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Bone tumor - Systematic approach and Differential diagnosis Tsitskari Maria, MD,EBIR Apollonion Private Hospital Bone tumor - Systematic approach The most important determinators in the analysis of a potential bone tumor are: 1 The morphology of the bone lesion on a plain radiograph ◦ Well-defined o...
Bone tumor - Systematic approach and Differential diagnosis Tsitskari Maria, MD,EBIR Apollonion Private Hospital Bone tumor - Systematic approach The most important determinators in the analysis of a potential bone tumor are: 1 The morphology of the bone lesion on a plain radiograph ◦ Well-defined osteolytic ◦ ill-defined osteolytic ◦ Sclerotic 2 The age of the patient The plain radiograph is the most useful examination for differentiating these lesions CT and MRI are only helpful in selected cases Bone tumor - Systematic approach 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 Bone tumor - Systematic approach Bone tumor - Systematic approach well defined lytic lesion Age 0-10 10-20 20-40 40+ EG, SBC NOF, Osteoblast, Fibr. Dysplasia, EG, SBC, ABC, Chondroblast, CMF Giant CT, Enchondroma, Chodrosarcoma(low grade, HPT- Brown tutor Osteoblastoma Metastases, Myeloma, Geode all ages infection Bone tumor - Systematic approach ill defined lytic lesion Age 0-10 10-20 20-40 40+ all ages EG, Ewing Osteosarcoma, Leukemia EG, Ewing Osteosarcoma, Giant CT Metastases, Myeloma, Chondrosarcoma (high grade) infection Bone tumor - Systematic approach sclerotic lesion Age 0-10 10-20 20-40 Osteosarcoma, Osteosarcoma, Fibr. dysplasia,EG, Osteoid osteoma, Osteoblastoma Enchondroma, Osteoma Bone Island, Parosteal Osteosarcoma, Healed Lesions: NOF, EG,SBC,ABC,Chondroblast 40+ all ages Metastases, Bone island infection Bone tumor - Systematic approach zone of transition In order to classify osteolytic lesions as well-defined or ill-defined, we need to look at the zone of transition between the lesion and the adjacent normal bone The zone of transition is the most reliable indicator in determining whether an osteolytic lesion is benign or malignant The zone of transition only applies to osteolytic lesions since sclerotic lesions usually have a narrow transition zone Small zone of transition - sharp, well-defined border , a sign of slow growth - 40 years consider also plasmacytoma, metastasis Wide zone of transition-ill-defined border, sign of aggressive growth - Ddg, infections and eosinophilic granuloma. two tumor-like lesions which may mimic a malignancy Bone tumor - Systematic approach narrow zone of transition NOF SBC ABC Bone tumor - Systematic approach wide zone of transition Bone tumor - Systematic approach periosteal reaction A periosteal reaction is a non-specific reaction and will occur whenever the periosteum is irritated by a malignant tumor, benign tumor, infection or trauma Two patterns of periosteal reaction: a benign and an aggressive type The benign type: benign tumors , following trauma An aggressive type: malignant tumors, in benign lesions with aggressive behavior, such as infections and eosinophilic granuloma. Bone tumor - Systematic approach benign periosteal reaction A benign type of periosteal reaction is a thick, wavy and uniform callus formation resulting from chronic irritation. Malignant lesions never cause a benign periosteal reaction Ost. Osteoma Bone tumor - Systematic approach Aggressive periosteal reaction This type of periostitis is multilayered, lamellated or demonstrates bone formation perpendicular to the cortical bone. It may be spiculated and interrupted - sometimes there is a Codman's triangle. A Codman's triangle refers to an elevation of the periosteum away from the cortex, forming an angle where the elevated periosteum and bone come together. Osteosarcoma Ewing Sarcoma Infection Bone tumor - Systematic approach Cortical destruction Cortical destruction is a common finding, and not very useful in distinguishing between malignant and benign lesions. Complete destruction may be seen in high-grade malignant lesions, but also in locally aggressive benign lesions like EG and osteomyelitis. More uniform cortical bone destruction can be found in benign and low-grade malignant lesions. Endosteal scalloping of the cortical bone can be seen in benign lesions like FD and low-grade chondrosarcoma Osteosarcoma Ewing Sarcoma Bone tumor - Systematic approach Cortical destruction Ballooning is a special type of cortical destruction. In ballooning the destruction of endosteal cortical bone and the addition of new bone on the outside occur at the same rate, resulting in expansion. This 'neocortex' can be smooth and uninterrupted, but may also be focally interrupted in more aggressive