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Usually solitary - usually found in middle-age adults - Associated with Gardner syndrome (if multiple) Pathogenesis - Slow growing - frequently arise / found inside skull or facial bones Lab / Gross - composed of woven / lamellar bone - Bosselated, round to oval sessile tumors - Deposited in a cortical pattern w / haversian-like systems.
Usually solitary - usually found in middle-age adults - Associated with Gardner syndrome (if multiple) Pathogenesis - Slow growing - frequently arise / found inside skull or facial bones Lab / Gross - composed of woven / lamellar bone - Bosselated, round to oval sessile tumors - Deposited in a cortical pattern w / haversian-like systems.
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Usually solitary - usually found in middle-age adults - Associated with Gardner syndrome (if multiple) Pathogenesis - Slow growing - frequently arise / found inside skull or facial bones Lab / Gross - composed of woven / lamellar bone - Bosselated, round to oval sessile tumors - Deposited in a cortical pattern w / haversian-like systems.
Hak Cipta:
Attribution Non-Commercial (BY-NC)
Format Tersedia
Unduh sebagai DOC, PDF, TXT atau baca online dari Scribd
Cancer Epidemiology Pathogenesis Lab/Gross Clinical Features Prognosis/Tx
- Usually solitary - Composed of - Of little
- Usually found - Slow growing woven/lamellar bone significance unless Osteoma in middle-age - Frequently arise/found - Bosselated, round to they cause - Do NOT transform adults inside skull or facial bones oval sessile tumors obstruction/imping to osteosarcoma - Associated with - Deposited in a e on structures Gardner cortical pattern w/ (brain/eye), syndrome (if haversian-like interfere w/ fx of multiple) systems oral cavity or cause cosmetic problems - By definition less - Predilection for than 2 cm in greatest - Painful due to - Readily treated Osteoid - Patients usually appendicular skeleton dimension excess PGE2 by conservative Osteoma in teens/twenties (femur/tibia most common) - Round to oval release from surgery - Men > women - Commonly arise in bone masses of osteoblasts - Malignant (2:1) cortex hemorrhagic gritty (nocturnal pain) transformation is tan tissue - Dramatically remote (except - Well circumscribed relieved by ASA when treated with and composed of a radiation; which morass of randomly promotes interconnecting malignant trans.) trabeculae of woven bone; rimmed by osteoblasts - Greater than or equal to 2 cm in size - Round to oval - Readily treated - More frequently involves masses of - Dull, achy pain by conservative Osteoblasto the spine hemorrhagic gritty - Not responsive to surgery ma - Commonly arise in bone tan tissue salicylates - Malignant cortex - Well circumscribed transformation is and composed of a remote (except morass of randomly when treated with interconnecting radiation; which trabeculae of woven promotes bone; rimmed by malignant trans.) osteoblasts - Does not induce a marked bony reaction - Distal femur, proximal - Standard: tibia; metaphysic plates - Hemorrhagic & - Painful, chemotherapy and - Most common in head, cystic progressively limb salvage - 75% in ages < shoulder, hip and knee* - Infiltrate marrow enlarging masses - @ time of dx: 10- Osteosarcom 20 - Genetic mutations are - Bulky tumors; gritty, - Sudden fractures 20% have a - More common fundamental gray-white - Codman’s pulmonary mets in men than - RB, p53, INK4A, CDK4, p16, - Formation of bone is triangle: triangular - 90% who die women CYCLIN D1, MDM2 characteristic; shadow between have metastases to - Cell cycle regulator genes cartilage/fibrous the cortex & raised the lung, bone, - Subtypes grouped tissue may be present ends of periosteum brain and according to anatomic - Pleomorphic cells w/ elsewhere portion, degree of diff, large hyperchromatic - Long term multicentricity, nuclei; bizarre giant survival 60-70% primary/secondary cells - Cartilage capped - Slow growing - Aka: exostosis - Develops in bones of outgrowth attached - Can be painful if - Multiple: endochondral origin; arise to underlying impinge on nerve - Rarely give rise to Multiple Heredity from metaphysic near the skeleton by bony - Frequently chondrosarcoma or Osteochondr Exostosis (AD; growth plate of long tubular stalk (protruding detected other sarcoma oma inactivation of bones (especially knee) mushroom) incidentally - Risk of malignant both EXT genes); - Occasionally found in - Range