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BONE TUMORS BENIGN BONE TUMORS PRESENTOR- UMESH YADAV JR-ORTHO,PGIMS ,ROHTAK UMY

Benign bone tumors

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A overview of individual benign bone tumors... intro, x ray app, diagnosis & management.... all in one

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BONE TUMORS

BENIGN BONE TUMORS

PRESENTOR- UMESH YADAVJR-ORTHO,PGIMS ,ROHTAK

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OVERVIEW• Bone tumors are very diverse in morphology and

biological potential (can be no big deal or rapidly fatal)

• MOST bone tumors are benign lesions• Most benign lesions are seen <30 years of age• A new bone tumor in the elderly is more likely to be

malignant• No bone is safe (though most primaries are in long

bones)• Location in the bone gives important Dx info• More common benign lesions typically present as

incidental findings (non-painful, stable size)• Be cautious with painful lesions and those that grow

relatively fast (over weeks or months)• Pathological fracture can be the first sign of tumor

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• Bone neoplasms are very difficult to diagnose specifically on radiologic testing alone

• So why is radiology important?– Exact location of lesion– Extent of growth/metastasis– Aggressiveness

• Best test for Dx= X-ray• Best test for staging= CT or MRI• Quick shout out to the pathologists– histologic

grade is the most important prognostic feature of bone sarcomas and essential for staging most of the bone tumor types.

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Primary

Bone Tumor

Tumor from Bone Tissue

benignOsteochondroma, Giant cell tumor, Osteoma, Chondroma, Chondroblastoma

malignant

Osteosarcoma, Chondrosarcoma, Fibrosarcoma

Tumor from Bone Affiliated Tissue

benign Osteoangioma, Odontogenic tumor (exp. Adamantinoma)

malignant

Ewing's sarcoma, Reticulum cell sarcoma of bone, Notochordoma, Myeloma

Metastatic Tumor

Carcinoma, Sarcoma, Neuroblastoma

Carcinoma, Sarcoma, Neuroblastoma

Classification of Bone Tumor

Tumor-like lesion Bone cyst, Fibrous dysplasia

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Benign tumors Bone-forming• Osteoid Osteoma• Bone islandCartilage Lesions• Chondroma• OsteochondromaFibrous lesions• Non ossifying fibroma• Cortical desmoid• Benign fibrous histocytoma• Fibrous displasia• Osteofibrous dysplasia• Desmoplastic fibroma

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Cystic LesionsUnicameral bone cystAneurismal bone cystIntraosseous ganglion cystEpideromoid cyst

Fatty tumorsLipoma

Vascular tumorsHemangioma

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INVESTIGATIONS• X-RAY--------------CT SCAN------------------MRI• TECHNETIUM 99 BONE SCAN- (scintigraphic) study that makes use of

Technetium99m (Tc99m-methylene diphosphonate (MDP)) as active agent. • 3 stages which follow IV injection of the tracer.• 1) Flow phase• 2 to 5-sec images are obtained for 60 seconds after injection• demonstrates perfusion and characterises the blood flow to a particular area• 2) Blood pool phase• the blood-pool image is obtained 5 min after injection• demonstrated the blood pool, not the blood flow• inflammation causes capillary dilatation and increased blood flow• If the study is going to be a triphasic bone scan, a third phase is added.• 3) Delayed phase• the bone image is obtained 2 - 4 hours later• urinary excretion has decreased the amount of the radionuclide in soft tissue

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FDG-PET• FDG-PET- Flourine 18-fluorodeoxyglucose labelled-Positron

Emission Tomography uses radioactive glucose to locate cancer. This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera

• Radiolabelled biocompound such as 2-fluoro-2-deoxy-D-glucose (FDG) is injected intravenously.

• Uptake of this compound followed by further breakdown occurs in the cells. Tumor cells have a high metabolic rate hence this compound is also metabolised by tumor cells.

• FDG is metabolised to FDG-6-phosphate which cannot be further metabolised by tumor cells hence it accumulates and concentrates in tumor cells. This accumulation is detected and quantified.

