Primary bone tumors presenter: ondari n.j FACILITATOr : prof . gakuu
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Transcript of Primary bone tumors presenter: ondari n.j FACILITATOr : prof . gakuu
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PRIMARY BONE TUMORS
PRESENTER: ONDARI N.J
FACILITATOR: PROF. GAKUU
28-10-2013
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Outline
Introduction Classification Epidemiology Evaluation Staging Principles of management Selected tumors Therapeautic advances
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Introduction
Forms 0.2% of human tumor burden
Primary malig bone tumors make 1% of all malignant tumors
Carcinoma commonly metastasize to LN except BCC
Sarcomas commonly metastasize hematogenously
Most have male predominance excep GCT, ABC
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Classification
Based on tissue of origin Bone Cartilage Fibrous tissue Bone marrow Blood vessels Mixed Uncertain origin
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Evaluation
History
Physical examination
Investigations; labs, imaging
Biopsy
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Analytic approach to evaluation of the bone neoplasm
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Evaluation; history
Age
Symptomatology Pain Swelling History of trauma Neurological sympts
Pathological fracture
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Evaluation; physical examination Lump/swelling
5S MTC
Effusion
Deformities
Regional nodes
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Evaluation; imaging
Plain radiograph
CT scan
MRI
Radionuclide scanning
PET
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Radiography
Information yielded by radiography includes : Site of the Lesion
Borders of the lesion/zone of transition
Type of bone destruction
Periosteal reaction
Matrix of the lesion
Nature and extent of soft tissue involvement
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Radiographic features of bone tumors
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Site of the Lesion
Determined by the laws of field behavior and developmental anatomy of the affected bone, a concept first popularized by Johnson. Parosteal osteosarcoma -posterior aspect of the distal
femur Chondroblastoma -epiphysis of long bones before skeletal
maturity Adamantinoma and osteofibrous dysplasia have a specific
predilection for the tibia A lesion's location can also exclude certain entities from the
differential diagnosis. E.g Giant cell tumor -articular end of bone.
Location in relation to the central axis of the bone esp in long tubular bone, such as humerus, radius, femur, or tibia. For example, simple bone cyst, enchondroma, or a focus of
fibrous dysplasia -always centrally located Eccentric location is Xteristically observed in aneurysmal
bone cyst, chondromyxoid fibroma, and nonossifying fibroma
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Predilection of Tumors for Specific Sites in the Skeleton
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Parosteal osteosarcoma Chondroblas
toma
Adamantinoma
Site of the lesion.
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Distribution of various lesions in a long tubular bone in a growing skeleton
Site of the lesion. Distribution of various lesions in a long tubular bone after skeletal maturity
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Location of epicenter of lesion usually determines site of its origin (medullary, cortical, periosteal, soft tissue, or in the joint)
Site of the lesion.
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Malignant lesions are seen predominantly in its anterior part (body)
Distribution of various lesions in a vertebra.
Benign lesions predominate in its posterior elements.
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Borders/margins of the Lesion
Margins determined by GRate hence benign or malignant
Three types of lesion margins are encountered: Sharp demarcation by sclerosis (IA margin), sharp demarcation without sclerosis (IB margin) Ill-defined margin (IC margin)
Slow-growing lesions -sharp sclerotic borders; usually indicates that a tumor is benign E.g nonossifying fibroma, simple bone cyst
Indistinct borders- typical of malignant or aggressive lesions
Post- Radio- or chemo of malignant bone tumors Can exhibit sclerosis and a narrow zone of transition
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Borders of the lesiondetermine its growth rate.
sharp sclerotic sharp lytic ill-defined.
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A: Sclerotic border typifies a benign lesion e.g nonossifying fibroma in the distal femur.
B: A wide zone of transition typifies an aggressive or malignant lesion e.g plasmacytoma involving the pubic bone and supraacetabular portion of the right ilium
Borders of the lesion.
