Bone and cartilage tumors benign and malignant
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Bone and cartilage tumors benign and malignant Presentation Transcript

  • 1. Bone and Cartilage tumors Benign and Malignant www.freelivedoctor.com
  • 2. Benign tumors of bone.
    • O STEOMA : i nvolves the skull and facial bones, w/extremely slow growth rate
    • ( H YPEROSTOSIS FRONTALIS)it may e xtends into the orbit or sinuses(Gardner´s
    • syndrome )Peak incidence: 40-50 years of age
    • O STEOID OSTEOMA : b enign, painful growth of the diaphysis of a long bone ( often the tibia or femur )
    • - Age : 5-25 years, mostly males
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  • 5. Benign tumors of bone.
    • -Symptoms: P ain is worse at night and is relieved with aspirin
    • - X rays: c entral radiolucency surrounded by a sclerotic rim.
    • - Micro: s mall ( < 2 cms ) lesion of the cortex with c entral nidus of osteoid surrounded by dense sclerotic rim of reactive cortical bone.
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  • 6. Benign tumors.
    • O STEOBLASTOMA : Similar to an osteoid osteoma but larger than 2 cms in size and often involv ing vertebrae.
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  • 7. Benign tumors.
    • O STEOCHONDROMA (exostosis)
    • - Benign bon e metaphyseal grow t hs capped with cartilage that originates from epiphyseal growth plate.
    • -It may affects a dolescent males as a firm, solitary growth a t the ends of long bones.
    • -It may be asymptomatic or cause pain, produc ing deformity, and can undergo with malignant transformation ( rare ly )
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  • 8. Osteochondroma www.freelivedoctor.com
  • 9. Benign tumors, Cartilage (cont.)
    • O STEOCHONDROMATOSIS ( Multiple hereditary exostosis) -Characterized with multiple , often symmetric, osteochondromas.
    • E NCHONDROMA : b enign cartilaginous growth within the medullary cavity of bone, usually involving the hands and feet.
    • -Is a t ypical solitary lesion often asymptomatic and require no treatment.
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  • 10. Enchondroma www.freelivedoctor.com
  • 11. Benign tumors. Cartilage
    • M ULTIPLE ENCHONDROMAS (Enchondromatosis)
    • O LLIER DISEASE : a n on hereditary s yndrome, with multiple enchondromas in hands and feet.
    • It may p resents with pain and spontaneous Fxs
    • It m ay undergo malignant transformation to chondrosarcoma.
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  • 12. Benign tumors. Cartilage (cont.)
    • M AFUCCI SYNDROME
    • Multiple enchondromas
    • Soft tissue hemangiomas
    • Increased risk of malignant transformation, ovarian Ca . and brain gliomas.
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  • 13. Maffucc i Syndrome www.freelivedoctor.com
  • 14. Maffucci Syndrome www.freelivedoctor.com
  • 15. Malignant Tumors of Bone.
    • O STEOSARCOMA ( Osteogenic sarcoma)
    • - Most common primary malignant tumor of bone
    • - Males> females. Most occur in teenagers ( ages 10-25)
    • - Patients with familial retinoblastoma have a high risk
    • - Clinical features: l ocalized pain and swelling
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  • 16. Malignant tumors of bone OSTEOSARCOMA(cont.)
    • Classic X ray findings: - Codman’s triangle ( periosteal elevation)
    • - Sunburst pattern
    • - Bone destruction
    • - Grossly: o ften involves the metaphyses of long bones , usually around the knee (distal femur/pro
    • ximal tibia.) and it may be seen as a large, firm,
    • white mass with necrosis and hemorrhage.
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  • 17. Malignant Tumors of Bone .Osteosarcoma
    • Micro: Anaplastic cells producing osteoid and bone.
    • T x : s urgery / chemotherapy
    • Prognosis: p oor (h ematogenous metastastasis to the lungs is a common complication)
    • “ SECONDARY” OSTEOSARCOMAS . Occur in elderly persons, associated with Paget’s disease, irradiation and chronic osteomyelitis
    • Highly agressive.
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  • 18. Osteosarcoma www.freelivedoctor.com
  • 19. Osteosarcoma www.freelivedoctor.com
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  • 27. C HONDROSARCOMA
    • Malignant tumor of cartilage
    • - Males> females age 30-60
    • -T umor may arise primarily or secondary to a preexisting enchondroma,exostosis or Paget’s disease.
