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    Bone tumor - Well definedHenk Jan van der Woude and Robin Smithuis

    Radiology department of the Onze Lieve Vrouwe Gasthuis, Amsterdam and the Rijnland hospital,

    Leiderdorp, the Netherlands

    Publicationdate January 1, 2011

    In the article Bone Tumors - Differen-

    tial diagnosis we discuss a systematicapproach to the differential diagnosisof bone tumors and tumor-like le-

    sions.In this article we wi ll discuss the dif-ferential diagnosis of well-definedosteolytic bone tumors and tu-mor-like lesions.

    Abbreviations used:

    ABC = Aneurysmal bone cystCMF = Chondromyxoid fibroma

    EG = Eosinophilic GranulomaGCT = Giant cell tumourFD = Fibrous dysplasia

    HPT = Hyperparathyroidismwith Brown tumorNOF = Non Ossifying Fibroma

    SBC = Simple Bone Cyst

    by Henk Jan van der Woude andRobin Smithuis

    Introduction

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    On the left the most commonwell-defined bone tumors and tu-mor-like lesions.

    These lesions are sometimes referredto as benign cystic lesions, which is amisnomer since most of them are not

    cystic, except for SBC and ABC.It is true that in patients under 30

    years a well-defined border meansthat we are dealing with a benign le-sion, but in patients over 40 years

    metastases and multiple myelomahave to be included in the differentialdiagnosis.

    On the left a table with well-definedosteolytic bone tumors and tu-mor-like lesions in differentage-groups.

    Notice the following:

    In patients In patients > 40

    years metastases and multiple

    myeloma are by far the mostcommon well-defined

    osteolytic bone tumors.Patients with Brown tumor inhyperparathyroidism should

    have other signs of HPT or beon dialysis.Differentiation between a

    benign enchondroma and a lowgrade chondrosarcoma can beimpossible based on imagingfindings only.

    Infection is seen in all ages.

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    Fegnomashic

    Most bone tumors present aswell-defined osteolytic lesions,sometimes referred to as 'bubbly le-sions'.

    It is important to have a good differ-ential diagnostic approach to theselesions.You can use the table above, but an-

    other way to look at the differentialdiagnosis of well defined osteolyticbone lesions is to use the mnemonic

    Fegnomashic, which is popularizedby Clyde Helms (1).

    Some prefer to use the mnemonicFogmachines, which is formed by thesame letters, but is a real word.

    Fibrous dysplasia

    Fibrous dysplasia is a benign disor-der characterized by tumor-like pro-liferation of fibro-osseus tissue andcan look like anything.

    FD most commonly presents as along lesion in a long bone.FD is often purely lytic and takes on

    ground-glass look as the matrix cal-cifies.In many cases there is bone expan-

    sion and bone deformity.The ipsilateral proximal femur is in-variably affected when the pelvis is

    involved.When FD in the tibia is considered,adamantinoma should be in the dif-

    ferential diagnosis.

    Discriminator:

    If periosteal reaction or pain ispresent, exclude fibrousdysplasia, unless there is afracture.

    More on Fibrous dysplasia

    Enchondroma

    Enchondroma is a benign cartilagetumor.

    Fibrous dysplasia: various presentations with or without sclerotic margin,

    with groundglass appearance, with calcifications or ossifications

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    Frequently it is a coincidental finding.

    In the phalanges of the hand it fre-

    quently presents with a fracture.It is the most common lesion in the

    phalanges, i.e. a well-defined lyticlesion in the hand is almost alwaysan enchondroma.

    In some locations it can be difficultto differentiate between enchon-

    droma and bone infarct.It is almost impossible to differenti-ate between enchondroma and lowgrade chondrosarcoma based on ra-

    diographic features alone.Ollier's disease is multiple enchon-dromas.

    Maffucci's syndrome is multiple en-chondromas with soft tissue heman-giomas.