lesions like GCT. Chondromyxoid myxoma GCT Bone tumor - Systematic approach Cortical destruction Ballooning is a special type of cortical destruction. In ballooning the destruction of endosteal cortical bone and the addition of new bone on the outside occur at the same rate, resulting in expansion. This 'neocortex' can be smooth and uninterrupted, but may also be focally interrupted in more aggressive lesions like GCT. Chondromyxoid myxoma GCT Bone tumor - Systematic approach Cortical destruction Malignant small round cell tumors (Ewing’s sarcoma, bone lymphoma and small cell osteosarcoma) The cortex may appear almost normal radiographically, while there is permeative growth throughout the Haversian channels These tumors may be accompanied by a large soft tissue mass while there is almost no visible bone destruction The radiograph does not shown any signs of cortical destruction Bone tumor - Systematic approach Location within the skeleton The location of a bone lesion within the skeleton can be a clue in the differential diagnosis. The illustration on the left shows the preferred locations of the most common bone tumors. In some locations, such as in the humerus or around the knee, almost all bone tumors may be found. Bone tumor - Systematic approach Location metaphysis-diaphysis-epiphysis Epiphysis few lesions are located in the epiphysis - In young patients : chondroblastoma, infection - In patients over 20 : giant cell tumor has to be included in the differential diagnosis - In older patients a geode, i.e. degenerative subchondral bone cyst must be added to the differential diagnosis. Metaphysis - NOF, SBC, CMF, Osteosarcoma, Chondrosarcoma, Enchondroma and infections. Diaphysis - Ewing’s sarcoma, SBC, ABC, Enchondroma, Fibrous dysplasia and Osteoblastoma. Bone tumor - Systematic approach Location: centric - eccentric - juxtacortical Centric in long bone SBC, eosinophilic granuloma, fibrous dysplasia, ABC and enchondroma Eccentric in long bone Osteosarcoma, NOF, chondroblastoma, chondromyxoid fibroma, GCT and osteoblastoma SBC NOF SBC Bone tumor - Systematic approach Location: centric - eccentric - juxtacortical Cortical Osteoid osteoma is located within the cortex and needs to be differentiated from osteomyelitis. Juxtacortical Osteochondroma. The cortex must extend into the stalk of the lesion. Parosteal osteosarcoma arises from the periosteum. Osteoid osteoma Subchondral cyst SBC Bone tumor - Systematic approach Matrix cations or mineralization within a bone lesion may be an important clue in the differential diag Two kinds of mineralization - a chondroid matrix in cartilaginous tumors like enchondromas and chondrosarcoma - an osteoid matrix in osseus tumors like osteoid osteomas and osteosarcomas. Chondroid matrix rings-and-arcs, popcorn, focal stippled or flocculent. left: Enchondroma middle: Peripheral chondrosarcoma, arising from an osteochondroma (exostosis) right: Chondrosarcoma of the rib Bone tumor - Systematic approach Matrix cations or mineralization within a bone lesion may be an important clue in the differential diag Osteoid matrix - a trabecular ossification pattern in benign bone-forming lesions - a cloud-like or ill-defined amorphous pattern in osteosarcomas osteosarcoma Osteoid osteoma r - well-defined osteolytic tumors and tumor-l r - well-defined osteolytic tumors and tumor-l Fibrous dysplasia FD most commonly presents as a long lesion in a long bone. FD is often purely lytic and takes on ground-glass look as the matrix calcifies. In many cases there is bone expansion and bone deformity r - well-defined osteolytic tumors and tumor-l Enchondroma Enchondroma is a benign cartilage tumor. Frequently it is a coincidental finding. It is the most common lesion in the phalanges, i.e. a well-defined lytic lesion in the hand is almost always an enchondroma. In the phalanges of the hand it frequently presents with a fracture. It is almost impossible to differentiate between enchondroma and low grade chondrosarcoma Ollier's disease is multiple enchondromas. Maffucci's syndrome is multiple enchondromas with soft tissue hemangiomas. r - well-defined osteolytic tumors and tumor-l Eosinophilic granuloma EG is a non-neoplastic proliferation of histiocytes and is also known as Langerhans cell histiocytosis. It should be included in the differential diagnosis of any sclerotic or osteolytic lesion, either welldefined or ill-defined, in patients under the age of 30 The diagnosis EG can be excluded in age > 30. EG is usually monostotic, but can be polyostotic. i) Osteolytic lesion arising from the neurocranium with associated soft tissue swelling ii) Mixed lytic-sclerotic lesion, not well defined with solid periosteal reaction