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GIANT CELL TUMOR

HESTI KURNIA
03013093
Definisi
Sinonim: osteoclastoma
Suatu lesi jinak, agresif dan destruktif
Ditandai dengan proliferasi sel-sel mononuklear pada stroma
dan sel datia yang tersebar menyerupai osteoklas.
Insidensi
5% dari seluruh tumor tulang primer
21% dari seluruh tumor jinak tulang.
80% jinak, 20% ganas
Insiden: 5 8% tumor tulang ganas, 15% tumor tulang
jinak
Jinak predominant untuk wanita, ganas biasanya laki laki
Usia: 20 40 th
Rasa nyeri dan gangguan gerak sendi
Terjadi umumnya di tulang panjang: femur distal, tibia
proksimal, radius distal, humerus proksimal (os sacrum
8%)
ANATOMI TULANG PANJANG
PATOGENESIS
PATOGENESIS
MANIFESTASI KLINIS
Pain and swelling
Pemeriksaan fisik: teraba massa dan penurunan ROM
CAMPANACCI RADIOGRAPHIC GRADING

Grade 1 - tumor has well


marinated border of a thin rim
of mature bone, and the
cortex is intact or slightly
thinned but not deformed.
Grade 2 - tumor has relatively
well defined margins, but no
radio opaque rim, cortex is thin
and expanded but is present.
Grade 3 - cortex is perforated
with extension of tumor into soft
tissue.
Enneking staging of Giant cell tumor
Based on clinical radiological and histopathological
features

Stage 1- LATENT(10 - 15%)


Patient is asymptomatic, discovered incidentally.
May be associated with pathological fractures.
Radiologically-tumor is intracapsular, with well defined
margins and sclerotic rim. no cortical destruction.

Stage 2 - ACTIVE ( 70-75 %)


Patient is symptomatic.
Often associated with pathological fracture.
Radiologically-intracapsular, has expanded or thinned
out cortex, but there is no breakthrough the cortex.
Stage 3 (aggressive) : 10 - 15 %
Symptomatic
Rapidly growing mass
Radiologically-Extracapsular, has cortical
perforation
Will show intense vascularity on angioram
DIAGNOSIS
Lab Darah: Kalsium, fosfat, alkali fosfatase.
Radiologi
Foto X-ray
CT Scan
MRI
Angiografi
Biopsi
FOTO X-RAY
Lesi di metafisis meluas kearah subartikuler pada epifisis,
sudah menutup
Lesi lusen eksentris
Sifat ekspansif dengan soap bubble appearance
Kadangkadang sulit membedakan antara jinak dan ganas
CT SCAN
MRI
ANGIOGRAFI
HISTOPATOLOGIS

KARAKTERISTIK SEL
Tipe 1: sel stroma mononuklear menyerupai
fibroblas, merupakan sel tumor
Tipe 2: dari monosit/makrofag, merupakan
prekursor giant cells
Tipe 3: multinucleated giant cells (>50 nuklei),
memiliki karakteristik seperti osteoklas
(mengabsorbsi tulang, mengandung enzim acid
phosphatase, carbonic anhydrase II)
MAKROSKOPIS
Epiphyseal end of long bone will be
expanded with thining of periosteum and
cortex, being easily broken by handling.
Composed of ragged, very friable,
readily bleeding tissue containing
variously sized cavitations and small cysts.
Colour - varies from reddish brown to
chocolate color in which vascular tissue
predominates, to greyish or mottled where
connective tissue is major component.
No evidence of periosteal new bone
formation.
The inner wall of tumor is lined by a fibrous
capsule from which the septae extend
inwards to partition the tumor.
Osteoblast Precursor Malignant Stromal Cells ++ RANKL
Expression Recruitment of Osteoclasts.

Round to Oval Mononuclear


Cells

Large Osteoclast Giant Cells:


Multinucleated, up to 50.
From monocytes.
TREATMENT
Non-operative
Can be used to augment or replace surgical
management depending on the specific clinical
scenario medications
1. Bisphosphonates: osteclast inhibitors which may
decrease the size of the defect in giant cell
tumors
2. Denosumab
monoclonal antibody against RANK-ligand
recent clinical trials suggest denosumab can decrease
the size of the bone defect in giant cell tumor
90% tumor necrosis
Operative
Extensive curettage and
reconstruction (with adjuvant treatment)
Indications
Lesions amenable to currettage
If no cortical breakthrough treat with curettage
and cementing
If significant cortical breakthrough
consider intercalary resection (with free fibular
graft) vs. amputation
10-30% recurrence with curettage
alone verses 3% with adjuvant
treatment (phenol, hydrogen
peroxide, argon beam, etc)
Curettage with bone
cementing
En bloc excision
Initial procedure of choice in more aggressive tumors

2 cm of normal bone is also excised

Defectt are filled with cancellous bone grafts, allograft or


prothesis.
Resection and
reconstruction with
Autograft
Resection of tumor in lesions of
sacrificable part

lower end ulna


upper end of fibula
phalanges
metatarsal rays

RECURRENCE IS ALMOST NILL


Amputation
Indications
hand lesions with cortical breakthrough who are not
amendable to intercalary resection
KOMPLIKASI
Keganasan (<1%)
Rekurensi (15 25%)
Fraktur patologis
Infeksi post operasi
TERIMA KASIH

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