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BIOPSY• Most conclusive test because it confirms if the tumor is

malignant or benign, the bone cancer type (primary or secondary bone cancer), and stage.

• TYPES-• 1. Needle biopsy: During this procedure, a small hole is made

in the affected bone and a tissue sample from the tumor is removed.

two types -• Fine needle aspiration: During this procedure, the tissue

sample is removed with a thin needle attached to a syringe. • Core needle aspiration: During this procedure, the surgeon

removes a small cylinder of tissue sample from the tumor with a rotating knife like device.

• 2. Incisional biopsy: During this procedure, the surgeon cuts into the tumor and removes a tissue sample.

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ENNEKING STAGINGEnneking described the most widely used staging system for benign

bone tumors . The stages are denoted by the Arabic numerals 1, 2, and 3, whereas

malignant bone tumors are classified by Roman numerals (I, II, III). Stage 1-LATENT- low biological activity, well marginated , often

incidental ,may resolve spontaneously. -NOF Stage 2-ACTIVE-Symptomatic,limited bone destruction, may present

with pathological fracture- ABC Stage 3-AGGRESSIVE- Bone destruction/Soft tissue extension, require

complete work-up and a removal with wide margins to avoid possible local recurrence. -GCT

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• For Malignant tumors ,adopted by the Musculoskeletal Tumor Society, and originally developed by Enneking

Stage Grade Local Extent Metastasis 1A low intracompartmental - 1B low extracompartmental - 2A high intracompartmental - 2B high extracompartmental - 3 Any Any + Grade is determined by histological parameters. Low-grade tumors generally have few mitotic figures, little if any

cellular atypia, and have a relatively non-infiltrative growth pattern. High-grade tumors tend to have marked cellular atypia,

hyperchromatism, and nuclear pleomorphism. They often demonstrate an infiltrative growth pattern

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CAMPANACCI STAGING• Radiological grading system

– Better for prognosticating aggressiveness then histology• Used for Giant Cell Tumours• GRADE 1--Intramedullary lesion confined to bone• GRADE 2--Thinned, expanded cortex• GRADE 3--Cortical breakout.

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SURGERY PRINCIPLES• Surgical margin is described by one of four terms—

intralesional, marginal, wide, or radical.• Intralesional Resection--Plane of surgical dissection

is within the tumor. • Often described as “debulking” because it leaves

behind gross residual tumor.• Marginal resection--achieved when the closest plane

of dissection passes through the pseudocapsule.( surrounding reactive tissue around tumor )

• For most benign lesions and some low-grade malignancies

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Wide Resection• Is achieved when the plane

of dissection is in normal tissue

• If the plane of dissection touches the pseudocapsule at any point, the margin should be defined as being marginal and not wide.

• for high-grade malignancies.

Radical resection• All the compartments that

contain tumor are removed en bloc.

• Involves removing the entire bone and the compartments of any involved muscles.

• Rarely used now a days

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Curettage

• Simple Curettage- -cortical window over the lesion

-bulk of the tumor is scooped out-cavity is enlarged back to normal host bone in each direction with a power burr.-copiously irrigated to remove any debris and tumor cells.

• Extended curettage-»use of adjuvants, such as liquid nitrogen,

phenol, polymethyl methacrylate, or thermal cautery

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CARTILAGE LESIONS

- Osteochondroma - Chondroma Enchondroma-arising in medullary

canal Periosteal chondroma/juxtacortical

chondromas-arise on bone surface

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Osteochondroma• Developmental anomalies rather than tumors.• They are usually sporadic, but can be part of:Hereditary multiple exostoses (HME) - also known as diaphyseal aclasisTrevor disease- Osteochondroma on epiphyseal side of growth plate.An osteochondroma can be either sessile or pedunculated, and is seen in the metaphyseal region typically projecting away from the epiphysis.

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• They most commonly arise from appendicular skeleton, especially around the knee .

• Lower limb - 50% of all cases femur (especially distal) - most common : 30%

• Tibia (especially proximal) - 15-20%

• Less common locations - feet, scapula

• upper limb• humerus - 10-20%• hands, pelvis• spine - the posterior elements of

spine are an uncommon, but not rare, site for these tumours

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• Pathologically Osteochondromas are essentially a part of the growth plate.