from 1-20 cm - In hereditary transformation is childhood pelvis, scapula and ribs - Composed of form: underlying higher in - Solitary: late - Rarely involve short tubular hyaline cartilage bones may be hereditary adolescence, bones of hands/feet covered peripherally bowed or syndrome early adulthood by perichondrium shortened - Men>women - Appearance of - Usually stop (3:1) disorganized growth growing @ time of plate growth plate closure - Benign tumor of hyaline cartilage - Usually < 3cm - Enchondroma (in medullary - Blue, gray-blue - Solitary cavity) – most common - or translucent color with chondromas rarely subperiosteal/juxtacortical a nodular - Most are undergo malignant - (on bone surface) configuration asymptomatic transformation Chondroma Endochondroma - Endochondromas are - Nodules of cartilage - Detected - are usually found primarily found in short and are well- incidentally Enchondromatoses in pts age 20-50 tubular bones (hand/feet) circumscribed w/ - Occasionally are are more & are solitary - Ollier disease = multiple hyaline matrix painful & cause associated with enchondromas - The cartilage @ the pathologic malignant Maffucci syndrome = periphery undergoes fractures transformation enchondromatosis + soft enchondral - Maffucci tissue hemangiomas ossification and the syndrome patients - Develop from the remains center frequently are at risk for other of growth plate cartilage in calcifies & dies malignancies bones that undergo - On x-ray: O ring sign (ovarian CA, brain enchondral ossification is present glioma) - Highly cellular, hyperlobulated nuclei w/ longitudinal - Benign tumor grooves - Rare - Most commonly found in - Sheets of compact - Usually painful - Distant Chondroblas - Young patients knee – less commonly found polyhedral (due to location metastases to the toma in teens in the pelvis/ribs and in older chondroblasts w/ well- near joint) lung are rare - Male: female = pts defined borders - Recurrences not (usually after 2:1 - Predilection for epiphyses - Mitotic activity & uncommon fracture/curettage) and apophyses necrosis frequently found - Chicken-wire pattern - Non-neoplastic osteoclast giant cells are scattered throughout - X-ray: well-defined lucency w/ spotty calc. - Well-circumscribed, solid, glistening tan- gray tumor - Nodules of hyaline cartilage and myxoid - Teens and tissue w/ fibrous - Simple curettage Chondromyx twenties - Most frequently in septae - Localized, dull - Do not pose a oid - Male metaphysic of long tubular - Greatest cellularity achy pain threat for Fibroma predominance bones @ periphery malignant - Rarest cartilage - In cartilaginous transformation tumor regions: tumor cells are in lacunae; in myxoid areas: cells are stellate - Varying degrees of cytologic atypia w/ hyperchromatic nuclei - X-ray: eccentric geographic lucency that is well delineated from bone by rim of sclerosis - Composed of - Low grade: malignant hyaline & causes reactive myxoid cartilage thickening of the - Myxoid: viscous, cortex - Patients are - Broad spectrum of findings ooze when cut; spotty - High grade: usually 40+ - Subclassifications: calcifications & destroys the cortex - Men are 2x Site: intramedullary or central necrosis; and forms soft more likely to be juxtacortical adjacent cortex is tissue mass affected than Histo: conventional, clear thickened/eroded - Painful, - Correlation Chondrosarc women cell, dedifferentiated or - Dediff: Low-grade progressively between grade & oma - No race mesenchymal w/ second high-grade enlarging masses biological behavior predilection - Significant number arise in component - None of grade 1 - Clear cell & association w/ pre-existing - Clear cell: sheets of metastasized mesenchymal enchondroma large, malignant cells - Size is another varients in - Arise in central portions of w/ abundant clear prognostic feature younger patients skeleton (pelvis, shoulder, cytoplasm, (> 10 cm behave (teens/twenties) ribs) osteoclast-type GC more aggressively) - Clear cell variant originates and intralesional - Metastasize in epiphyses of long tubular reaction bone preferentially to bones formation the lungs/skeleton - Rarely involves distal - Mesenchymal: - Tx = wide extremities Islands of well-diff surgical excision hyaline cartilage - Mesenchymal & surrounded by small dediff additionally round cells treated w/ chemo - X-ray: Prominent endosteal scalloping; more radiolucent = high grade - FCD: - FCD: Developmental asymptomatic; - FCD: for those