Bone and Cartilage tumors Benign and Malignant.ppt

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  • FSH (Landouzy-Dejerine)
  • Bone and Cartilage tumors Benign and Malignant.ppt

    1. 1. Bone and Cartilage tumors Benign and Malignant Department of Pathology
    2. 2. Benign tumors of bone. <ul><li>O STEOMA : i nvolves the skull and facial bones, w/extremely slow growth rate </li></ul><ul><li>( H YPEROSTOSIS FRONTALIS)it may e xtends into the orbit or sinuses(Gardner´s </li></ul><ul><li>syndrome )Peak incidence: 40-50 years of age </li></ul><ul><li>O STEOID OSTEOMA : b enign, painful growth of the diaphysis of a long bone ( often the tibia or femur ) </li></ul><ul><li>- Age : 5-25 years, mostly males </li></ul>
    3. 5. Benign tumors of bone. <ul><li>-Symptoms: P ain is worse at night and is relieved with aspirin </li></ul><ul><li>- X rays: c entral radiolucency surrounded by a sclerotic rim. </li></ul><ul><li>- Micro: s mall ( < 2 cms ) lesion of the cortex with c entral nidus of osteoid surrounded by dense sclerotic rim of reactive cortical bone. </li></ul>
    4. 6. Benign tumors. <ul><li>O STEOBLASTOMA : Similar to an osteoid osteoma but larger than 2 cms in size and often involv ing vertebrae. </li></ul>
    5. 7. Benign tumors. <ul><li>O STEOCHONDROMA (exostosis) </li></ul><ul><li>- Benign bon e metaphyseal grow t hs capped with cartilage that originates from epiphyseal growth plate. </li></ul><ul><li>-It may affects a dolescent males as a firm, solitary growth a t the ends of long bones. </li></ul><ul><li>-It may be asymptomatic or cause pain, produc ing deformity, and can undergo with malignant transformation ( rare ly ) </li></ul>
    6. 8. Osteochondroma
    7. 9. Benign tumors, Cartilage (cont.) <ul><li>O STEOCHONDROMATOSIS ( Multiple hereditary exostosis) -Characterized with multiple , often symmetric, osteochondromas. </li></ul><ul><li>E NCHONDROMA : b enign cartilaginous growth within the medullary cavity of bone, usually involving the hands and feet. </li></ul><ul><li>-Is a t ypical solitary lesion often asymptomatic and require no treatment. </li></ul>
    8. 10. Enchondroma
    9. 11. Benign tumors. Cartilage <ul><li>M ULTIPLE ENCHONDROMAS (Enchondromatosis) </li></ul><ul><li>O LLIER DISEASE : a n on hereditary s yndrome, with multiple enchondromas in hands and feet. </li></ul><ul><li>It may p resents with pain and spontaneous Fxs </li></ul><ul><li>It m ay undergo malignant transformation to chondrosarcoma. </li></ul>
    10. 12. Benign tumors. Cartilage (cont.) <ul><li>M AFUCCI SYNDROME </li></ul><ul><li>Multiple enchondromas </li></ul><ul><li>Soft tissue hemangiomas </li></ul><ul><li>Increased risk of malignant transformation, ovarian Ca . and brain gliomas. </li></ul>
    11. 13. Maffucc i Syndrome
    12. 14. Maffucci Syndrome
    13. 15. Malignant Tumors of Bone. <ul><li>O STEOSARCOMA ( Osteogenic sarcoma) </li></ul><ul><li>- Most common primary malignant tumor of bone </li></ul><ul><li>- Males> females. Most occur in teenagers ( ages 10-25) </li></ul><ul><li>- Patients with familial retinoblastoma have a high risk </li></ul><ul><li>- Clinical features: l ocalized pain and swelling </li></ul>
    14. 16. Malignant tumors of bone OSTEOSARCOMA(cont.) <ul><li>Classic X ray findings: - Codman’s triangle ( periosteal elevation) </li></ul><ul><li>- Sunburst pattern </li></ul><ul><li>- Bone destruction </li></ul><ul><li>- Grossly: o ften involves the metaphyses of long bones , usually around the knee (distal femur/pro </li></ul><ul><li>ximal tibia.) and it may be seen as a large, firm, </li></ul><ul><li>white mass with necrosis and hemorrhage. </li></ul>