• Separates and continues growing independently, without an associated epiphysis

• The medullary cavity is continuous with the parent bone, and they are capped by hyaline cartilage

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Osteochondroma• Clinically,

osteochondromas present as slow-growing masses, which can be painful if they impinge on a nerve or if the stalk is fractured. In many cases, they are detected as an incidental finding.

Osteochondroma. On this lateral view of the ankle, a benign osteochondroma is seen projecting posteriorly on a stalk. The end (arrows) is often covered with a cartilaginous cap. These lesions always occur near a joint but point away from it.

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• MRI• MRI is best at assessing cartilage

thickness (and thus assessing for malignant transformation), presence of oedema in bone or adjacent soft tissues and visualising neurovascular structures in the vicinity.

• The cartilage cap of osteochondromas appears the same as cartilage elsewhere, with intermediate to low signal on T1 and high signal on T2 and STIR weighted images.

• A cartilage cap of over 1.5cm in thickness is suspicious for malignant degeneration.

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T1 T2 STIR (FAT SUPPRESSION)

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S/S

• M C symptom of an osteochondroma is a painless mass near the joints. The knee and shoulder are more commonly involved.

• If the stalk of a pedunculated osteochondroma breaks, pain and swelling may start immediately.

• If located under a tendon-- Snapping of the tissue over the tumor may cause activity-related pain.

• If located near a nerve or blood vessel, such as behind the knee causing numbness and tingling in that extremity, periodic changes in blood flow, loss of pulse or changes in color of the limb.

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RISK OF MALIGNAT TRANSFORMATION- 1%- SOLITARY OSTEOCHONDROMA 5%- MULTIPLE HEREDITARY EXOSTOSIS

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TREATMENTNonsurgical Treatment

Most of the time, solitary osteochondroma is not removed surgically. Observe it & take regular X-rays to keep track of any changes.

Surgical TreatmentWhen surgery is recommended, it is best to wait until growth complete (a mature skeleton by X-ray evaluation) before removing a solitary osteochondroma. This decreases the chance of the tumor growing back. Surgery may be considered if the osteochondroma: Is causing pain with activity Puts pressure on a nerve or blood vessel Has a large cap of cartilage The osteochondroma is removed at the level of the normal bone. Some of the inside of the bone may also be removed.

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Hereditary multiple exostosis - autosomal dominant- 2 Genes-EXT1-Ch8 , EXT2- Ch 11- < 10 yrs Clinical features : - knobby appearance - short stature or even dwarf ( limbs short in relation to

trunk ) - Deformity of forearm - in 40 – 60 % ulna short , radius bowed , loss of pronation n

supination , tibiofibular synostosis , genu valgum , coxa valga

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DD : multiple enchondromatosis , achondroplasia

Treatment : excision of symptomatic exostosis correction of deformity & limb length discrepancy

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Enchondroma• Common benign medullary cartilaginous neoplasm• Usually found in children or young adults which can lead to

pathological fractures or undergo malignant degeneration.• 3-10 % of all bone tumours and 12-24 % of benign bone

tumours• Enchondromas are most frequently diagnosed in childhood

to early adulthood with a peak incidence of 10-30 years.• Complicated by a pathological fracture or malignant

transformation into a low grade chondrosarcoma• (clinically if an enchondroma is painful in the absence of a

fracture, it should be considered malignant

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ENCHONDROMA• Enchondromas arise from rests of

growth plate cartilage/chondrocytes that subsequently proliferate and slowly enlarge and are composed of mature hyaline cartilage.

• they are seen in any bone formed from cartilage.

• Two syndromes are associated with multiple enchondromas:

Ollier diseaseMaffucci syndrome.

• small tubular bones of the hands and feet : 50%

• large tubular bones e.g. femur, tibia, humerus

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ENCHONDROMA

• Rarely an enchondroma may extend through the cortex and demonstrate a exophytic growth pattern. This is known as an enchondroma protuberans, and may either be seen sporadically or as part of Ollier disease.

