NIP.132313010 Berdasarkan bentuknya tulang dibagi menjdi : tulang panjang (mis. femur, humerus), tulang pipih (mis. Pelvis), dan tulang pendek ( mis, phalang). Tulang panjang dibagi menjadi beberapa regio : diaphysis, epiphysis, metaphysis. Penumbuhan tulang berawal dari epiphyseal plate, dimana dimulainya endochondral ossification. Epiphyseal plate most common site of occurrence of most primary bone tumors. Histologi : tulang matur terdiri dari outer compact layer ( cortex, cortical bone, compact bone) dan central spongy region ( spongiosa, medulla, cancelous bone) Osteoporosis - Penurunan massa tulang - Tidak dapat memperbaiki maupun mempertahankan massa tulang - After menopause estrogen deficiency - Causes : multiple Osteomalacia - Unmineralized bone matrix resulting from a diminished rate of mineralization - Congenital and acquired metabolic abnormalities - Penurunan serum calcium, phosphorus, dan keduanya. Bacterial osteomyelitis may be caused by a large variety of microorganisme 70 – 90% due to coagulase-positive staphylococci. Other microorganisme : klebsiella, proteus, pseudomonas, streptococcus, etc. Local or exogenous causes, or through to hematogenous route. Can be acute, subcute or chronic Lytic lesion in metaphysis with periosteal reaction. In spine, the disc space is narrowed and the vertebral end plates are eroded. Can resemble malignant bone tumor because of lytic and regenerative changes. May see bone necrosis of central region (sequestrum) and new bone forming around it (involucrum). Inflammation dominated by lymphocytes and plasma cells. Infection of a prosthesis is a different process, usually neutrophilic. Bacterial stains are rarely helpful. Treatment is antibiotics and surgical drainage. About 5% of acute cases develop into chronic osteomyelitis. Merupakan infeksi oleh Mycobacterium tuberculosis. Hematogenous infection Dapat terjadi dimana saja, biasanya spine and hip pada anak-anak dan knee pada orang dewasa. Radiologi joints, destruction of subchondral bone. Mikroskopis Organisms, bacilli, 0.3 - 4 microns. BTA (+) . Ditemukan pembentukan tuberkel, terdiri dari serbukan limfosit, proleferasi sel epithelioid, sel datia langhans dan nekrosis perkijuan. Infarct Aseptic (avascular) bone necrosis Osteochodritis dissecans Radiation necrosis Usually ages 30's - 40's. Predominantly male. Either medullary (in marrow), or subarticular bone. Usually femoral or humeral head. 80% associated with alcohol abuse or steroid therapy. Joint pain and osteoarthritis when near joint. MRI shows marrow edema early. Conventional x-ray shows no abnormalities for months, followed by increased density. If involving joint, possibly subchondral radiolucent crescent sign. Loss of nuclei in bone. Necrosis of marrow fat and hematopoietic tissue may be easier to appreciate. Surrounding granulation tissue and sclerotic bone. Complications include secondary osteoarthritis, fracture, secondary infection, sarcoma. The terms avascular/aseptic necrosis are often used when the infarct involves the end of a long bone. See also Osteochondritis dessicans. If infarct underlies joint, it leads to osteoarthritis. Usually young adults. Most common site is the lateral aspect of medial femoral condyle. Most cases are probably related to trauma. Small area of bone necrosis adjacent to articular surface, totally or partially separated from original site. Specimen is a portion of bone with articular cartilage. A loose body is formed when the detached fragment remains viable in the joint space (the bone dies but the cartilage survives) 90% > 55 tahun Focal or diffuse. Lumbosacral spine, pelvis, skull, and long bones. Rare in ribs. Most common in England, Australia, and Western Europe. May be caused by slow virus (seen by electron microscopy) in osteoclasts. Radiolucent early, then radiodense due to coarse trabeculae. One of the few lesions that can uniformly enlarge bone. Resorptive phase (early), increased osteoclast and osteoblast activity with fibrous stroma. Intermediate and late phase, excessive osteoblast activity with poorly organized bone, thick trabeculae, and numerous, prominent, scalloped remodeling lines in a mosaic pattern. Benign by itself, but carries a high risk of sarcoma, usually osteosarcoma, 1% for single lesions, 10% for multiple lesions (accounts for 3% of osteosarcomas). Early changes. There is prominent osteoclastic activity, resulting in bone resorption. A well-established case, with thick, irregularly shaped bone trabeculae. = Osteoarthrosis, Degenerative joint disease. Increasing frequency with age. Most commonly hip and knee. Charcot joint, older patients. Gradual onset of joint pain and stiffness. X-ray reveals loss of cartilage, sclerosis of subchondral bone, possibly osteophytes, and subchondral cysts. Early/mild