Pathology (bone tumors)

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amirh899
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234782
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Pathology (bone tumors)
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2013-09-15 13:44:09
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bone tumors
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bone tumors
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  1. What is the mc bone tumor?
    metastasis from breast cancer
  2. What are the mc primary bone tumors?
    Tumors of hematopoietic origin (mc-->multiple myeloma)
  3. What is the mc non-hematopoietic malignant bone tumor?
    Osteosarcoma
  4. What are the mc benign bone tumor?
    osteochondroma and fibrous cortical defect
  5. Which bone tumor is deposited as lamellar bone?
    Osteoma
  6. What are the features of osteoma?
    • Slow growing sessile project from subperiosteal surface of cortex
    • Associated with Gardner syndrome
    • Involves facial bone
    • Consists of both woven and lamellar  bone
  7. What are the features of osteoid osteoma and osteoblastoma?
    • Osteoid osteoma--> <2 cm, cortex of femur or tibia, severe nocturnal pain (PGE2), relieved by aspirin, severe bony reaction
    • Osteoblastoma --> >2 cm, vertebra, dull pain, not relieved by aspirin, mild bony reaction
  8. What is the histological feature of osteoid osteoma and osteoblastoma?
    • Well circumscribed and composed of randomly interconnecting trabeculae of woven bone that are prominently rimmed by osteoblasts
    • Osteoid osteomas, especially those that arise beneath the periosteum, usually elicit a tremendous amount of reactive bone formation that encircles the lesion.
    • The actual tumor, known as the nidus, manifests radiographically as a small round lucency that may be centrally mineralized
  9. What is the main characteristic of the cancerous cells in osteosarcoma?
    produce bone matrix
  10. What are the predisposing condition for osteosarcoma?
    Paget disease, bone infarcts, and prior irradiation, Li Fraumni syndrome, and retinoblastoma
  11. What is the mc type of osteosarcoma?
    • Metaphysis of long bones and is primary, solitary, intramedullary, and poorly differentiated.
    • 50% around the knee
  12. What are the histological features of osteosarcoma?
    • Cyst, hemorrhage, necrosis
    • Destroying cortex and marrow
    • Vascular invasion
    • Bizarre GC and mitoses
    • The formation of bone by the tumor cells is characteristic
    • The neoplastic bone usually has a coarse, lace-like architecture
    • Other matrices, including cartilage or fibrous tissue, may be present in varying amounts. When malignant cartilage is abundant, the tumor is called chondroblastic osteosarcoma.

  13. What is the mc symptom of osteosarcoma?
    painful, progressively enlarging masses
  14. What is the radiological presentation of osteosarcoma?
    • Large destructive, mixed lytic and blastic mass with infiltrative margins 
    • The tumor frequently breaks through the cortex and lifts the periosteum, resulting in reactive periosteal bone formation. The triangular shadow between the cortex and raised ends of periosteum is known radiographically as Codman triangle.
    • Sunburst pattern
  15. What is the mc benign bone tumor?
    Osteochondroma (exostosis)
  16. What is osteochondroma?
    benign cartilage-capped tumor that is attached to the underlying skeleton by a bony stalk
  17. What are the features of multiple hereditary exostosis syndrome?
    • Autosomal dominant
    • Germline loss-of-function mutations in EXT1 or EXT2 genes, whereas inactivation of only EXT1 has been detected in sporadic tumors.
    • Encode proteins that function in the biosynthesis of heparin sulfate
    • Defective endochondral ossification
  18. What is the origin of osteochondroma?
    Only in bones of endochondral origin and arise from the metaphysis near the growth plate of long tubular bones, especially about the knee
  19. What is the histology of osteochondroma?
    • The cap is composed of benign hyaline cartilage varying in thickness and is covered peripherally by perichondrium.
    • The cartilage has the appearance of disorganized growth plate and undergoes enchondral ossification, with the newly made bone forming the inner portion of the head and stalk.
    • The medullary cavity of the osteochondroma and bone are in continuity.

