Anatomy of the upper and lower extremities

Neoplasms of Musculoskeletal Tissue

SALTER CH. 14

Dwi Setia Branzfy- 2208436627

Multiyus Hasnah-220843
Anatomi Ekstremitas Atas dan
Ekstremitas Bawah
Ekstremitas Atas
Ekstremitas Bawah
NEOPLASM-LIKE LESIONS AND
TRUE NEOPLASMS OF BONE - Terms
 Tumor  less precise than neoplasm : a new and abnormal formation of cells, a
process that progresses throughout the life of the patient unless some type of
therapy intervenes
 Neoplastic cells do not reach maturity  continue to divide by mitosis more rapidly
than normal cells  producing progressive lesion
 If neoplastic cells demonstrate the ability to initiate independent growth in distant
sites (metastases) : malignant ; referred to as cancer.
 Primary neoplasms : arise from “local inhabitants” ; secondary neoplasms : “outside
invaders”
 Benign neoplasm : remains localized in its primary site ; may be neoplastic, or
may be not.
Classifications

 Based on cell origin : osteogenic, chondrogenic, fibrogenic, angiogenic,

myelogenic (for both neoplasm-like lesions and the true neoplasm of bone)
Classification of Neoplasm-like Lesions of Bone

A. Osteogenic fibrous defect)

1. Osteoma (ivory exostosis) 2. Nonosteogenic fibroma (nonossifying
2. Single osteochondroma ( fibroma)
osteocartilaginous exostosis) 3. Monostotic fibrous dysplasia
3. Multiple osteochondromata (multiple 4. Polyostotic fibrous dysplasia
hereditary exostoses) 5. Osteofibrous dysplasia ( Campanacci
4. Osteoid osteoma syndrome)
5. Benign osteoblastoma (giant osteoid 6. "Brown tumor" (hyperparathyroidism)
osteoma) D. Angiogenic
B. Chondrogenic 1. Angioma of bone (hemangioma and
1. Enchondroma lymphangioma)
2. Multiple enchondromata (Ollier’s 2. Aneurysmal bone cyst (ABC)
dyschondroplasia) E. Uncertain origin
C. Fibrogenic 1. Simple bone cyst (unicameral bone cyst)
1. Subperiosteal cortical defect ( metaphyseal (UBC)
Classification of True Primary Neoplasms
of Bone
A. Osteogenic 1. Angiosarcoma of bone
1. Osteosarcoma (osteogenic sarcoma) E. Myelogenic
2. Surface osteosarcoma (parosteal 1. Myeloma of bone (multiple myeloma)
sarcoma; periosteal sarcoma) 2. Ewing's sarcoma (Ewing's tumor)
B. Chondrogenic 3. Hodgkin's lymphoma of bone
1. Benign chondroblastoma 4. Non-Hodgkin's lymphoma (reticulum
2. Chondromyxoid fibroma cell sarcoma)
3. Chondrosarcoma 5. Skeletal reticuloses (Langerhans' cell
C. Fibrogenic histiocytosis

1. Fibrosarcoma of bone 6. Leukemia

2. Malignant fibrous histiocytoma of F. Uncertain origin

bone 1 . Giant cell tumor of bone (
D. Angiogenic osteoclastoma)
Classification by WHO (Apley)

 Benign lesion of bone : does not invade surrounding tissue or spread elsewhere in the body
 Intermediate (locally aggressive) lesions of bone : can destroy bone and surrounding tissue (e.g. osteoblastoma) ; recurrence is
frequent ; en bloc resection is sometimes required
 Intermediate (rarely metastasizing) lesions :behave in a similar way to locally aggressive lesions, occasionally demonstrate the
ability to spread to distant sites (<2 %) e.g. Giant cell tumor of bone
 Malignant tumours : truly aggressive with the potential for both local extension and metastases to distant sites.
General Consideration