• Almost all enchondromas are located in the medullary cavity of tubular bones.

D/ds:• bone infarct , chondrosarcoma, intraosseous ganglion• other benign lytic bone lesions,• metastases• granulomatous disease : sarcoidosis, tuberculosis

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IMAGING• X-ray & CTTypically enchondromas are small 1 - 2cm lytic lesions with non-aggressive features.narrow zone of transitionsharply defined scalloped margins : may have mild endosteal scallopingexpansion of the overlying cortex may be present but there should not be cortical breakthrough unless fracturedChondroid calcifications may be present : rings and arcs calcification-STIPPLED/PUNCTATE/POPCORNno periosteal rxn.

• The majority of enchondromas more frequently arise in the metaphyseal region,.

A cartilaginous lesion in an epiphysis is more likely to be a chondrosarcoma .

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IMAGES

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IMAGING• MRI• MRI is useful in evaluating for soft tissue extension and for

confirming the diagnosis.• Enchondromas appear as well circumscribed somewhat lobulated

masses replacing marrow.• T1 Intermediate to low signal• T1 C+ (Gd)enhancement is variable, and may be seen both peripherally or of translesional septae. Similar pattern of enhancement may be seen in chondrosarcomas.• T2 Typically of background intense high signal

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MRI

• T1 T2 STIR

• Hypointense Hyperintense

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OLLIER”S DISEASE• Ollier disease also known as

enchondromatosis, is a non-hereditary, sporadic, skeletal disorder characterised by multiple enchondromas that are principally located in the metaphyseal regions.

• Plain films show multiple enchondromas. Larger lesions can show cartilage calcification in a typical rings and arcs pattern.

• Imaging characterestics are of same as ENCHONDROMAS

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MAFUCCI’S SYNDROME• Maffucci syndrome is a congenital non

hereditary mesodermal dysplasia characterised by multiple enchondromas with soft-tissue cavernous haemangiomas.

• Imaging findings are multiple enchondromas seen associated with soft tissue swelling and phleboliths.• Enchondromas degenerate into

chondrosarcomas in 15-51% of cases and soft-tissue haemangiomas to vascular sarcomas in 3-5%.

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OLLIER’SDISEASE

MAFFUCI’SDISEASE

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Chondroblastoma• Rare benign cartilaginous neoplasms( CODMAN

TUMOR )• Less than 1% of all primary bone tumours, occurring

predominantly in young patients (< 20 years of age). There is a male predilection.

• Pathologically composed of chondroblasts, chondroid matrix, cartilage with occasional giant multi-nucleated cells.* with surrounding chondroblasts.

• Aneurysmal bone cysts can be seen secondarily to underlying chondroblastoma.

• Patient may present as synovitis of knee.

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SITE

• Epiphysis of a long bone (70% occurring in the humerus (most frequent), femur and tibia, ~ 10% are found in the hands and feet)

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IMAGING• X-rays • epiphyseal• well defined lytic lesions; either smooth or lobulated margins with

a thin sclerotic rim• Internal calcifications can be seen in up to 40-60% of cases• They range in size from 1-10cm, with most being 3-4cm at diagnosis

• CT • better delineation of the relationship to the growth plate and

articular surface• Solid periosteal reaction (seen in up to 50% of cases) and internal

calcification (calcified matrix seen in ~ 1/2 of cases) and cortical breach are also more easily appreciated.

• Endosteal scalloping may be seen

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image

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IMAGING• MRI• Ideal for the evaluation of transphyseal or

transcortical extension.• Demonstrating associated surrounding bone

marrow oedema.• These lesions have signal typical of cartilage:T1 - lesion itself is of low to intermediate signalT2 / STIR - lesion is of intermediate to high signal• Fluid-fluid levels may occasionally be seen .

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image

• T1 T2 STIR

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MICROSCOPY

• Sheets of chondroblasts with background of chondroid matrix.

• Polygonal cells with distinct cytoplasm.• Dystrophic calcification surrounding individual

cells – “CHICKEN WIRE “ appearance.• Abundant Giant cells• Secondary ABC in 20 % cases.