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Type of Bone Destruction
Mechanisms of bone destruction Direct effect of tumor cells Incr osteoclastic activity
Cortical bone is destroyed less rapidly than trabecular bone. Loss of cortical bone appears earlier on radiography trabecular bone must be destroyed (about 70% loss of
mineral content) before the loss becomes radiographically evident
Bone destruction can be described as geographic (type I) - benign lesions moth-eaten (type II) and permeative (type III) - rapidly growing infiltrating
tumors
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Patterns of bone destruction. geographic
a uniformly affected area within sharply
defined borders
moth-eaten
rapidly growing infiltrating lesions
permeative type
characteristic of round cell tumors
myeloma Ewing sarcomagiant cell tumor.
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Periosteal Response the pattern of periosteal reaction is an indicator of the
biologic activity of a lesion . periosteal reactionsthat can be categorized as;
uninterrupted (continuous) or I nterrupted (discontinuous).
Any widening and irregularity of bone contour may represent periosteal activity.
An uninterrupted periosteal reaction indicates a long-standing (slow-growing), usually indolent, benign process.
There are several types of solid periosteal reaction: a solid buttress e.g aneurysmal bone cyst and chondromyxoid
fibroma; a solid smooth or elliptical layer e.gosteoid osteoma and
osteoblastoma; a single lamellar reaction, such as accompanies Langerhans cell
histiocytosis Sunburst (“hair-on-end”) or onion-skin (lamellated) pattern
. Codman triangle
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An uninterrupted periosteal reaction usually indicates a benign process, whereas an interrupted reaction indicates a malignant or aggressive nonmalignant process
Types of periosteal reaction.
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Examples of Nonneoplastic and Neoplastic Processes Categorized by Type of Periosteal Reaction
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Interrupted type of periosteal reaction
sunburst pattern -osteosarcoma
lamellated or onion-skin type in ewing sarcoma
Ewing sarcoma -lamellated type
Codman triangle (arrow)
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Type of Matrix The matrix represents the intercellular material produced
by mesenchymal cells E.g osteoid, bone, chondroid, myxoid, and collagen material .
Type of matrix allows differentiation of some similar-appearing E.g differentiating osteoblastic from chondroblastic processes.
Calcifications in the tumor matrix, point to a chondroblastic process. Calcifications typically appear as punctate (stippled),
irregularly shaped (flocculent), or curvilinear (annular or comma-shaped, rings and arcs).
Differential diagnosis of stippled, flocculent, or ring-and-arc calcifications includes enchondroma, chondroblastoma, and chondrosarcoma.
A completely radiolucent lesion may be either fibrous or cartilaginous in origin tumor-like lesions, such as simple bone cysts or intraosseous
ganglion
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C. by the presence of a solid sclerotic mass, such as in parosteal osteosarcoma
Types of matrix: osteoblastic
A. fluffy, cotton-like densities within the medullary cavity, e.g in this case of osteosarcoma of the distal femur
B. presence of the wisps of tumor-bone formation, like in this case of osteosarcoma of the sacrum
The matrix of a typical osteoblastic lesion is characterized by the presence of the following features
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Types of matrix: chondroid matrix A: Schematic representation of various appearances of chondroid matrix calcifications.
B: Enchondroma displays a typical chondroid matrix
C: Chondrosarcoma with characteristic chondroid matrix
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Soft Tissue Mass
A bone lesion associated with a soft tissue mass should prompt the question of which came first.
Is the soft tissue lesion an extension of a primary bone tumor, or is it a primary soft tissue tumor invading bone?
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Radiographic features differentiating primary soft tissue tumor invading bone from primary bone tumor invading soft tissues.
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Benign Versus Malignant Nature clusters of features that can be gathered from
radiographs can help in favoring one designation over the other .
Benign lesions usually have well-defined sclerotic borders exhibit a geographic type of bone destruction the periosteal reaction is solid and uninterrupted, and there is no soft tissue mass.