 Incidence
 Malignant neoplasms (primary lesions in musculoskeletal tissues) : rare ; 1% of malignant
diseases (5% in childhood)
 Benign / neoplasm-like lesion: less rare.
 Secondary neoplasm : common
 Age : distinctive
 Osteosarcoma : childhood and adolescence
 Ewing’s sarcoma : adolescents and young adults
 Osteoclastoma (Giant Cell Tumor) , chondrosarcoma, fibrosarcoma : middle adult life
 Multiple myeloma : older adults
 Metastatic neoplasms: in the elderly
 Anatomical site : significant
 Diagnosis
 Possible with complaints such as: unexplained pain, swelling, lump, or decrease in function
 Evaluation and treatment : highly specialized ; hence if suspected  REFER to tertiary care
musculoskeletal oncology unit (for a definitive treatment by oncological orthopaedic surgeon,
radiologists, oncological pathologist, radiation oncologists, medical oncologists, rehabilitation
physicians)

 Complete history, physical examination, diagnostic imaging, laboratory investigation, staging of the
neoplasm, and biopsy
Clinical Features

 Recent local trauma  brings the preexisting neoplasm to the attention

 Slowly growing neoplasm/ neoplasm-like lesion  seldom cause symptoms, or
may interfere with function; or pathological fracture
 Malignant neoplasms: pain (progressively more severe; disturb sleeping) ; sudden
pain  pathological fracture
 Local swelling/lump
 Benign: firm, nontender
 Malignant: diffuse, tender ; if lesion is vascular: warm skin, dilated superficial veins
 Near joint: decreased ROM, painful movement
Diagnostic Imaging and Correlation with
Pathology
 Plain radiography (x-rays), plain (conventional) tomography, CT, MRI, and
scintigraphy (bone scan).
 Plain radiography
 Initial method
 Reveals location and size, resorption of bone, the margin of the lesion (whether clear
or fuzzy), bone reaction, effect of the lesion on the cortex (none, expansion,
penetration).
 Occasionally reveals neoplasm by accident.
 Some neoplasms incite local osteoclastic resorption bone ( osteolytic appearance) ;
some incite osteoblastic deposition of bone (osteosclerosis appearance)
 The reactive bone may almost obscure the
underlying neoplasm
 Slowly growing lesion : deep cortex is
eroded; periosteum reacts by depositing
bone , hence bone expansion
 Elevated periosteum produces reactive bone
in the angle where it is still attached
triangular-shaped area of reactive bone :
Codman's triangle
 Elevation of the periosteum in "stages" 
successive layers of periosteal reactive bone
 "onion-skin" appearance
 Malignant neoplasm grows beyond cortex 
blood vessels keep pace and grow radially
from the cortex  neoplastic and reactive
bones are formed along radiating vessels 
“sunburst” appearance
 Weakened bone by osteoclastic resorption 
pathological fracture
 MBD : Radiographic appearances may be osteolytic type ; or osteosclerotic type
 The only true cyst : simple bone cyst  neoplasm-like lesion (Fig. 14.12). Other osteolytic
lesions may appear to be cystic radiographically, but in fact solid lesions (Fig. 14.13).
 Signs : neither specific nor constant ; "spot diagnosis” : an example more of
cleverness than of wisdom.
 All available data from the various diagnostic methods must be correlated to
reach a high standard of diagnostic accuracy.
 Plain (Conventional) Tomography
 Sections or slices of tissue at varying depth  helpful in
evaluating abnormalities within high-contrast tissue such as
bone
 Replaced to a large extent by CT and MRI
 Computed Tomography (CT)
 Accurate images of “sections” or “slices” ; varying
densities are more clearly differentiated with less
radiation compared to plain tomograms.
 Preferred for depicting site and extent , as well as skip
lesions , soft tissue extent within bone
 Better bone detail in deep regions
 Useful in detecting areas of ossification and
calcification , subtle pathological fractures
 Reveal very small pulmonary metastasis
 Magnetic Resonance Imaging
 Non-ionizing radiofrequency radiation
 Better images of soft tissues, bone marrows, nerves,
blood vessels, relationship of neoplasms to those
structures
 May provide physiological and anatomical data
(combined with contrast and spectroscopy)
 Bone marrow : low signal intensity in T1-weighted
images (dark) ; soft tissue extension and neoplasms
have high signal intensity (bright) in T2-weighted
images
 Fat: high signal on both T1 and T2 , fibrous lesions :
low signal in both T1 and T2
 Useful in staging of malignant neoplasms
 Scintigraphy (Bone Scan)
 Use of bone-seeking radionuclides such as technetium-99-labeled
polyphosphate reflect changes of local blood flow in bone, local
metabolic activity (especially increased bone formation)
 Increased uptake : “hotspots”
 Revealing highly vascular lesions
Laboratory Investigations