Fibrous - FCD: Found in defect; found in distal incidentally found; that enlarge; Cortical 30%-50% of femur/prox tibia limited growth require biopsy to Defects & children >2 - Those that grow frequently potential and most r/o other type of Nonossifying develop into NF; one half are undergo tumor Fibroma bilateral/multiple spontaneous resolution; few progressively enlarge & can cause path. fracture - Benign tumor; likened to - Well-circumscribed, - Monoostotic: localized developmental intramedullary boys=girls, most arrest lesions; vary greatly common in early - Components of normal in size - Rare to have adolescence bone are present, but do not - Moderately cellular sarcomatous Fibrous - Polyostotic: differentiate - Trabeculae mimic transformation Dysplasia manifests - Monoostotic: most Chinese characters (polyostotic slightly earlier common; ribs, femur, tibia, - Nodules of hyaline involvement than jawbones, calvaria and cartilage w/ required) monoostotic humerus; don’t evolve disorganized growth - Use of radiation - Polyostotic - Polyostotic: femur, skull, plate appearance increases risk of disease: somatic tibia; craniofacial - Cystic degeneration, malignant mutations in G- involvement present in half; hemorrhage and transformation protein leads to all forms have propensity to foamy macs are excess cAMP; involve shoulder/pelvic common findings girls more girdle - Xray: ground glass frequently - Polyostotic Disease: appearance w/ well- affected than multiple bones + defined margins boys endocrinopathies; McCune- Albright syndrome; sexual precocity, hyperthyroidism, adrenal hyperplasia; bone & café-au-lait spots frequently unilateral - Large, hemorrhagic, tan-white masses - Destroy underlying - Middle aged & - Fibroblastic collagen- bone - Enlarging painful Fibrosarcom elderly producing sarcomas of bone - Frequently extend masses - Prognosis a& - Fibrosarcoma: - Usually arise de novo; into soft tissue - Pathologic depends on grade; Malignant male=female some secondary tumors - Fibrosarcoma: fracture is a high-grade have Fibrous - MFH: men more - Usually in metaphyses of malignant fibroblasts frequent poor prognosis Histiocytoma frequently long bones & pelvic flat arranged in a complication affected bones herringbone pattern; most low- intermediate grade - MFH: Background of spindle fibroblasts arranged in storiform pattern; large, ovoid, bizarre multinuc tumor GC intermixed; generally high-grade - Xray: permeative/lytic - Average= 10- - Arise in medullary 15 years old cavity - Boys more freq. - Usually invade than girls cortex & periosteum - Present as - More common producing soft tissue painful, enlarging - Treatment in whites - Primary malignant small mass masses includes Ewing - t(11;22) most round cell tumors - Composed of sheets - Affected site is chemotherapy and Sarcoma common; also - Usually arises in diaphyses of uniform, small tender, warm and surgical excision w/ & t(21;21) or of long tubular bones round cells that are swollen or w/o radiation PNET t(7;22) (femur, flat bones of pelvis) slightly larger than - Some patients - At least 50% of - Fusion of EWS lymphocytes have systemic cases are long gene to a - Tan-white tumor findings: fever, term cures; 75% 5 member of ETS that contains areas of elevated SED rate, year survival family hemorrhage & anemia, transcription necrosis leukocytoses factor (mainly - Scant cytoplasm, FLT1) rich in glycogen - EWS-FLI1 forms - Homer-Wright dominant rosettes is indicative oncogene; of neural diff constitutively - Contains fibrous active septae but generally transcription little stroma factor - Xray: destructive stimulating cell lytic tumor w/ proliferation permeative margins and extension into soft tissue - Large and red-brown - Believed to have monocyte - Uniform oval macrophage lineage mononuclear cells - Conservative - Uncommon benign tumor; that have indistinct - Location leads to surgery (40-60% Giant Cell - Patients but locally aggressive cell membrane and complaints of recurrence rate) Tumor twenties to - Involve both epiphyses and appear to grow in a arthritis-type - Up to 4% forties metaphyses in adults; syncytium symptoms metastasize to the confined to growth plate in - Mitoses are frequent - Occasionally lung adolescents - Necrosis, present as - Sarcomatous - Most commonly arise in hemorrhage, pathologic fracture transformation is knee hemosideran rare - Most are solitary deposition and reactive bone formation are common secondary features