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TREATMENT• The goal for treatment of chondroblastoma is to

remove the tumor and prevent damage to the end of the affected bone.

• Treatment may include:Surgical removal of the tumor -

Biopsy and curettage with possible use of adjuvant liquid nitrogen or phenol, or a mechanical burr. It may be necessary to reconstruct articular surfaces due to subchondral erosion.

Bone grafting /bone cement usedAny joint invasion is usually secondary to previous

instrumentation.

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BONE FORMING TUMORS

Osteoid osteoma Osteoblastoma Bone island

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OSTEOID OSTEOMA

- Osteoblastic mass called nidus surrounded by zone of reactive sclerosis

- 2nd decade - M > F- Site : proximal femur ( mc ) , tibia , spine ( posterior elements ) Not seen in bones of membranous origin C/F : Dull pain , worse at night , relieved with

NSAID s , not related to position or function , often aggravated by alcohol

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- Pain is elicited by local pr. - O.O is suspected in spine when a pt < 30 yrs

complains of constant back pain , when spine is stiff and scoliotic & SLRT is positive with no signs of nerve root compression.

Radiological findings : Radiolucent nidus ( 1.5 cm) with reactive sclerosis in cortex ,

DD : Osteoblastoma , Non-suppurative osteomyelitis of garre , Brodies abscess, Stress # , Diagnosis : Tc99 bone scan – Inc uptake “Headlight in fog “ & “Double – density sign “CT – BULLS EYE appearance

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Pathology : Nidus – osteoblasts & nonmyelinated axons Stroma – osteoblasts , osteoclasts , fibroblasts & blood

filled capillaries - Transform into osteoblastoma but no malignant

transformation. Treatment : Self limiting lesions – conservative treatment with

NSAIDs – not well tolerated - Surgery : to eradicate pain producing nidus ( accurate

localization is hence crucial ) methods : - en bloc resection or burr down tech. Latest is percutaneous radiofrequency ablation

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OSTEOBLASTOMA

- Histologically similar to O.O but differing in progressive growth & absence of reactive perifocal bone formation.

- Potentially malignant - Mc site : vertebral column ( post. Elements)- C.F : pain of varying intensity pathological # neurological problems Radiographic features : nothing particularly distinctive well circumscribed lesion , CT – COTTON WOOL appearance due to irregular opacities

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- Radiopacity expression of quantity & degree of maturation of osteoid substance.

Bone scan – localizing smaller lesions esp in spine Pathology : very vascular DD : Osteoid osteoma

Osteosarcoma ( increase in sr. ALP). Treatment : curettage & bone grafting

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FIBROUS LESIONS

Nonossifying fibroma Cortical desmoidBenign fibrous histiocytoma Fibrous dysplasia Osteofibrous dysplasia

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NON OSSIFYING FIBROMA - Also called Fibrous cortical defect or Metaphyseal cortical

defect or Fibrous xanthomas- Mc musculoskeletal tumor - Occurs in 30 % of children - 1st two decades - Site : femur , tibia , humerus ( 8 % - multiple lesions) - Asymptomatic - X ray : well defined , eccentric , radiolucent lesion in

metaphysis ,multilocular app or rim of sclerosis , doesn’t expand the cortex & no periosteal reaction.

- Treatment : observation

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FIBROUS DYSPLASIA -intrinsic defect of endochondral bone maturation immature

ossification pattern -Characterized by replacement of normal bone & marrow by

fibrous tissue & small woven spicules of bone weakbone .- Solitary or multifocal - ALBRIGHT ‘ S SYN : polyostotic F.D + café au lait spots +

endocrinopathies .- MAZABRAUD SYN- Polyostotic FD+ Intramuscular myxomas- Site : rib , femur , humerus , tibia , maxilla - C.F : monostotic – asymptomatic bone pain , skeletal

deformities .