 CBC , including WBC differential  elevated in osteosarcoma and Paget's

disease .
 ESR (Erythrocyte Sedimentation Rate)
 Serum acid phosphatase , PSA
 Elevated in Ewing’s sarcoma
 elevated in carcinoma of the prostate
 Serum calcium
 Serum protein electrophoresis
 Elevated in multiple myeloma and
metastatic bone disease  abnormal pattern in multiple myeloma
and metastatic bone disease
 Serum phosphorus
 Urinary Bence-Jones protein
 Lowered in hyperparathyroidism (with
"brown tumors" )  elevated in multiple myeloma.
 Serum alkaline phosphatase
Staging of Benign, Potentially Malignant,
and Malignant Neoplasms of Bone
 By Enneking in 1980,1983,1986  benign, potentially malignant, and malignant
neoplasms of bone
 Methods : plain radiography, CT scan (local and chest) MRI, and scintigraphy
(bone scan).
 Factors involved:
 The histological grade of the lesion – benign, potentially malignant, low-grade
malignancy, high-grade malignancy
 The size of the lesion and compartment(s) involvement
 Metastasis
 Enneking’s surgical staging system of bone sarcoma

 Purpose: to determine the prognosis ; to plan the ideal method of treatment (chemo,
radiation, surgical resection) , international standardization for clinical outcomes of
various forms of treatment
Biopsy
 Essential to avoid 2 serious errors: underdiagnosis and overdiagnosis
 Investigations by Mankin:
 Biopsies are improperly performed or misinterpreted
 Associated with preventable complications (2-12 times) when performed in a secondary referring hospital compared to
tertiary referral center.
 Biopsy
 performed by oncological orthopedic surgeon who is going to carry out patient’s definitive treatment ;
 should be longitudinal rather than transverse (extremity) ;
 should transgress minimum number of compartments  avoid contamination
 Must be adequate in size representative

 Necrotic tissue post neoadjuvant chemotherapy  appropriateness of chemotherapy chosen  better prognosis
 Final diagnosis and treatment  combined opinions of oncological orthopaedic surgeon, diagnostic imager, radiation
oncologist, medical oncologist, and pathologist.
Principles and Methods of Treatment

 Principles
1. Final evaluation (including staging and biopsy) : done by the oncological orthopaedic
surgeon who also provide definitive treatment in tertiary care orthopaedic oncological unit
2. Compassionate communication
3. Treatment must be based on accurate diagnosis
 failure to treat a patient early for a malignant lesion is serious ; yet needless radical surgery of a limb
on the basis of a mistaken diagnosis is also serious.
4. Prognosis and the choice of treatment method must consider quantity of patient’s remaining
life and quality of that life
5. Patient, relatives or both are involved in the decision-making process – advantages and
disadvantages of various treatment options should be presented beforehand.
6. Members of Interdisciplinary oncological unit  involved in diagnosis and treatment
7. Surgical methods must be planned meticulously
Methods of Treatment