    15. 17. Malignant Tumors of Bone .Osteosarcoma <ul><li>Micro: Anaplastic cells producing osteoid and bone. </li></ul><ul><li>T x : s urgery / chemotherapy </li></ul><ul><li>Prognosis: p oor (h ematogenous metastastasis to the lungs is a common complication) </li></ul><ul><li>“ SECONDARY” OSTEOSARCOMAS . Occur in elderly persons, associated with Paget’s disease, irradiation and chronic osteomyelitis </li></ul><ul><li>Highly agressive. </li></ul>
    16. 18. Osteosarcoma
    17. 19. Osteosarcoma
    18. 27. C HONDROSARCOMA <ul><li>Malignant tumor of cartilage </li></ul><ul><li>- Males> females age 30-60 </li></ul><ul><li>-T umor may arise primarily or secondary to a preexisting enchondroma,exostosis or Paget’s disease. </li></ul><ul><li>- Clinical presentation: progressively e nlarging mass with pain and swelling, that typically involves the pelvic bones, spine, and shoulder girdle. </li></ul><ul><li>- Micro: composed of atypical chondrocytes and chondroblasts, often with multiple nuclei in a lacuna r structure </li></ul>
    19. 28. Chondrosarcoma
    20. 31. G IANT CELL BONE TUMOR (Osteoclastoma) <ul><li>Uncommon malignant neoplasm containing multinucleated giant cells admixed with stromal cells. Females>males, with ages between 20-50 years </li></ul><ul><li>Clinical features : b ulky mass with pain and Fx. </li></ul><ul><li>X rays : e xpanding lytic lession surrounded by a thin rim of bone. </li></ul><ul><li>It m ay have also a soap bubble appearance </li></ul><ul><li>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. </li></ul>
    21. 36. G IANT CELL TUMOR(cont.) <ul><li>Micro: m ultiple osteoclast-like giant cells that are distributed within a background of mononuclear stromal cells. </li></ul><ul><li>T x : s urgery / curetage or bloc k resection </li></ul><ul><li>Prognosis: locally aggressive with a high rate of recurrence. </li></ul>
    22. 37. Giant cell tumor
    23. 38. E WING SARCOMA <ul><li>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) </li></ul><ul><li>Clinical features : pain , swelling and tenderness </li></ul><ul><li>Classic translocation t11;22 which produces the EWS- FL11 fusion protein </li></ul><ul><li>X - ray : concentric onion skin layering of new pe r iosteal bone. </li></ul>
    24. 39. Ewing sarcoma
    25. 43. E WING SARCOMA(cont.) <ul><li>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. </li></ul><ul><li>Micro: s heets of undifferentiated small round blue cells resembling lymphocytes. </li></ul><ul><li>Characteristic Homer - Wright pseudorosettes </li></ul><ul><li>Frequently the t umor al cells erode cortex and periosteum and invade surrounding tissues. </li></ul><ul><li>Tx.: c hemotherapy, surgery and / or radiation </li></ul><ul><li>Prognosis: 5 year survival rate of 75% </li></ul>
    26. 44. NEUROMUSCULAR DISORD. <ul><li>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”). </li></ul><ul><li>Diseases can be classified as involving: A. Motor neuron B. Peripheral nerves C.Neuro muscular junction D. Muscles </li></ul>
    27. 45. NEUROMUSCULAR DIS.(cont) <ul><li>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. </li></ul>
    28. 46. NEUROMUSCULAR DISORD. <ul><li>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 </li></ul>
    29. 47. NEUROMUSCULAR DISORD. <ul><li>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 </li></ul>