changes occur on surface of cartilage, decreased basophilia, vertical clefts, loss of chondrocyte nuclei, reparative changes (chondrocyte clones). Late/severe changes include erosion of cartilage, myxoid cysts within trabecular bone (subchondral cyst), thickening of underlying bony trabeculae, formation of bone spurs at joint edge. Loose bodies are pieces of broken off cartilage and bone. Affects about 1% of world's population. Peak incidences occur in 30's and 50's, though can affect all ages. Affects women 3 - 5 times more often than men. Joints of feet and hands are nearly always affected. Frequently affected joints include elbows, knee, wrists, ankles, hips, spine and temporomandibular articulations. Typically slow and insiduous onset in more than half of patients. 10% have acute onset. Often family history. 70% are HLA-DR4. Combined features of RA, splenomegaly and neutropenia known as Felty's syndrome. Earliest changes occur in synovium, hyperemia, proliferation of synovial lining cells and infiltration by plasma cells and lymphocytes (forming pannus, causing erosion of joint cartilage). Second phase, synovium and granulation tissue penetrate bone forming erosions, subchondral cysts and osteoporosis, resulting in fractures. Following cartilage destruction, pannus bridges apposing bones, forming fibrous ankylosis and eventually bony ankylosis. Extraarticularmanifestations include rheumatoid nodules, composed of necrotic center with fibrin, surrounded by histiocytic inflammatory reaction, often arranged in palisading fashion. Other pathologic findings include necrotizing arteritis and amyloidosis. Klasifikasitumor yang direferensikan banyak center adalah klasifikasi menurut WHO. Pada umumnya keganasan pada tulang terjadi de novo. Terdapat beberapa lesi jinak pada tulang yang dapat menjadi predisposisi menjadi keganasan, antara lain paget’s disease, chondromatosis, osteochondromatosis, fibrous dysplasia dan osteofibrous dysplasia. Limaparameter yang penting diketahui dalam keganasan tulang adalah : umur, jenis tulang, area tulang ( epiphysis, diaphysis, metaphysis ), Ro appearance, microscopic appearance. Osteoma merupakan tumor jinak tulang, yang biasanya terjadi pada daerah tulang pipih misalnya tengkorak dan tulang wajah. Biasanya terjadi pada umur 40 – 50 tahun dengan ratio laki-laki dan wanita adalah 2:1 Penderita dengan Gardner’s syndrome , biasanya terjadi multipel osteoma dgn beberapa kelainan ( intestinal polyposis dan soft tissue tumors) Secara mikroskopis tampak tulang matur, padat dengan gambaran lamellarnya, tumbuh hiperplastis. Benign bone neoplasm 10 – 30 yrs 2:1 male-female ratio Inntense pain most prominent symptoms, more intense at night, relieved by NSAID Every bone Most are centered in the cortex (85%) X-ray : radiolucent central nidus, seldom larger than 1,5 cm. This nidus is surrounded by a peripheral sclerotic reaction Microscopically : sharply delineated central nidus is composed of more or less calcifiedosteoid lined by plump osteoblasts and growing within highly vascularized connective tissue Osteosarcoma Osteosarcoma atau osteogenic sarcoma merupakan tumor ganas primer tulang yang paling banyak terjadi. Biasanya mengenai penderita usia muda yaitu 10 – 25 tahun. Pada usia tua, insidensi terbanyak terjadi pada usia lebih dari 40 tahun. Tumor ini lebih sering terjadi pada laki-laki dibandingkan wanita. Predisposing factor : 1. Paget’s disease 2. Radiation exposure 3. Chemotherapy 4. Preexisting benign bone lesion 5. Foreign bodies 6. Trauma Arising de novo Metaphyseal area of the long bones, particularly the lower end of the femur, the upper end of the tibia, and the upper end of the humerus. From its usual origin in the metaphysisof a long bone, the tumor may : 1. Spread along the marrow cavity 2. Invade the adjacent cortex 3. Elevate or perforate the periosteum 4. Extend into the soft tissues 5. Extend into the epiphysis 6. Extend into the joint space 7. Form satellite nodules independent from main tumor mass 8. Metastasize trough the bloodstream to distant sites, particularly the lung Molecular genetic features : 1. The most common cytogenetic abnormalities detected in osteosarcoma involve chromosomes 1,2,6, 12and 17. 