 Surgical Procedure
 Chemotherapy
 Radiation Therapy (Radiotherapy)
Surgical Procedure
 Degree of resection:
 Intracapsular (intralesional) resection e.g. curettage
 Marginal resection
 Wide local resection
 Radical resection (all, or a large part of the involved bone plus
all involved soft tissue compartment[s])
 2 main types of radical resection : limb-sparing (limb-salvage) procedures and amputation (disarticulation)
 Limb-sparing procedure : more widely performed – survival rates are much the same, but required criteria
and complications differ
 Limb-sparing procedure criteria:
 No skip lesions
 Lesions are resectable without jeopardizing limb function
 Reconstruction of residual defect is feasible
 Reconstruction:
 Large bone allograft (complications: Infection, delayed or nonunion , late pathological fracture)
 Arthrodesis
 Custom-made endoprosthesis (complications : loosening , mechanical failure)

 Other reconstruction for LE in children: rotationplasty (Van Nes procedure)

 Pathological fracture through malignancy  will not heal (destruction exceeds reparative process)  need
rigid IM metallic fixation to relieve pain (palliative) ; may need MMA bone cement
Chemotherapy

 Effective chemotherapeutic agents : dramatic long-term survival improvement of

malignant neoplasm patients
 Neoadjuvant chemotherapy : pre-op ; adjuvant : post-op
 Percentage of necrotic cells in resected neoplasm after a course of neoadjuvant
therapy : effectiveness of chemotherapy and prognosis
 Toxic effects: neutropenia, thrombocytopenia, wound complications, infection,
nausea, alopecia, and delayed healing (of bone allografts).
 Sensitive : osteosarcoma, Ewing's sarcoma, malignant fibrous histiocytoma,
rhabdomyosarcoma.
 Resistant (unresponsive) : Chondrosarcoma, fibrosarcoma of bone, and soft
tissue sarcomas
 4 groups of chemotherapeutic agents:
 Alkylating agents (cyclophosphamide, cisplatin )
 Anti neoplasm antibodies ( doxorubicin, actinomycin D)
 Folate antagonists (methotrexate with citrovorum " rescue")
 Antimetabolites (mercaptopurine, 5-flurouracil, i.e. 5-FU)
Radiation therapy

 ionizing radiation  relatively selective destruction of the more rapidly

multiplying cells in malignant neoplasms and certain other conditions.
 Immature cells and undifferentiated cells : vulnerable ( radiosensitive)
 Radiation at therapeutic level  profound chromosomal changes in cells 
effects are apparent during next cell division (mitosis) fail to divide
 Neoplasm cells : rapid turnover early effects
 Cells at the bed of neoplasms (fibrous tissue and blood vessels)  fibrosis and
ischemia
 Disintegration of radium  alpha, beta, gamma rays  greatest ability to
penetrate tissues
 Quantitative physical unit : Gray (Gy) (=100 rad)
 Neoplasm of bone : relatively radioresistant  requires high dosage of radiation
(70 Gy or more , fractionated over a period of several weeks)
 Relatively radiosensitive: Ewing's sarcoma and malignant lymphoma of bone
 Relatively radioresistant: osteogenic sarcoma and chondrosarcoma

 Adverse effects of radiotherapy: epiphyseal plate damage with resultant growth

disturbance; radiation necrosis of bone with subsequent pathological fracture;
and radiation-induced malignancy (rare)
Osteosarcoma