    Features that favor the diagnosis of a

    low-grade chondrosarcoma:

    Higher ageSize > 5 cmActivity on bone scan

    Fast enhancement on dynamiccontrast enhanced MR series

    Endosteal scalloping of thecortical bone

    Discriminators :

    Must have calcification except

    in phalanges.No periostitis.

    leftFat suppressed coronal

    PD-image of the knee. Typicalenchondromas in the femurand tibia as seen frequently as

    coincidental finding inMR-examinations.

    middleWell-defined lytic lesion in therib with cortical thinning.right

    Well-defined lytic lesion with asclerotic margin and withoutcalcifications in the end

    phalanx.

    Eosinophilic granuloma

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    EG is a non-neoplastic proliferation

    of histiocytes and is also known asLangerhans cell histiocytosis.It should be included in the differen-

    tial diagnosis of any sclerotic or oste-olytic lesion, either well-defined orill-defined, in patients under the age

    of 30.The diagnosis EG can be excluded in

    age > 30.EG is usually monostotic, but can bepolyostotic.

    left

    Osteolytic lesion arising fromthe neurocranium withassociated soft tissue swelling.

    middleMixed lytic-sclerotic lesion, notwell-defined with solid

    periosteal reaction.right

    Sharply defined osteolyticlesion of the skull. There is no'button sequestrum', which ismore or less pathognomonic.

    Discriminator:

    Must be under age 30.

    Giant cell tumor

    Giant cell tumor is a lesion withmultinucleated giant cells.

    In most cases it is a benign lesion.Malignant GCT is rare and differentia-

    tion between benign or malignantGCT is not possible based on the ra-diographs.

    GCT is also included in the differen-tial diagnosis of an i ll-defined oste-olytic lesion, provided the age and

    the site of the lesion are compatible.

    Discriminators:

    Epiphyses must be closed.Must be an epiphyseal lesion

    and abut the articular surface.Must be well-defined andnon-sclerotic margin.

    Must be eccentric.

    Giant cell tumor in the tibia abuts the articular surface

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    NOF

    NOF is a benign well-defined, solitarylesion due to proliferation of fibroustissue.

    It is the most common bone lesion.NOF is frequently a coincidental find-ing with or without a fracture.

    NOF usually has a sclerotic borderand can be expansile.

    They regress spontaneously withgradual fill in.NOF may occur as a multifocal lesion.

    The radiographic appearance is al-most always typical, and as such ad-ditional imaging and biopsy is not

    warranted.

    Discriminators:

    Must be under age 30.

    No periostitis or pain.

    Osteoblastoma

    Osteoblastoma is a rare solitary, be-nign tumor that produces osteoid andbone.

    Consider osteoblastoma when ABC isin the differential diagnosis of aspine lesion (figure).

    A typical osteoblastoma is larger than2 cm, otherwise it completely resem-

    bles osteoid osteoma.

    Discriminator:

    Mention when ABC ismentioned.

    Metastases

    Metastases are the most commonmalignant bone tumors.Metastases must be included in the

    differential diagnosis of any bone le-sion, whether well-defined or ill-de-fined osteolytic or sclerotic in age >

    40.Bone metastases have a predilectionfor hematopoietic marrow sites:

    spine, pelvis, ribs, cranium and prox-imal long bones: femur, humerus.

    Metastases can be included in the dif-

    NOF: typical presentation as an eccentric, multi-loculated subcortical

    lesion with a central lucency and a scalloped sclerotic margin.

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    ferential diagnosis if a younger pa-tient is known to have a malignancy,like neuroblastoma, rhabdomyosar-

    coma, retinoblastoma.Most common osteolytic metastases:

    kidney, lung, colon and melanoma.Most common osteosclerotic metas-tases: prostate and breast.

    Discriminator:

    Must be over age 40.

    Multiple Myeloma

    Multiple myeloma must be included

    in the differential diagnosis of anylytic bone lesion, whether well-de-

    fined or ill-defined in age > 40.The most common location is in theaxial skeleton (spine, skull, pelvis

    and ribs) and in the diaphysis of longbones (femur and humerus).Most common presentation: multiple

    lytic 'punched out' lesions.Multiple myeloma doe not show anyuptake on bone scan.

    Discriminator:

    Must be over age 40.

    Multiple Myeloma (2)

    Differential diagnosis:

    multiple lesions: metastases.

    solitary lesion: chondrotumor,GCT and lymphoma.

