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MUSCULOSCELETAL

Dr. Dyah
Marianingrum,M.Kes,Sp.PA

OUTLINES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

BONES
Bone Modeling, Remodeling, and Peak Bone Mass
Bone Growth and Development
Developmental Abnormalities in Bone Cells, Matrix, and
Structure
MALFORMATIONS AND DISEASES CAUSED BY DEFECTS IN
NUCLEAR PROTEINS AND TRANSCRIPTION FACTORS
DISEASES CAUSED BY DEFECTS IN HORMONES AND
SIGNAL TRANSDUCTION MECHANISMS
DISEASES ASSOCIATED WITH DEFECTS IN EXTRACELLULAR
STRUCTURAL PROTEINS
Type 1 Collagen Diseases (Osteogenesis Imperfecta)
Diseases Associated with Mutations of Types 2, 9, 10,
and 11 Collagen
DISEASES ASSOCIATED WITH DEFECTS IN FOLDING AND
DEGRADATION OF MACROMOLECULES
Mucopolysaccharidoses
DISEASES ASSOCIATED WITH DEFECTS IN METABOLIC
PATHWAYS (ENZYMES, ION CHANNELS, AND
TRANSPORTERS)
Osteopetrosis
DISEASES ASSOCIATED WITH DECREASED BONE MASS
Osteoporosis
DISEASES CAUSED BY OSTEOCLAST DYSFUNCTION
Paget Disease (Osteitis Deformans)
DISEASES ASSOCIATED WITH ABNORMAL MINERAL
HOMEOSTASIS
Rickets and Osteomalacia
Hyperparathyroidism
Renal Osteodystrophy
Fractures

Osteonecrosis (Avascular Necrosis)


InfectionsOsteomyelitis
PYOGENIC OSTEOMYELITIS
TUBERCULOUS OSTEOMYELITIS
SKELETAL SYPHILIS
Bone Tumors and Tumor-Like Lesions
BONE-FORMING TUMORS
Osteoma
Osteoid Osteoma and Osteoblastoma
Osteosarcoma
CARTILAGE-FORMING TUMORS
Osteochondroma
Chondromas
Chondroblastoma
Chondromyxoid Fibroma
Chondrosarcoma
FIBROUS AND FIBRO-OSSEOUS TUMORS
Fibrous Cortical Defect and Non-Ossifying Fibroma
Fibrous Dysplasia
Fibrosarcoma Variants
Miscellaneous Tumors
EWING SARCOMA/PRIMITIVE NEUROECTODERMAL
TUMOR
GIANT-CELL TUMOR
ANEURYSMAL BONE CYST
METASTATIC DISEASE

JOINTS
SOFT-TISSUE TUMORS AND TUMOR-LIKE LESIONS

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Tulang (sistem skeletal)


memiliki fungsi yang vital
dalam kehidupan:
homeostasis mineral
tempat elemen2
hematopoietik
penunjang mekanis utk
pergerakan
melindungi visera
menentukan ukuran dan
bentuk tubuh

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

Tulang merupakan:
Organ :
t.d. jar. tulang,
tulang rawan,
lemak, sumsum
tulang,
pemb.darah, saraf
dan jar. ikat fibrous
Jaringan :
secara mikroskopik
t.d. sel2 tulang.

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi


Tulang (sistem skeletal) :
struktural
proteksi
metabolik
Disusun oleh 206 buah
tulang:
Axial : cranium, columna
vertebralis, thoraks
Appendicular :
ekstremitas
Berdasarkan bentuknya:
Tulang panjang
Tulang pipih
Tulang pendek
Ireguler
Sesamoid
Berdasarkan embriologik:
Membranous
Endokondral

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

Secara makroskopik t.d. :