 Extremely malignant neoplasm ; arises from primitive (poorly differentiated)

cells in the metaphyseal region of a long bone in young individuals. Also
referred to as osteogenic sarcoma  because of its genesis from the osteoblastic
series of primitive mesenchymal cells
 Relatively rare ; osteosarcoma is the second most common (being exceeded only
by myeloma).
 Majority : children, adolescents, and young adults. The most common sites : the
lower end of femur, upper end of tibia or fibula, upper end of humerus, and pelvis.
 In older persons : a complications of Paget's disease  poor prognosis
 Osteosarcoma grows rapidly ; locally destructive , may be
osteosclerotic or osteolytic.
 Erodes the cortex of the metaphyseal region  more risk for
pathological fracture  grows wildly beyond the confines of
the bone  lifts the periosteum  reactive bone forms in the
angle between elevated periosteum and bone  Codman's
triangle
 Blood vessels radiate through the neoplasm from the cortex to
the elevated periosteum  Combination of reactive bone and
neoplastic bone deposited along blood vessels  "sunburst"
appearance (50% of osteosarcomas) (Fig. 14.8). Osteosarcoma
metastasizes to the lungs very early in the course of its
development
 Symptom : pain (initially mild and intermittent
 more severe and constant) , interfering joint
function, mass develops rapidly ; tender (Fig.
14.2 ) ; very vascular ; warm overlying skin;
dilated superficial skin;

 ALP : usually elevated

 Radiology: often (but not always) characteristic.

 To determine the intraosseous and extraosseous
extent : MRI. Scintigraphy is useful in detecting
"skip" lesions ; CT scans of the chest : detects
minute pulmonary metastases
 All three modalities are required to complete the
staging of a given osteosarcoma.
 During the past few decades  combination of neoadjuvant
(preoperative) and adjuvant (postoperative) chemotherapy +
effective surgical procedures  dramatic increase in the 5-year
disease-free survival to more than 70%.
 The chemotherapeutic agents being used include high-dose
methotrexate , adriamycin, doxorubicin, cisplatin, and ifosphamide.
 Surgical : limb-sparing procedure (more frequently performed) ;
radical resection (amputation/disarticulation)  long-term survival
rate similar with higher complication rate with limb-sparing
operation

 Retinoblastoma ( RB1 ) gene tumor-suppressive gene ; the

absence leads to the development of the retinoblastoma as well as
osteosarcoma.
 Variants of Osteosarcoma

 Rare subtype

Telangiectatic Small-cell Osteosarcoma

• Ro : completely lytic, with cortical • Ro : classic
disruption and soft tissue extension

• Histo : scant osteoid matrix • Histo : sheets of small, round blue

cells
• Th/ same as conventional
osteosarcoma • Th / chemo different – similar to other
round blue-cell tumor (e.g. Ewing’s
sarcoma)
• Progn : similar outcome
• Progn : slightly worse
Parosteal Osteosarcoma
 Low-grade osteosarcoma from osteoblastic cells of the periosteum ;
afflicts adolescent and young adult ; at the distal end of femur ;
radiographically dense lesion
 Pain is not an early clinical feature ; pathological fracture rare ;
metastasis late hence prognosis better
 Early total resection  permanent cure in 80% patients
 Periosteal Sarcoma
 more aggressive than the parosteal
variation ; Tends to erode the
cortex from the outside , may also
invade the soft tissues.

 Treatment is similar to parosteal

osteosarcoma’s ; adjuvant
chemotherapy may be added (if
higher grade is found
histologically)
 ..some additions from WHO
 LGCOS – Low Grade Central Osteosarcoma
 Low-grade malignant bone-forming neoplasm, originates within the intramedullary
cavity
 Rare ; 1-2% of all osteosarcomas ; young adults (third decade of life)
 Metaphysis of long bones (femur and tibia most common)
 Clinical features
 Pain ; pre-op period tends to be longer than conventional osteosarcoma ; average > 2 years
 Imaging : large intramedullary expansile lytic and/or sclerotic lesions with coarsened
incomplete trabeculations
 Dd/ fibrous dysplasia ; desmoplastic fibroma, low-grade fibrosarcoma
 Diagnostic criteria
 Essential: bone tumor with compatible imaging; intramedullary location; predominantly
fibroblastic osteosarcoma with mild nuclear atypia and well-formed neoplastic bony
trabeculae. \
 Desirable: MDM2 amplification.
 Prognosis/
 Good if widely resected
 Metastatic rate <5%
 5 and 10-year survival rate : 90% and >80%
 Chemotherapy : for dedifferentiated tumours
 High-grade Surface Osteosarcoma
 High-grade malignant bone-forming neoplasm arising
on the surface of the bone
 <1% of osteosarcoma, male predominance; second
decade of life
 Most common sites : femur, tibia, humerus ; diaphyseal
or diametaphyseal
 Clinical features : swelling and pain of short duration
 Imaging : radiodense mass extending into soft tissue from
the bone surface; ill-defined immature ossification ;
radiating bone spicules ; cortical erosion and medullary
involvement in half of patients
 Macroscopic appearance and histopathology

