoma.
- 68. • Between theages of 5 and 15 years • Presentation with pathological fracture is classical, especially with humeral lesions. Location • Proximal humerus – most common • Proximal femur – next common Other sites in adults • Calcaneus and • Posterior iiac blade SIMPLE BONE CYST
- 69. Radiological Features • Initially,SBCs are located in the proximal metaphysis of the humerus or femur and progress toward the diaphysis with skeletal growth, eventually reaching the middiaphysis • Usually lies centrally in the shaft, expanding the bone symmetrically and thinning the cortex. • The lesion is typically 6–8 cm in size. • Apparent trabeculation is common, but periosteal reaction is not seen without fracture, • Fracture may result in fragment of cortex penetrating the cyst lining, resulting in the fallen fragment sign
- 70. MRI • T1W images- low-to-intermediate SI • Marked hyperintensity on T2W or STIR images • These appearances are altered by the presence of fracture, in which case haemorrhage may result in the presence of FFLs and pericystic oedema Major differential diagnosis • ABC and • Fibrous dysplasia.
- 71. • Usually painless •Usually before the age of 30 years • Commonest sites – monostotic FD – ribs, proximal femur , craniofacial bones • FD may be associated with a variety of syndromes • Portions of bone replaced by fibrous connective tissue and poorly formed trabecular bone Monoostotic • 70 to 85% Polyostotic • Invt of 2 bones to more than 75% skeleton FIBROUS DYSPLASIA
- 72. McCune–Albright’s syndrome – •Polyostotic FD (typically unilateral) • Ipsilateral café au lait spots and • Endocrine disturbance, most commonly precocious puberty in girls Mazabraud’s syndrome - • FD (most commonly polyostotic) and soft-tissue myxomata.
- 73. Radiological Features • Geographiclesion that may cause bone expansion and deformity with diffuse ground- glass matrix mineralisation • A thick sclerotic margin (‘rind’ sign) is characteristic • Varus deformity of the proximal femur (‘shepherd’s crook’ deformity) is a characteristic late finding.
- 74. • Skeletal scintigraphyis the best technique for identifying polyostotic disease • CT beautifully demonstrates the ground-glass matrix
- 75. MRI - isointenseon T1W intermediate SI or hyperintense on T2W,
- 76. LANGERHANS CELL HISTIOCYTOSIS (LCH) •Spectrum of disorders characterised by the idiopathic proliferation of histiocytes (Langerhans cells), which can involve virtually any organ and present either as focal/multifocal lesions Three forms of the disease • Eosinophilic granuloma, • Letterer–Siwe disease • Hand–Schüller–Christian disease, • Between the ages of 5 and 15 years
- 77. Location • Skull, pelvis,spine, mandible and ribs Radiological Features • Long bone lesions are usually located centrally within the diaphysis (~60%), followed by the metaphysis/ metadiaphysis • Lesions are lytic, showing a fairly aggressive pattern of bone destruction with occasional reactive medullary sclerosis. • A multi-laminated periosteal response is commonly seen, while endosteal scalloping and mild bone expansion are also features Irregular lytic lesion (arrows) with a multi-laminated periosteal response
- 78. BONE ISLAND • Abone island consists of well-differentiated mature bone tissue within the marrow • Also referred to as enostosis • Usually it is seen as a coincidental finding. • In patients with breast- or prostate cancer a bone island can be mistaken for an osteoblastic metastasis. • A bone island normally does not show increased uptake on a bone scan
- 81. Small lytic lesion withinthe mid tibial diaphysis.
- 82.
- 84. MAFUCCIS’S SYNDROME- HEMANGIOMAAND MULTIPLE ENCHONDROMATOSIS
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- 88. POLYOSTOTIC FIBROUS DYSPLASIA •:TYPICAL GROUND GLASS APPEARANCE. significant expansion of the mid-diaphysis of the tibia and fibula, with the characteristic ground glass appearance to the matrix of the fibrous dysplastic lesions.
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- 91. • Expansile lyticlesion with a thin sclerotic margin located centrally in the metadiaphysis of distal left tibia and extending to the physis. There are subtle internal septations
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- 96. ENCHONDRO MA • Lytic geographiclesion with expansion and chondroid matrix (arrows) are seen on this radiograph of the proximal phalanx of the index finger.
- 98. • Distal femurreadily shows the coarse dot-like, popcorn-like mineralization of chondroid matrix.