Substantia compacta : padat,
lapisan luar tulang, meliputi
80% sistem skeletal. Fungsi
utama biomekanik.
Substantia spongiosa
(trabecular, medullary bone) :
berongga, terdapat pd ujung2
tulang panjang.
Anatomy of bone. A schematic representation of cortical and trabecular bone. The longitudinal section (left)
shows the vasculature entering the periosteum via the periosteal perforating arteries and coursing through the
bone perpendicular to the long axis in Volkmann canals. The vessels that proceed longitudinally, or parallel to
the long axis, are located in haversian canals. Each artery is accompanied by a vein. Within the cortex,
osteocytes reside in lacunae, and their cell processes extend into the canaliculi. The cross-sectional view (right)
illustrates the various types of lamellar bone in the cortex. Circumferential lamellar bone is located adjacent to
the periosteum and borders the marrow space. Concentric lamellar bone surrounds the central haversian canals
to form an osteon. Each layer of the concentric lamellar bone displays a change in the pitch of the collagen
fibers, such that each layer has a different arrangement of collagen. The interstitial lamellar bone occupies the
space between osteons. The marrow space is filled with fat, and its trabecular bone is contiguous with the
cortex. Multinucleated osteoclasts are present, and palisaded osteoblasts surround the bone surfaces. The
perforating arteries from the periosteum and the nutrient artery from the marrow space communicate within

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Anatomi & Histologi

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Sebagian besar terdiri dari matriks organik (osteoid)


dan mineral calcium hydroxyapatite memberi
kekuatan dan sifat keras pada tulang.
Tulang merupakan jaringan yg bersifat dinamis
continuously resorbed, renewed, and remodeled :
proses ini dilakukan sel2 tulang yg diatur oleh
sejumlah faktor transkripsi, sitokin, dan faktor
pertumbuhan.

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Jenis sel2 tulang :


Sel osteoprogenitor : sel tunas mesenkim
pluripoten yang terletak di sekitar permukaan
tulang
Osteoblas dan sel pelapis permukaan terletak di
permukaan tulang : untuk sintesis, transport dan
menyusun banyak protein matriks, serta memulai
proses mineralisasi.
Osteosit merupakan transformasi dari osteoblas
yg telah dikelilingi deposit matriks organik.
Osteoklas : sel yg berperan meresorpsi tulang.

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Figure 26-1 Bone cells and their interrelated activities. Hormones, cytokines, growth factors, and signal-transducing
molecules are key in their formation and maturation, and allow communication between osteoblasts and osteoclasts.
Bone resorption and formation in remodeling are coupled processes that are controlled by systemic factors and local
cytokines, some of which are deposited in the bone matrix. BMP, bone morphogenic protein; LRP5/6, LDL receptor
related proteins 5 and 6.

BONES

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Figure 26-2 A, Active osteoblasts synthesizing bone matrix. The surrounding spindle cells represent osteoprogenitor
cells. B, Two osteoclasts resorbing bone.

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Bone Modeling, Remodeling, & Peak Bone Mass


Sekumpulan osteosit, osteoblas dan osteoklas bekerja secara
terpadu mengontrol pembentukan dan resorpsi tulang unit
fungsional : basic multicellular unit (BMU).
Pada awal kehidupan, saat tulang tumbuh dan membesar
(modeling) pembentukan tulang predominan.
Saat tulang telah mencapai maturitas, penguraian dan
pembaruan tulang yg berperan utk maintenance : remodeling
kemungkinan dimulai di tempat2 yg mengalami fatigue dan
microdamage
pada orang dewasa, sekitar 1 juta BMU aktif setiap saat
melakukan remodeling atau mengganti 10% tulang setiap
tahun.
Peak bone mass tercapai pada usia dewasa muda setelah
berhentinya pertumbuhan tulang

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Bone Modeling, Remodeling, & Peak Bone Mass


Peak bone mass tercapai pada usia dewasa muda setelah
berhentinya pertumbuhan tulang
ditentukan oleh berbagai faktor :
polimorfisme reseptor vitamin D dan LRP5/6
nutrisi
aktifitas fisik
usia
status hormonal
sejak awal dekade keempat : resorpsi > formasi
penurunan massa tulang terus-menerus.

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Bone Growth & Development

Chapter 26 - Bones, Joints, and Soft-Tissue Tumors

Bone Growth & Development

Figure 26-5
Active growth plate with ongoing
enchondral ossification.
1, Reserve zone.
2, Zone of proliferation.
3, Zone of hypertrophy.
4, Zone of mineralization.
5, Primary spongiosa.

SOFT TISSUE

Diagnosa

Soft tissue tumor

Lymphangioma