 Diagnostic criteria :
 Essential : bone tumour with compatible imaging,
histologically high-grade osteosarcoma, arising on the
surface of the bone without a substantial intraosseus
component.

 Prognosis and prediction:

 5 year survival rate : 62%
 Good response to neo-adjuvant + localized  good
prognosis
Secondary osteosarcoma
 Osteosarcoma arising in abnormal bone
 Related terminology : Paget sarcoma; osteosarcoma in
Paget disease of bone (PDB) , radiation-associated
osteosarcoma
 Localization : femur (34%), pelvis (24%), humerus
(24%) ; metaphyseal in long bone ; at the site of
radiotherapy : flat bone of chest wall and pelvis
 Signs and symptoms : pain, enlarging mass ; history of
bone condition
 Imaging: lytic (65%) destruction of pagetic bone ; large
extraosseus mass
 Epidemiology : most common sarcoma associated with PDB
 50% of bone sarcomas in patients aged > 50 years are secondary to PDB
 Paget sarcoma in PDB : 0.7-6.3 %
 6th-7th decade of life
 Radiation- associated osteosarcoma : after treatment of tumours ; 0.03% of radiotherapy patients
 Etiology
 Ionizing irradiation ; bone infarction ; orthopaedic implants
 Macroscopic appearance and Histopathology
 Similar as osteosarcoma
 Essential and desirable diagnostic criteria
 Essential: compatible imaging; history of previous radiation therapy or an underlying bone abnormality; osteosarcoma
histology.
 Desirable: histological evidence of underlying abnormal bone
 Prognosis
 Worse than conventional osteosarcoma ;
 Paget sarcoma :2-year survival rate : 25 % ; 29% already metastasized to lungs
 radiation-associated osteosarcoma : 5-year survival rate 10-32%
Ewing’s Tumor (Ewing’s Sarcoma)
 Rapidly growing malignant neoplasm , arises from
primitive cells of the bone marrow ; usually in the
medullary cavity of long bones.
 5% of malignant neoplasms of bone ; develops in
children, adolescents, and young adults ; in the
femur, tibia, ulna, and metatarsals.
 Beginning within the medullary cavity 
perforates the cortex  elevates the periosteum 
repeated elevation and consequent reactive bone
formation  laminated, or "onionskin" appearance
seen radiographically
 Ewing's tumor metastasizes early ;
 Microscopically  poorly differentiated round cells of marrow origin , contain
intracellular glycogen  detected by acid-Schiff stain.
 Ewing's tumor grows rapidly  outgrows its blood supply  central areas
become necrotic  enter the bloodstream  systemic manifestations e.g. slight
fever, moderate leukocytosis, elevated sedimentation rate ; compromised local
blood supply  resultant avascular osteonecrosis.
 The principal symptom : pain of progressive severity.
 Palpable soft tissue mass , moderately tender ; relatively
little bone destruction , considerable reactive bone from the
periosteum
 The pain, local tenderness, systemic manifestations and
radiographic features differential diagnosed with
chronic osteomyelitis and eosinophilic granuloma.
 Certain method of diagnosis : surgical biopsy and
histological examination of representative samples of the
lesion.
 Prognosis : extremely grave - mortality rate within the first few years after
diagnosis was approximately 95%.