    • - Clinical presentation: progressively e nlarging mass with pain and swelling, that typically involves the pelvic bones, spine, and shoulder girdle.
    • - Micro: composed of atypical chondrocytes and chondroblasts, often with multiple nuclei in a lacuna r structure
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  • 28. Chondrosarcoma www.freelivedoctor.com
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  • 31. G IANT CELL BONE TUMOR (Osteoclastoma)
    • Uncommon malignant neoplasm containing multinucleated giant cells admixed with stromal cells. Females>males, with ages between 20-50 years
    • Clinical features : b ulky mass with pain and Fx.
    • X rays : e xpanding lytic lession surrounded by a thin rim of bone.
    • It m ay have also a soap bubble appearance
    • Gross: o ften involves the epiphyses of long bones, usually around the knee ( distal femur and proximal tibia) seen a r ed brown mass with cystic degeneration.
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  • 36. G IANT CELL TUMOR(cont.)
    • Micro: m ultiple osteoclast-like giant cells that are distributed within a background of mononuclear stromal cells.
    • T x : s urgery / curetage or bloc k resection
    • Prognosis: locally aggressive with a high rate of recurrence.
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  • 37. Giant cell tumor www.freelivedoctor.com
  • 38. E WING SARCOMA
    • Malignant neoplasm of undifferentiated cells arising within the marrow cavity M ales are affected slightly more often than females, most occur in teenagers ( 5-20)
    • Clinical features : pain , swelling and tenderness
    • Classic translocation t11;22 which produces the EWS- FL11 fusion protein
    • X - ray : concentric onion skin layering of new pe r iosteal bone.
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  • 39. Ewing sarcoma www.freelivedoctor.com
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  • 43. E WING SARCOMA(cont.)
    • Gross: o ften affects the diaphyses of long bones with most common sites like femur, pelvis and tibia seen a w hite tan mass with necrosis and hemorrhage.
    • Micro: s heets of undifferentiated small round blue cells resembling lymphocytes.
    • Characteristic Homer - Wright pseudorosettes
    • Frequently the t umor al cells erode cortex and periosteum and invade surrounding tissues.
    • Tx.: c hemotherapy, surgery and / or radiation
    • Prognosis: 5 year survival rate of 75%
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  • 44. NEUROMUSCULAR DISORD.
    • The “motor unit”consists of a lower motor neuron(anterior horn cell or neuron in cranial nerve nuclei), its axon and the muscle fibers innervated by it. The number of muscle fibers innervated varies from a few fibers(oculo-motor muscles) to several hundreds(extremity muscles). Muscles fibers of one motor unit are scattered in a wide area in a random fashion(“checkerdboard”).
    • Diseases can be classified as involving: A. Motor neuron B. Peripheral nerves C.Neuro muscular junction D. Muscles
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  • 45. NEUROMUSCULAR DIS.(cont)
    • A. DISEASES OF MOTOR NEURON. -Etiology of most of the motor neuron dis.(AML, progressive muscular atrophy) is not known, but may be caused by several agents: adriamycin, vincristine, aluminium  perikaryon(neuronal cell body) primarily affected w/loss of microtubules and nuclear displacement + increased cytoplasmic neurofilaments or tangled masses of neurofila- ments. Viral infections(Polyomyelitis, Herpes encephalitis, Varicella-zoster) may also affect the motor neuron or sensory ganglia.
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  • 46. NEUROMUSCULAR DISORD.
    • B. DISEASES OF PERIPHERAL NERVES I. Axonal degeneration(axonal neuropathy) a. Wallerian degeneration(crush/cut along a myelinated fiber) b. Axonal degeneration caused by other diseases (less axoplasm leakage+ more inflammation + chronic evolution): 1.Proximal axonal degeneration: intoxic. w/ IDPN(BB-Iminodipropionitrite)  shrinkage of distal axons due to focal proximal blockage. 2.Distal axonal degeneration: earliest changes occur in the most distal portion of axons
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  • 47. NEUROMUSCULAR DISORD.
    • DISEASES OF PERIPHERAL...(cont.) Many diseases w/earlier changes in the most distal portion of axons  slow spread to proximal structures  perikaryon cannot support the terminal axon, vgr.: --Hereditary neuropathies. -HSMN I (Charcot-Marie-Tooth)disease, the MOST common of these, inherited as autosomal- dominant is usually present in childhood/early adulthood(PMP 22 gene/17p11.2-p12 locus), characterized by progressive atrophy of leg mus- cles,foot drop/deformed feet w/less sensory defect
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  • 53. NEUROMUSCULAR DISORD.