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RADIOGRAPHIC APPEARENCERadiographic features : - Epiphysis or metaphysis or diaphysis - Well defined geographic lytic lesions - Ground glass matrix : intramedullary radiolucencies- Cortical thinning - Secondary deformities esp. in wt , bearing bones - Shepherd’s crook deformity ( microfractures – on tension side of bone ) - Poorly defined areas of osteolysis suspect - Cortical destruction malignant - Soft tissue involvement transformation

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Shepherd's crook deformity

• Coxa varus angulation of the proximal femur, classically seen in femoral involvement by fibrous dysplasia, although may be seen in other disorders such as Paget disese of bone and osteogenesis imperfecta.

• The shape of the proximal femur resembles that of the staff carried by herders (shepherds), which is known as a crook.

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Shepherd's crook deformity

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TREATMENT- Monostotic lesions asymp no Rx Indications : severe deformity persistent pain pathological # - Curettage & grafting ( cortical allograft prfrd.

d/t slower absorption)- Internal fixation +/- osteotomy - Bisphosphonates : beneficial

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CYSTIC LESIONS

Unicameral bone cyst Aneurysmal bone cystIntraosseous ganglion cyst

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UNICAMERAL BONE CYST

- Developmental anamoly of physis - Transient failure of ossification of physeal cartilage &

cyst formation- < 20 yrs age , M > F - Spontaneously resolve in late adolescence , rarely persist

into adulthood - Site : proximal humerus , proximal femur , calcaneum . Active cysts are juxtaposed to physis (within 1 cm)C.F : usually asymptomatic , pathological #

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- Cysts progress from active to quiescent to involutional stage . - cysts usually shrink as patient approaches skeletal maturity.

- Radiographic features : metaphysis of immature skeleton ,

DIGNOSTIC- well marginated , centrally located ,purely lytic lesion.

radiolucent that expand and thin the cortex . FALLEN FRAGMENT SIGN - # fragment in the cyst DD : ABC & fibrous dysplasia

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FALLEN

FRAGMENT SIGN

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Bone cyst of humerus

Pathologic fracture“Falling Fragment Sign”

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MRIMR signal characteristics for an uncomplicated lesion includeT1 - low signalT2 - high signalUsually there no fluid-fluid levels unless there has been a complication with haemorrhage.

CT and MRI add little to the diagnosis, but are however helpful in eliminating other entities that can potentially mimic a simple bone cyst

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T1 T2

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TREATMENT

Small asymp lesions in upper extremities-observation & follow up

Larger lesions Symptomatic lesions need Rx Lesions in lower extremities Various options are Curettage +/ - bone grafting +/- int. fixation Aspiration & inj of steroids or bone products

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Aneurysmal bone cyst (ABC)• Benign , Expansile • Primarily seen in children and adolescents (80% l <20yrs of

age)• Blood-filled spaces of variable size separated by

connective tissue (trabeculae of bone or osteoid tissue) and osteoclast giant cells

• Not lined by endothelium.Types • Primary• Secondary (e.g chondroblastoma, fibrous dysplasia, giant cell tumour (GCT), osteosarcoma)

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• Location Long bones – tibia & fibula (24%), femur (13%),Spine 20-30% (posterior elements).sacrum

• A variant of ABCs is the giant cell reparative granuloma.( SOLID ABC)

• seen in the tubular bones – hand , feet craniofacial skeleton.

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Radiographic features • X ray sharply defined, expansile osteolytic lesions, with thin sclerotic margins.Eccentricity is typical.But very often missed out due to cortical thinning due to ballooning.CT Demonstrates these findings to a greater degree, and is also better at assessing cortical breach and extension into soft tissues.Fluid fluid levels (better than MRI)

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MRI

• MRIDemonstrate Fluid Fluid level in lesion.To distinguish between Primary and secondary (if solid component Is present.)

The cysts are of variable signal, with surrounding rim of low T1 and T2 signal. Focal areas of high T1 and T2 signal are also seen presumably representing areas of blood of variable age.SBC vs ABC on MRI- Presence of double density fluid level & intralesional septations- ABC

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BONE SCAN

• Doughnut sign - increased uptake peripherally with a photopenic centre.