 Treatment : combination of neoadjuvant chemotherapy and radical surgical

resection (either a limb-sparing procedure, when feasible, or amputation) 
followed by adjuvant chemotherapy with or without radiation therapy
 increased the 5-year disease-free rate from 5% to more than 50%.
.. Some additions from WHO – ST and
Bone Tumours
 Ewing Sarcoma : small round cell sarcoma showing gene fusions
involving one member of the FET family of genes (usually EWSR1)
and a member of the ETS family of transcription factors.
 Localization : diaphysis and diaphyseal-metaphyseal portions of
long bones, pelvis, ribs, any bones.
 Clinical features: locoregional pain, palpable mass, pathological
fracture , fever.
 Ro : poorly defined osteolytic-permeative lesions + classic
multilayered periosteal reaction (onion-skin appearance).
 Epidemiology : the second most common malignant bone tumor in
children and young adults after osteosarcoma , M:F ratio of 1.4:1
 Macroscopic appearance : The cut surface of untreated Ewing
sarcoma specimens is greyish-white and soft, and it frequently
includes areas of hemorrhage and necrosis
 Diagnostic criteria
 Essential: small round cell morphology; CD99 membranous
expression.
 Desirable: FET-ETS fusion detection (in selected cases).
 Prognosis and prediction
 With current multimodal therapy : 65-70% cure rate for localized
disease.
 Metastatic and early-relapsing tumors have a poor prognosis,
with a 5-year survival rate of < 30%.
 The presence of metastases appears to be the main prognostic
factor. Other negative prognostic factors include the anatomical
location of the tumor, such as the pelvis. Complete pathological
response to neoadjuvant chemotherapy is a favorable
prognostic factor {
Giant-cell Tumor of Bone
(Osteoclastoma)
 Potentially malignant, and sometimes frankly malignant, neoplasm ; in the
cancellous end of long bones ; young adults

 Develop in the region of the former epiphysis of long bones  Rare before the
age of 20 years. Most common sites : lower end of radius, upper end of tibia,
lower end of femur, and upper end of humerus. Usually extends to the articular
cartilage
 GCT : Locally destructive neoplasms. The cancellous and cortical
bone are resorbed from the inside  periosteum deposits bone on the
outside -- > the end of the bone eventually becomes expanded.
 Two-thirds of these neoplasms are benign in their behavior, one sixth
are locally aggressive, and one sixth become frankly malignant  but
tend to metastasize late.
 Areas of hemorrhage within the lesion are common. Microscopically,
osteoclastomas consist of a vascular network of stromal cells and
large numbers of multinucleated giant cells.
 Complains: local pain ; some disturbance of joint function .
 Radiographic appearance ; local bone destruction , expansion of the end of the
bone
 Treatment:
 GCT tends to recur after simple curettage. Therefore, the original operation should be
as aggressive as necessary. A local recurrence : indication for radical excision of the
entire lesion in a limb-sparing procedure  followed by replacement with methyl
methacrylate, an autogenous bone graft, an osseocartilaginous allograft, or a
custom-made endoprosthesis as a joint replacement. For the most aggressive giant
cell tumors, or for local recurrence, radiotherapy is one option that can be used in an
attempt to avoid amputation.
GCT of Bone (WHO Classific. Of Tumor)

 Definition : a locally aggressive and rarely metastasizing neoplasm composed of

neoplastic mononuclear stromal cells with a monotonous appearance admixed with
macrophages and osteoclast-like giant cells. A small subset of cases are malignant.

 Essential and Desirable diagnostic criteria

 Essential: bone tumour with compatible imaging; an osteolytic circumscribed tumour
involving the epiphysis, generally in a skeletally mature individual; numerous large
osteoclasts together with a mononuclear cell neoplastic component without atypia.
 Desirable: Detection of H3.3 p.Gly34—mutated cells