2. Mutations of p53 have been found in about 20% of osteosarcoma The key feature for the diagnosis in the detection of osteoid and/or bone (calcified osteoid) produced directly by the tumor cells without interposition of cartilage. Osteoid is recognized by its eosinophilic- staining quality, its glassy appearance, irregular contour, surrounded by a rim of osteoblast. Microscopic variants and special types : 1. Telengiectatic 2. Small cell 3. Fibrohistiocytic 4. Anaplastic 5. Well differentiated intramedullary Other variant of osteosarcoma are defined on the basis of t0pographic, clinical, or radiographic features, or combination of them : 1. Parosteal osteosarcoma 2. Periosteal osteosarcoma 3. Osteosarcoma of the jaw 4. Osteosarcoma in paget’s disease Pathologic diagnosis before instituting definitive therapy : biopsy Needle biopsy in experienced hands is extremely reliable Laboratory test are of no great value in the diagnosis of osrteosarcoma. The only abnormality detected with some frequency is elevation of serum alkaline fosfatase The therapy of osteosarcoma of the extremities has usually consisted of amputation or disarticulation, depending on the location of the tumor. A common benign cartilaginous tumor Most frequently in the small bones of hands and feet, particularly the proximal phalanges About 30% chondromas are multiple. Multiple enchondromas having predominantly unilateral distribution ollier’s disease The association of multiple endchondromas with soft tissues hemangiomas Maffuci’s syndrome Microscopically are composed of mature lobules of hyaline cartilage. Osteochondroma Osteochondroma atau disebut osteochondromatosis bila terjadi multipel, merupakan tumor jinak tulang yang paling banyak terjadi. Biasanya mengenai usia muda yaitu 10 – 30 tahun. Tumor ini terjadi pada daerah metafise pada tulang panjang yaitu femur, tibia, maupun humerus. Secara radiologis tampak tulang dengan exostosis, sessile atau dengan tangkai. Biasanya kurang dari 5 cm. Adanya kemungkinan keganasan apabila cartilagenous cap lebih dari 3 cm, irregular margins of cartilage cap, lucent zones pada lesi. Mikroskopis tampak cartilage cap ( jarang lebih dari 1 cm) yang diliputi oleh jarinagn fibrosa. Dibawah cartilage cap, terdapat endochondral ossification zone yang berhubungan dengan bony pedicle. Pedicle menyambung dengan medulla tulang. Chondrosarcoma
Chondrosarcoma merupakan tumor ganas
tulang rawan. Biasanya terjadi pada usia 30 – 60 tahun. Predileksi tumor ini adalah tulang pelvis, costa, femur, humerus dan vertebrae. Dibagi menjadi 2 kategori mayor yaitu : conventional chondrosarcoma dan chondrosarcoma variants Conventional chondrosarcoma : central, peripheral, juxtacortical Microscopically : show remarkably wide range of differentitation, the common denominator being the production of cartilaginous matrix and the lack of direct bone formation by the tumor cell. This range in differentiation is the basis for the grading of these tumor into well, moderately, and poorly differentiated. Chondrosarcoma variants : 1. Clear cell chondrosarcoma 2. Dedifferentiated chondrosarcoma 3. Mesenchymal chondrosarcoma Giant Cell Tumor of the Bone Giant Cell Tumor atau disingkat dengan GCT pada tulang, b iasanya terjadi pada usia 20 – 40 tahun. Tumor ini paling sering terjadi pada wanita. Biasanya terjadi pada tulang panjang, dan dapat mengenai metafisis ataupun epifisis. Radiologis ditemukan lesi osteolitik dengan batas tegas, dan memberikan gambaran ‘soap bubble’ Secara mikroskopis tampak massa tumor terdapat banyak osteoclast-type giant cells dengan jumlah inti yang banyak (20-30). Ditemukan pula sel bentuk spindel yang plump (gemuk ) dengan inti mempunyai karateristik yang sama dengan inti pada giant cell, mitosis normal dan tidak ditemukan pleomorphism. Dapat ditemukan daerah yang nekrotik maupun perdarahan. Rhabdomyosarcoma merupakan tumor ganas yang berasal dari sel otot lurik. Tumor ini dapat terjadi pada anak-anak maupun usia tua. Pada anak-anak biasanya mempunyai predileksi terutama di kepala, sedangkan usia tua biasanya di ekstremitas. Terdapat 3 kategori mayor rhabdomyosarcoma yaitu pleomorphic, embryonal, dan alveolar rhabdomyosarcoma. Secara mikroskopis diagnosis rhabdomyosarcoma adalah ditemukan adanya sel rhabdomyoblast, yaitu sel dengan sitoplasma yang eosinofil dan dengan inti yang pleomorifk, hiperkromatis dan banyak ditemukan mitosis. Dapat ditemukan pula sel limfosit, sel plasma dan sel eosinofil Fibrosarcoma
Fibrosarcoma adalah tumor ganas jaringan
lunak yang berasal dari sel fibroblas. Tumor ini paling banyak mengenai usia 30 – 50 tahun dengan lokasi tersering adalah ekstremitas bawah. Gambaran mikroskopis fibrosarcoma secara khas ditemukan sel-sel spindel yang tumbuh hiperplastis dan membentuk susunan fasikulus yang memberikan gambaran khas herringbone appearance. Radiologis ditemukan gambaran yang khas yaitu sun burst appearance, periosteal reaction dan codman’s triangle. Secara mikroskopis pada tumor ini harus ditemukan adanya pembentukan osteoid ganas, yaitu dengan gambaran eosinofilik, glassy appearance, irregular countours, dan dikelilingi oleh osteoblast. Gambaran lain adalah seperti gambaran keganasan pada umumnya yaitu sel dengan inti yang pleomorfik, hiperkromatis dengan mitosis yang banyak dan adanya struktur microtrabeculae yaitu massa berwarna basofil dan menyerupai hifa jamur.