Malignant tumors often exhibit poorly defined borders with a wide zone of
transition; bone destruction appears in a moth-eaten or
permeative pattern, and the periosteum shows an interrupted, sunburst, or
onion-skin reaction with an adjacent soft tissue mass.
NB-benign lesions may also exhibit aggressive features
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Radiographic features that may help differentiate benign from malignant lesions
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Grading of bone sarcomas
Criteria for grading Cellularity Nuclear features Mitotic figures necrosis
Correlates with prognosis in some tumors E.g chondrosarcoma, malig vascular tumors
Some not amenable to histological grading e.g monomorphic tumors Ewing, MM, lymphoma
Some always high grade Sometimes not useful in predicting prognosis
Adamantinoma, chordoma
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Staging of bone tumors
Benign tumors (Enneking staging of benign tumors) Stage 1 - latent Stage 2 - active Stage 3 - aggressive
Malignant tumors TNM staging AJCC staging system Musculoskeletal tumor society staging
system(enneking) Surgical staging
Note Benign tumors - classified using Arabic
numerals(1,2,3) Malignant tumors - classified using roman
numerals(I,II,III)
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William F. Enneking M.D
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Enneking classification systems
Enneking classification of benign tumors Latent, active, aggressive
Enneking surgical staging of malignant tumors
Enneking classification of local procedures Intracapsular, marginal, extended, radical
Enneking classification of amputations Intracapsular, marginal, extended, radical
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Enneking classification of local procedures
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Enneking classification of amputations
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Enneking staging of benign tumors
Stage 1; Latent Well defined margin Grows slowly and then stops Heals spontaneously eg osteoid osteoma Neglible recurrence after intracapsular resection
Stage 2; Active Progressive growth limited by natural barriers Well defined margin but may expand thinning cortex e.g ABC Negligible recurrence after marginal excision Rx marginal resection
Stage 3; aggressive Growth not limited by natural barriers e.g GCT Mets present in 5% of these pts Have high recurrence after intracapsular or marginal
resection Extended resection preferred
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Enneking surgical Staging of malignant tumors
Incorporates
degree of differentiation Low grade(stage I) or High grade(stage II)
Local extent of tumor Intracompartmental - A Extracompartmental - B
distant spread metastasis
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Enneking surgical Staging of malignant tumors
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AJCC staging for bone sarcomas Based on
Tumor grade Low grade(I) High grade(II)
Tumor size <8cm -A >8cm -B
Presence and location of mets Skip mets -III Pulm mets -IVA Non-pulm mets -IVB
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Bone biopsy
Options Needle biopsy
90% accuracy at determining malignancy Accuracy at determining specific tumor much lower Absence of malignant cells less re-assuring than
incisional biopsy Core biopsy
Provides accurate diagnosis in 90% of cases incisional biopsy
Primary resection instead of biopsy can be done in; Small(<3cm) subc mass- marginally resected if
likely malignant Characteristic radiographic appearance of benign
lesion Painful lesion in an expendable bone e.g prox
fibula, distal ulna
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Tumour Biopsy Principles 1
1.Biopsy done only after evaluation & imaging is complete. determine xteristics and local extent of the tumor and mets Staging helps determine the exact anatomic approach to tumor Biopsy superimposes radiologic changes at the biopsy site, and
there4 can alter the interpretation of the imaging studies.
2. Place small incisions whenever possible- skin & capsule
3. The biopsy track be considered contaminated with tumor cells. Track excised en bloc with the tumor subsequently.
4. The surgeon should be familiar with incisions for limb salvage surgery, and also with standard and nonstandard amputation flaps.
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Needle biopsy track contaminated patellar tendon
Multiple needle tracks contaminate quadriceps tendon
Needle track placed posteriorly, location that would be extremely difficult to resect en bloc with tumor if it had proved to be sarcoma.