iii) Sharply defined osteolytic lesion of the skull. There is no ‘button sequestrum', which is more or less pathognomonic. i ii iii r - well-defined osteolytic tumors and tumor-l Giant Cell Tumor Giant cell tumor is a lesion with multinucleated giant cells In most cases it is a benign lesion Malignant GCT is rare and differentiation between benign or malignant GCT is not possible based on the radiographs Discriminators: Epiphyses must be closed Must be an epiphyseal lesion and abut the articular surface Must be well-defined and nonsclerotic margin. Must be eccentric r - well-defined osteolytic tumors and tumor-l NOF NOF is a benign well-defined, solitary lesion It is the most common bone lesion. NOF is frequently a coincidental finding with or without a fracture. NOF usually has a sclerotic border and can be expansile. They regress spontaneously with gradual fill in. Discriminators: Must be under age 30. No periostitis or pain. typical presentation eccentric,multiloculated , subcortical lesion with a central lucency and a scalloped sclerotic margin r - well-defined osteolytic tumors and tumor-l Osteoblastoma Osteoblastoma is a rare solitary, benign tumor that produces osteoid and bone Consider osteoblastoma when ABC is in the differential diagnosis of a spine lesion A typical osteoblastoma is larger than 2 cm r - well-defined osteolytic tumors and tumor-l Metastases Metastases are the most common malignant bone tumors Metastases must be included in the differential diagnosis of any bone lesion, whether well-defined or illdefined osteolytic or sclerotic in age > 40 Bone metastases have a predilection for hematopoietic marrow sites: spine, pelvis, ribs, cranium and proximal long bones: femur, humerus Most common osteosclerotic metastases: prostate and breast. r - well-defined osteolytic tumors and tumor-l Multiple Myeloma The most common location is in the axial skeleton (spine, skull, pelvis and ribs) and in the diaphysis of long bones (femur and humerus). Most common presentation: multiple lytic 'punched out' lesions Multiple myeloma doe not show any uptake on bone scan. Discriminator: - Must be over age 40. r - well-defined osteolytic tumors and tumor-l Multiple Myeloma Differential diagnosis: multiple lesions: metastases. solitary lesion: chondrotumor, GCT and lymphoma. - numerous osteolytic lesions and permeative cortical destruction pattern - in the left sacral wing there is a larger lesion with a high density due to replacement of fatty bone marrow by myeloma r - well-defined osteolytic tumors and tumor-l Aneurysmal Bone Cyst ABC is a solitary expansile well-defined osteolytic bone lesion, that is filled with blood Sometimes an underlying lesion like GCT, osteoblastoma or chondroblastoma can be found ABC can occur almost anywhere in the skeleton Under 30 r - well-defined osteolytic tumors and tumor-l Solitary Bone Cyst SBC is a true cystSBC frequently presents with a fracture. Sometimes a fallen fragment is appreciated Predilection sites: proximal humerus and femur Differential diagnosis: ABC, FD when cystic. r - well-defined osteolytic tumors and tumor-l Hyperparathyroidism Brown tumors can occur in any bone and present as osteolytic lesions with sharp margins Septa and ridges may be seen Differential diagnosis: ABC, metastases and GCT depending on location and age r - well-defined osteolytic tumors and tumor-l Infection Infection or osteomyelitis is the great mimicker of bone tumors It has a broad spectrum of radiographic features and occurs at any age and has no typical location. In the chronic stage it can mimic a benign bone tumor (Brodies abscess) In the acute stage it can mimic a malignant bone tumor with ill-defined margins, cortical destruction and an aggressive type of periostitis - an eccentric ill-defined lesion seen on both sides of the physeal plate in the proximal tibia - coronal T1-weighted MR image reveals a well-defined epi-metaphyseal lesion There is a dark peripheral zone of reactive sclerosis, and extensive edema with low signal Intensity in the metaphysis r - well-defined osteolytic tumors and tumor-l Infection ill-defined osteolytic lesion in the proximal metaphysis of the tibia with extensive reactive sclerosis and solid periosteal reaction r - well-defined osteolytic tumors and tumor-l Chondroblastoma Epiphyseal lesion The patella, carpal and tarsal bones can be regarded as epiphysis concerning the differential diagnosis. Must be under age 30. extensive edema and fluid fluid level due to 2ary ABC r - well-defined osteolytic tumors and tumor-l Chondromyxoid fibroma Chondromyxoid Fibroma is a rare lesion. CMF resembles NOF. Preferential sites: proximal tibia and foot. There is an eccentric osteolytic lesion in the