    On the left a CT-image of a patientwith multiple myeloma.

    Notice the numerous osteolytic le-sions and permeative cortical de-struction pattern.In the left sacral wing there is alarger lesion with a high density due

    to replacement of fatty bone marrow

    Multiple Myeloma: multiple lytic lesions in the humerus

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    by myeloma (red arrow).

    Aneurysmal Bone Cyst

    ABC is a solitary expansile well-de-

    fined osteolytic bone lesion, that isfilled with blood.

    It is named aneurysmal because it isexpansile.ABC is thought to be the result of areactive process secondary to trauma

    or increased venous pressure.Sometimes an underlying lesion likeGCT, osteoblastoma or chondroblas-

    toma can be found.ABC can occur almost anywhere inthe skeleton.

    Discriminators:

    Must be under age 30.Must be expansile

    More on ABC

    Solitary Bone Cyst

    Solitary bone cyst, also known asunicameral bone cyst, is a true cyst.

    Many well-defined osteolytic lesionsare often called cystic, but this is a

    misnomer.SBC frequently presents with a frac-ture.Sometimes a fallen fragment is ap-

    preciated.Predilection sites: proximal humerusand femur.

    Usually less expansion comparedwith ABC.Differential diagnosis: ABC, FD when

    cystic.SBC may migrate from metaphysis to

    diaphysis during growth of the bone.

    Discriminators:

    Must be under age 30.Must be centric

    Hyperparathyroidism

    Brown tumors can occur in any bone

    ABC of the proximal fibula: well-defined, expansile osteolytic lesion with

    thin peripheral bone shell

    SBC: well-defined osteolytic lesion without expansion of the proximalmeta-diaphysis of the humerus with pathologic fracture

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    and present as osteolytic lesions withsharp margins.Septa and ridges may be seen.

    Differential diagnosis: ABC, metas-tases and GCT depending on location

    and age.

    On the left a patient who had a ne-

    frectomy for renal cell carcinoma andwho was on dialysis.

    Multiple well-defined osteolytic le-sions were found on a follow up CTscan.

    The differential diagnosis includedmetastases and Brown tumors in hy-perparathyroidism.

    Biopsy revealed Brown tumor.

    Discriminators:

    Must have other signs of HPT.

    Infection

    Infection or osteomyelitis is the greatmimicker of bone tumors.

    It has a broad spectrum of radio-graphic features and occurs at anyage and has no typical location.

    In the chronic stage it can mimic abenign bone tumor (Brodies ab-

    scess).In the acute stage it can mimic a ma-lignant bone tumor with ill-defined

    margins, cortical destruction and anaggressive type of periostitis.Only when there is a thick solid pe-

    riosteal reaction we can recognize thenon-malignant underlying process.

    Discriminators:

    None.

    Chondroblastoma

    The patella, carpal and tarsal bonescan be regarded as epiphysis con-ceirning the differential diagnosis.

    On the left a chondroblastoma lo-cated in the patella.

    Discriminators :

    must be under age 30.

    Osteomyelitis

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    must be in the epiphysis.

    Chondromyxoid Fibroma

    Chondromyxoid Fibroma is a rare le-sion.

    CMF resembles NOF.Preferential sites: proximal tibia andfoot.

    Although the name suggests that CMFis a chondroid lesion, calcifications

    are usually not seen.

    On the left images of a CMF.There is an eccentric osteolytic lesionin the metaphysis of the proximal

    tibia.On the inner side there is a scleroticmargin.

    On the outer side there is a regularcortical destruction with peripheralbone layer.

    The MR also shows a sclerotic marginwith low signal intensity.

    Discriminators :

    Mention when an NOF ismentioned.

    On the left a summary of things tolook for in well-defined osteolytic le-

    sions.

    Chondroblastoma: notice extensive edema (middle) and fluid-fluid level

    due to secondary ABC.

    Chondromyxoid Fibroma

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    Fundamentals of Skeletal Radiology, second editionby Clyde A. HelmsW. B. Saunders company 1995

    1.

    Discriminative features of well-defined osteolytic lesions

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