    30. 53. NEUROMUSCULAR DISORD. <ul><li>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). </li></ul>
    31. 56. NEUROMUSCULAR DISORD. <ul><li>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. </li></ul>
    32. 60. S TRIATED MUSCLE DISORDERS ( Dystrophies, Myositis and Tumors ) <ul><li>INFLAMMATORY DISORDERS. </li></ul><ul><li>P OLYMYOSITIS : </li></ul><ul><li>It may affect a dults, females with bilateral progressive, proximal muscle weakness </li></ul><ul><li>Micro: e ndomysial lymphocytic inflammation ( mostly cytotoxic T8 ) </li></ul><ul><li>Skeletal muscle fiber degeneration and regeneration. </li></ul>
    33. 61. D ERMATOMYOSITIS. <ul><li>It may affect c hildren or adults, females, with bilateral and proximal muscle weakness </li></ul><ul><li>Also: s kin rash of the upper eyelids , periorbital edema </li></ul><ul><li>Micro: p erimysial and vascular lymphocytic inflammation </li></ul><ul><li>with p erifascicular fiber atrophy </li></ul><ul><li>Skeletal muscle fiber degeneration and regeneration. </li></ul><ul><li>Increased risk of lung, stomach, ovarian and breast cancers. </li></ul>
    34. 62. Dermatomyositis
    35. 63. M USCULAR DYSTROPHIES <ul><li>DUCHENNE MUSCULAR DYSTROPHY </li></ul><ul><li>MOST COMMON and severe form of muscular dystrophy. </li></ul><ul><li>X linked inheritance </li></ul><ul><li>Dystrophin gene in Xp 21 </li></ul><ul><li>( Mutation results in a virtual absence of dystrophin protein ) </li></ul><ul><li>Normal at birth with onset of symptoms by age 5 , with progressive muscular weakness of proximal , shoulder and pelvic girdles. </li></ul><ul><li>C ALF PSEUDOHYPERTROPHY </li></ul><ul><li>Heart failure and arrhytmias may occur </li></ul><ul><li>Progressive r espiratory failure and pulmonary infections </li></ul><ul><li>Increased serum creatine kinase </li></ul><ul><li>Muscle fibers of various sizes , necrosis, degeneration and regeneration fibers </li></ul><ul><li>Fibrosis </li></ul><ul><li>Fatty infiltration. </li></ul>
    36. 65. Duchenne muscular dystrophy <ul><li>D x : m uscle biopsy with immunostains show s decreased dystrophin protein </li></ul><ul><li>DNA analysis by PCR. </li></ul>
    37. 66. Duchenne muscular dystrophy
    38. 68. B ECKER MUSCULAR DISTROPHY <ul><li>It is a less common condition </li></ul><ul><li>The observed m utation produces an altered dystrophin protein </li></ul><ul><li>Later onset with variable progression </li></ul><ul><li>Cardiac involv e ment is rare ly seen </li></ul><ul><li>Patients have a r elatively normal life span </li></ul>
    39. 70. MUSCULARY DISTROPHY(cont.) <ul><li>- Inclusion body myositis. </li></ul><ul><li>- Myasthenic Syndromes </li></ul><ul><li>- Inflammatory Neuropathies. </li></ul>
    40. 75. S OFT TISSUE TUMORS . <ul><li>Adipose tissue.: Lipomas –Liposarcomas </li></ul><ul><li>Fibrosarcoma </li></ul><ul><li>Rhabdomyoma , rhabdomyosarcoma </li></ul><ul><li>Smooth muscle </li></ul><ul><li>Vascular tumors </li></ul><ul><li>Peripheral nerve tumors. </li></ul>
    41. 90. SOFT TISSUE TUMORS <ul><li>RHABDOMYOSARCOMA (cont.) </li></ul><ul><li>Dx: -Excisional biopsy -Immunochemistry +vimentin +desmin +actin +myoglobin </li></ul>
    42. 91. SOFT TISSUE TUMORS <ul><li>SMOOTH MUSCLE TUMORS. 1. Leiomyoma 2. Leiomyosarcoma </li></ul><ul><li>VASCULAR TUMORS 1. Hemangiomas 2. Angiosarcomas </li></ul><ul><li>SYNOVIALSARCOMA </li></ul>

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