metaphysis of the proximal tibia On the inner side there is a sclerotic margin On the outer side there is a regular cortical destruction with peripheral bone layer The MR also shows a sclerotic margin with low signal intensity. mor -ill-defined osteolytic tumors and tumor-li Chondrosarcoma Most commonly presents as a well-defined lesion, but uncommonly it can be encountered as an ill-defined lytic lesion It may have calcifications ill-defined osteolytic lesion with endosteal scalloping there are cloud-like calcifications indicating a chondroid matrix mor -ill-defined osteolytic tumors and tumor-li Eosinophilic Granuloma EG is like osteomyelitis a great mimicker of benign and malignant bone tutors Must be included in the differential diagnosis of almost any ill-defined osteolytic bone lesion in patients under the age of 30 May also present as a well-defined lesion. mor -ill-defined osteolytic tumors and tumor-li Ewing Sarcoma Most common presentation: ill-defined osteolytic lesion with multiple small holes in the diaphysis of a long bone in a child with a large soft tissue mass Presentation with pain, mass, fever, anemia and leukocytosis Most common location: femur, iliac bone, fibula, rib, tibia Differential diagnosis: Osteosarcoma, lymphoma, infection and EG Frequently aggressive type of periosteal reaction, but never a benign type Ill defined zone of transition aggressive type of periosteal reaction mor -ill-defined osteolytic tumors and tumor-li Ewing Sarcoma There is a permeative destruction pattern with irregular cortical destruction aggressive periosteal reaction sunburst type mor -ill-defined osteolytic tumors and tumor-li Giant Cell Tumor Giant cell tumor is a benign lesion, but can be locally aggressive In many cases the margin is ill-defined These tumors often thin the cortex and may expand into the soft tissues surrounding the bone Giant cell tumor of the distal radius with illdefined margins, destruction of the subchondral bone plate and extension towards the soft tissues On the right a giant cell tumor in the proximal tibia with somewhat better defined margin and non-interrupted cortical bone mor -ill-defined osteolytic tumors and tumor-li Metastases May present as well-defined osteolytic, ill-defined osteolytic and also as sclerotic bone lesion Majority of osteolytic metastases originate from breast, lung, kidney, colon, melanoma and thyroid mor -ill-defined osteolytic tumors and tumor-li Osteosarcoma Hallmark of osteosarcoma is the production of bony matrix, which is reflected by the sclerosis seen on the radiograph Usually typical malignant features - permeative-motheaten pattern of destruction - irregular cortical destruction - aggressive (interrupted) periosteal reaction Occasionally an osteosarcoma may present as a lytic or mixed lytic-sclerotic lesion. - a mixed osteolytic and sclerotic lesion in the proximal humerus with irregular cortical destruction - there is an aggressive periosteal reaction and a soft tissue mass umor - sclerotic bone tumors and tumor-like l I VINDICATE : which means 'I clear myself from accusation umor - sclerotic bone tumors and tumor-like l Bone infarct The term bone infarction is used for osteonecrosis within the diaphysis or metaphysis If the osteonecrosis is located in the epiphysis, the term avascular osteonecrosis is used Typical presentation: central lesion in metaphysis or diaphysis with a well defined serpentiginous border. Causes: corticosteroid use, sickle cell disease, trauma, Gaucher's disease, renal transplantation umor - sclerotic bone tumors and tumor-like l Bone infarct On MR imaging bone infarcts are characterized by irregulair serpentiginous margins with low signal intensity on both T1 and T2 WI and with intermediate to high fat signal in the center part Enhancement after i.v. Gadolinium is usually minimal or absent At the periphery of the infarct a zone of relative high signal intensity on T2WI may be found umor - sclerotic bone tumors and tumor-like l Bone infarct- avascular necrosis umor - sclerotic bone tumors and tumor-like l Bone infarct On MR imaging bone infarcts are characterized by irregulair serpentiginous margins with low signal intensity on both T1 and T2 WI and with intermediate to high fat signal in the center part Enhancement after i.v. Gadolinium is usually minimal or absent At the periphery of the infarct a zone of relative high signal intensity on T2WI may be found umor - sclerotic bone tumors and tumor-like l Bone island Benign lesion consisting of well-differentiated mature bone tissue within the medullary cavity Also known as enostosis Usually round or oval Can be found in any bone. Occasionally slowly enlargement can be seen Can be large at