Examples of poorly performed biopsies
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Tumour Biopsy Principles 2
5. If a tourniquet is used; The limb is elevated before inflation Avoid exsanguination by compression.
6. contaminate as little tissue as possible. Avoid transverse incisions The deep incision should go thru single
muscle compartment (muscle belly) rather than through an intermuscular plane.
Major neurovascular structures should be avoided.
Care should be taken not to contaminate flaps.
Minimal retraction should be utilized to limit soft tissue contamination.
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Example of poorly performed biopsy
Transverse incisions should not be used
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Tumour Biopsy Principles 3
7. If possible soft tissue extension of a bone lesion should be sampled
8. If a hole must be made in the bone, it should be round or longitudinally oval to minimize stress concentration and prevent a subsequent fracture. A fracture may preclude a subsequent limb
salvage surgery. PMMA is plugged into the hole to contain a
hematoma - minimal.
9. Biopsy should be taken from the periphery of the lesion, which contains the most viable tissue. Biopsy material may be sent for M/C/S if in doubt
regarding infection
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If hole must be made in bone during biopsy, defect should be round to minimize stress concentration, which could lead to pathological fracture
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Biopsy resulted in irregular defect in bone, which led to pathological fracture
Examples of poorly performed biopsies
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Tumour Biopsy Principles 4
10. A frozen section should be sent intraop to ensure that diagnostic tissue has been obtained. If a tourniquet has been used it should be deflated
and meticulous haemostasis ensured before closure.
11. Drains should not be used routinely. If a drain is used, it should exit in line with the
incision. The wound should be closed tightly in layers.
12. operating surgeon should accompany specimen to pathologist if feasible Discuss with the pathologist about clinical findings,
imaging, intraop findings and the specimen
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Drain site was not placed in line with incision
Example of poorly performed biopsy
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Principles of management
Multidisciplinary team approach Benign asymptomatic tumors
If certain observe If in doubt biopsy
Benign symptomatic or enlarging tumors Biopsy Excision/ curretage
Suspected malignant tumors If primary admit for work-up Staging Choices; amputation, limb sparing surgery,
adjuvant therapy
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Benign tumors - not aggressive
Bone-forming tumors Osteoid osteoma Bone island
Cartilage lesions Chondroma Osteochondroma
Fibrous lesions Nonossifying fibroma Cortical desmoid Benign fibrous
histiocytoma Fibrous dysplasia Osteofibrous dysplasia Desmoplastic fibroma
Cystic lesions Unicameral bone cyst Aneurysmal bone cyst Intraosseous ganglion cyst Epidermoid cyst
Fatty tumors Lipoma
Vascular tumors Hemangioma
Other nonneoplastic lesions Paget disease Brown tumor-
hyperparathyroidism Bone infarct Osteomyelitis
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Aggressive benign tumors Giant cell tumor
Chondroblastoma
Chondromyxoid fibroma
Osteoblastoma
Langerhans cell histiocytosis
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Osteoid Osteoma
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Bone Island
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CARTILAGE LESIONS
Enchondroma
Olliers disease
Maffuci synrome
Chondroma
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CARTILAGE LESIONSOsteochondroma
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Fibrous lesionsNonossifying fibroma
Shepherd’s crook appearance
Polyostotic Fibrous dyspalsiaFibrous dysplasia
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Cystic lesions
Unicameral bone cystAneurysmal bone cyst
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Aggressive benign tumors
Giant cell tumor Chondroblastoma
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Chondromyxoid fibroma
Aggressive benign tumors
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Malignant Tumors of Bone Osteosarcoma Chondrosarcoma Ewing sarcoma Chordoma Adamantinoma Malignant vascular tumors Malignant fibrous histiocytoma and
fibrosarcoma Multiple myeloma and plasmacytoma Lymphoma Metastatic carcinoma
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Osteosarcoma
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Chondrosarcoma
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Ewing Sarcoma
may be confused with osteomyelitis
Commonly affects diaphysis with onion skin appearance
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Adamantinoma
Bubble-like appearance
85% occur in tibia
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The end
Thank you