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TREATMENT

Excision – cortical based or surface tumors Extended intralesional curettage & grafting –

central lesions Embolisation – vertebral & pelvic ABC Low dose radiation – effective method but not used

d/t malignant transformation.

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ANJALI ,20F,ABCRx-Curettage+ G Bone grafting

PRE OP IMMEDIATE POST OP FOLLOW UP

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DHRUV 8/M ABCRx-curettage+ Chron os grafting

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GIANT CELL TUMOURDEFINITION:

Distinct neoplasm arising from non-bone forming supportive connective tissue of marrow with network of stromal cells regularly interspersed with giant cells. ( Jaffe & Liechtenstein )

-75-80% OF PATIENTS 20-50 YRSMale:Female- 1:1.3 (Benign) -3:1 (Malignant)

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Site• 55% AROUND THE KNEE • 10% in the distal radius• 6% in the proximal humerus• SPINE rarely involved (commoner in the sacrum)• In the head and neck region the maxilla and mandible are more commonly involved.

• SIGNS&SYMPTOMS

1. PAIN

2. SWELLING

3. JOINT RESTRICTION

4. MUSCLE WASTING

5. NEUROLOGICAL SIGNS

6. PATHOLOGICAL #

7. PULMONARY METS- 3 %

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PathologyGROSS- End of bone is expanded. Eccentric lesion at the epiphyseo-metaphyseal region. Thin periosteum. Fleshy dark brown, soft, friable mass. Cystic spaces seen.

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PathologyMicroscopy- Vascularized network of round,oval or spindle shaped

stromal cells and multinucleated giant cells( 40-60 nuclei /cell) with numerous centrally placed nuclei

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GRADING SYSTEM

Jaffe,Lichenstein and Portis(1940)

GRADE 1-o Conventional GCTGRADE 2-o Boderline tumoursGRADE 3-o Sarcomatous type of stroma

Modified gradingSannerkin et al(1980)

• Malignant GCT- with frank sarcomatous changes and full metastatic potential

• Borderline GCT- without sarcomatous changes but with abnormal mitoses or vascular permeation or both

• Conventional GCT- without features of any of the above two types

No correlation exists between histological grading and clinical behavior of the tumour.

Hence grading not widely accepted.

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RADIOLOGYType of Osteolysis

• Geographic destruction (I)

• Moth-eaten (II) Permeative(III)

Lodwick

1A 1B 1C

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RADIOLOGY• Expansile

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RADIOLOGY

• TRABACULATION

PURE LYTIC (60%) FINE TRABACULTION(40%)

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SCINTI GRAPHYLess useful

Inconsistent uptake“Doughnut sign”

M.R.I.Soft tissue spread

Joint breachLocate N.V. bundleT1-Dark,T2-Bright

ABC-20%

C.TIntraossous content

Intra articular spreadCortical breachSite of window

ANGIO GRAPHYLocate vessels

type of feedersFor embolisation

INVESTIGATIONS

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SURGICAL TREATMENTStage1& Stage2 --- Intralesional or Marginal Excision

Stage3 --- Wide resection with Reconstruction

Radiation, Embolaisation

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Curettage & Bone GraftingINDICATION STAGE-1&2

ADEQUATE WINDOW

MOTORISED BURR

( 20,000 rpm ) BISPHOSPHONATES- Locally or systemically prevent recurrence.IV ZOLENDRONIC ACID USE FOR PRIMARY GCT UNDER STUDY

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DEPENDING ON SITE

• AROUND KNEE-Hemicondylar osteoarticular allograft reconstruction / rotation hinge endoprosthesis.

• AGGRESSIVE LESION OF DISTAL RADIUS- Primary resection & reconstruction with proximal fibular autograft( arthroplasty or arthrodesis)

• EXPENDABLE BONES( DISTAL ULNA/PROX. FIBULA)- Priamry resection without reconstruction.

• Spine /pelvis- Irradiation/embolization/both• PULMONARY METS- Resection

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‘E’ OF GCT

• EPIPHYSEAL• ECCENTRIC• EXPANSILE• EXTENDED CURETTAGE• EXCISION –IF EXTENDED CURETTAGE FAILS

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