    • DISEASES OF PERIPHERAL...(cont.) --Hereditary neuropathies... -HSMN II(CMT2A) also AD, with similar manifestations than CMT I but without nerve enlargement and presentation at a la- ter age. Linked to chromosome 1p35-p36. -HSMN III(Dejerine-Sottas disease) is an AR condition that begins slowly in early childhood w/delayed acquisition of motor skills and involvement of muscles of limbs and trunk  enlar gement of nerves easy to detect. Genetic heteroge nicity(PMP 22, MPZ, PRX and EGR2).
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  • 56. NEUROMUSCULAR DISORD.
    • DISEASES OF PERIPHERAL...(cont.) --Acquired metabolic/toxic neuropathies. -Diabetic peripheral neuropathy: w/symme tric neuropathy involving distal sensory/mo tor nerves + dysfunction of autonomic nervous system(20-40% of cases).It can also present as a single peripheral or cranial neuroneuropathy(oculomotor nerve). -Metabolic/nutritional: chronic liver disease,resp. insuff., renal failure, thiamine def.,Vit.B12,B6,E. Chronic alcoholism, etc.
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  • 60. S TRIATED MUSCLE DISORDERS ( Dystrophies, Myositis and Tumors )
    • INFLAMMATORY DISORDERS.
    • P OLYMYOSITIS :
    • It may affect a dults, females with bilateral progressive, proximal muscle weakness
    • Micro: e ndomysial lymphocytic inflammation ( mostly cytotoxic T8 )
    • Skeletal muscle fiber degeneration and regeneration.
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  • 61. D ERMATOMYOSITIS.
    • It may affect c hildren or adults, females, with bilateral and proximal muscle weakness
    • Also: s kin rash of the upper eyelids , periorbital edema
    • Micro: p erimysial and vascular lymphocytic inflammation
    • with p erifascicular fiber atrophy
    • Skeletal muscle fiber degeneration and regeneration.
    • Increased risk of lung, stomach, ovarian and breast cancers.
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  • 62. Dermatomyositis www.freelivedoctor.com
  • 63. M USCULAR DYSTROPHIES
    • DUCHENNE MUSCULAR DYSTROPHY
    • MOST COMMON and severe form of muscular dystrophy.
    • X linked inheritance
    • Dystrophin gene in Xp 21
    • ( Mutation results in a virtual absence of dystrophin protein )
    • Normal at birth with onset of symptoms by age 5 , with progressive muscular weakness of proximal , shoulder and pelvic girdles.
    • C ALF PSEUDOHYPERTROPHY
    • Heart failure and arrhytmias may occur
    • Progressive r espiratory failure and pulmonary infections
    • Increased serum creatine kinase
    • Muscle fibers of various sizes , necrosis, degeneration and regeneration fibers
    • Fibrosis
    • Fatty infiltration.
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  • 65. Duchenne muscular dystrophy
    • D x : m uscle biopsy with immunostains show s decreased dystrophin protein
    • DNA analysis by PCR.
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  • 66. Duchenne muscular dystrophy www.freelivedoctor.com
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  • 68. B ECKER MUSCULAR DISTROPHY
    • It is a less common condition
    • The observed m utation produces an altered dystrophin protein
    • Later onset with variable progression
    • Cardiac involv e ment is rare ly seen
    • Patients have a r elatively normal life span
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  • 70. MUSCULARY DISTROPHY(cont.)
    • - Inclusion body myositis.
    • - Myasthenic Syndromes
    • - Inflammatory Neuropathies.
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  • 75. S OFT TISSUE TUMORS .
    • Adipose tissue.: Lipomas –Liposarcomas
    • Fibrosarcoma
    • Rhabdomyoma , rhabdomyosarcoma
    • Smooth muscle
    • Vascular tumors
    • Peripheral nerve tumors.
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  • 90. SOFT TISSUE TUMORS
    • RHABDOMYOSARCOMA (cont.)
    • Dx: -Excisional biopsy -Immunochemistry +vimentin +desmin +actin +myoglobin
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  • 91. SOFT TISSUE TUMORS
    • SMOOTH MUSCLE TUMORS. 1. Leiomyoma 2. Leiomyosarcoma
    • VASCULAR TUMORS 1. Hemangiomas 2. Angiosarcomas
    • SYNOVIALSARCOMA
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