presentation. Bone scan shows no high activity, opposed to low-grade intraosseous osteosarcoma umor - sclerotic bone tumors and tumor-like l Chondroblastoma Typical presentation: well-defined osteolytic lesion in tarsal bone, patella or epiphysis of a long bone in a 20-year old with pain and swelling in a joint Abundant edema is almost always present DD: Ganglion cyst, osteomyelitis, GCT, ABC, enchondroma A lesion predominantly calcified Coronal T1W image shows lobulated margins and peripheral low SI due to the calcifications. umor - sclerotic bone tumors and tumor-like l Chondrosarcoma Most common malignant bone tumor, which is almost always low-grade Primary sites of origin: proximal long bones, around knee, pelvis and shoulder girdle, usually central and metaphysical Radiological hallmark: formation of a chondroid (cartilagenous) matrix, which presents as punctuated, stippled or popcorn-like calcifications umor - sclerotic bone tumors and tumor-like l Chondrosarcoma calcified lesion in the proximal tibia without suspicious features Ddg: enchondroma. mixed lytic and sclerotic mass arising from the fifth metacarpal bone there are calcified strands within the soft tissues T2-weighted axial MR image demonstrates high signal intensity of the tumor in the metacarpal bone with extension of a lobulated soft tissue mass. umor - sclerotic bone tumors and tumor-like l Chondrosarcoma umor - sclerotic bone tumors and tumor-like l Melorrheostosis Is a dysplasia of the bone, characterized by apposition of mature bone on the outer or inner surface of cortical bone Usually one bone is involved The epiphysis, metaphysis and diaphysis may be involved. Usually new bone is added to one side of the cortex only Complete envelopment may occur Melorrheostosis of the ulna with the appearance of candle wax Axial T1-weighted MR image shows homogeneous low signal intensity due to the compact bone apposition umor - sclerotic bone tumors and tumor-like l Metastases Sclerotic metastases most commonly originate from prostate and breast cancer and less frequently from lung cancer, lymphoma or carcinoid umor - sclerotic bone tumors and tumor-like l Osteochondroma A bony protrusion covered by a cartilaginous cap Growth of the osteochondroma takes place in the cap Accordingly, growth of osteochondromas is allowed until a patient reaches adulthood and the physeal plates are closed Growth of osteochondromas at adult ages, which is characterized by a thick cartilaginous cap (high SI on T2WI) should raise the suspicion of progression to a peripheral chondrosarcoma umor - sclerotic bone tumors and tumor-like l Osteochondroma Malignant transformation Radiographic features that should raise the suspicion of malignant transformation on plain radiographs or CT include: Growth of osteochondroma in skeletally mature patient Irregular or indistinct surface of lesions focal lucent regions in interior of lesions erosion or destruction of adjacent bone presence of soft tissue mass with scattered or irregular calcifications broad-based osteochondroma with extension of the cortical bone into the stalk of the lesion. thick cartilage cap: lytic peripheral part with subtle calcifications- this part corresponds to a zone of high SI on T2-WI with FS umor - sclerotic bone tumors and tumor-like l Osteoid osteoma Small osteolytic lesion (up to 1.5 cm) with or without central calcification. Surrounded by a prominent zone of reactive sclerosis due to a periosteal and endosteal reaction, which may obscure the central nidus. In juxta-articular localisation, the reactive sclerosis may be absent. Localisation: femur, tibia, hands and feet, spine (arch) umor - sclerotic bone tumors and tumor-like l Osteosarcoma hly malignant formation or reactive sclerosis ay occur in older patients. en or permeative pattern of the transition zone with irregular cortical destruction and an interrupted perioste e appears as tumor elevates periosteum from underlying bone. Cortical soft tissue extension may produce ra umor - sclerotic bone tumors and tumor-like l Osteosarcoma umor - sclerotic bone tumors and tumor-like l Osteosarcoma The sagittal T1WI and Gd-enhanced T1W-image with fatsat show a large tumor mass infiltrating a large portion of the distal femur and extending through the cortex into the soft tissues. umor - sclerotic bone tumors and tumor-like l Paget’s disease of the bone Paget disease is a chronic disorder of unknown origin with increased breakdown of bone and formation of disorganized new bone The most common appearance is the mixed lytic-sclerotic Pathognomonic triad of bone expansion, cortical thickening and trabecular bone thickening in the mixed lytic and sclerotic phase of Paget's disease of right hemipelvis