  20. What is the course of osteochondroma?
    • In multiple hereditary exostosis the underlying bones may be bowed and shortened, reflecting an associated disturbance in epiphyseal growth.
    • Osteochondromas usually stop growing at the time of growth plate closure. 
    • In multiple hereditary exostosis, they give rise to a chondrosarcoma or some other type of sarcoma.
  21. What is the mc intaosseous cartilage tumor?
    Enchondroma
  22. What is the origin of chondroma?
    hyaline cartilage that usually occur in bones of enchondral origin
  23. What is the mc place for endchondroma?
    Medulla of  Metaphysis of short tubular bones of the hands and feet
  24. What are Ollier and Maffucci syndrome?
    • multiple enchondromas --> Ollier disease.
    • Enchondromatosis is associated with soft-tissue hemangiomas-->  Maffucci syndrome.
  25. What are the histological features of chondroma?
    • Less 3 cm and grossly are gray-blue and translucent
    • Well-circumscribed nodules of benign hyaline cartilage 
    • Calcification may be present
  26. What is the radiological feature of enchondroma?
    • The unmineralized nodules of cartilage produce well-circumscribed oval lucencies that are surrounded by a thin rim of radiodense bone (C or O ring sign).
    • The nodules scallop the endosteum, but usually leave the cortex intact
  27. What are the symptoms and complications of enchondroma?
    • Pathological fracture
    • Deformity if multiple
    • Sarcoma if multiple
    • Maffucci syndrome also at risk of developing ovarian carcinomas and brain gliomas.
  28. What are the general features of chondroblastoma?
    • Rare benign
    • Teen age
    • Predilection for epiphyses and apophyses 
    • Arise around the knee >hip
  29. What are the morphological features of chondroblastoma?
    • 1) Sheets of compact polyhedral chondroblasts that have well-defined cytoplasmic borders, moderate amounts of pink cytoplasm, and nuclei that are hyperlobulated with longitudinal grooves
    • 2) Mitotic activity and necrosis are frequently present.
    • 3) The tumor cells are surrounded by scant amounts of hyaline matrix that is deposited in a lace-like configuration; nodules of well-formed hyaline cartilage are distinctly uncommon.
    • 4) When the matrix calcifies it produces a characteristic chicken-wire pattern of mineralization
    • 5) Non-neoplastic osteoclast-type giant cells 
  30. What are the clinical features of chondroblastoma?
    • Usually painful, effusions and restriction joint mobility.
    • Radiographically, they produce a well-defined geographic lucency that commonly has spotty calcifications. 
    • Recurrence common
    • Rarely pulmonary metastasis
  31. What are the hallmarks of chondromyxoid fibroma?
    • Very rare
    • Can be misdiagnosed as sarcoma
    • Only benign tumor that show atypia
    • Metaphysis of long tubular bones
    • Nodules of poorly formed hyaline cartilage and myxoid tissue delineated by fibrous septae.
    • In the cartilaginous regions the tumor cells are situated in lacunae; however, in the myxoid areas, the cells are stellate.
  32. What are the predisposing factor for chondrosarcoma?
    About 15% of conventional chondrosarcomas (usually peripheral tumors) arise from a preexisting enchondroma or osteochondroma.
  33. What is the difference between epidemiology of chondrosarcoma and osteosarcoma?
    • Osteosarcoma<20 y
    • Chondrosarcoma >40 y
  34. What are the histological features of chondrosarcoma?
    • Conventional--> malignant hyaline and myxoid cartilage, In predominantly myxoid variants, the tumors are viscous and gelatinous and the matrix oozes from the cut surface
    • Dedifferentiated chondrosarcomas: Conventional low-grade chondrosarcomas have a second high-grade component that has the morphology of a poorly differentiated sarcoma
    • Clear cell chondrosarcoma: abundant clear cytoplasm, numerous osteoclast-type giant cells, and intralesional reactive bone formation, which often causes confusion with osteosarcoma. Seen in the young
    • Mesenchymal chondrosarcoma: islands of well-differentiated hyaline cartilage surrounded by sheets of small round cells, which can mimic Ewing sarcoma.
  35. What is the mc place for chondrosarcoma?
    • Central portions of the skeleton, including the pelvis, shoulder, and ribs
    • The clear cell variant arise in epiphyses of long tubular bones
  36. What is the difference in location between  enchondroma and chondrosarcoma ?
    In contrast to enchondroma, chondrosarcoma rarely involves the distal extremities.
  37. What are the manifestations of chondrosarcoma?
    • Painful, progressively enlarging masses.
    • The nodular growth pattern of the cartilage produces prominent endosteal scalloping radiographically. The calcified matrix appears as foci of flocculent densities.
    • A slow-growing, low-grade tumor causes reactive thickening of the cortex, whereas a more aggressive high-grade neoplasm destroys the cortex and forms a soft-tissue mass
  38. What is the most important determinant of biological behaviors of chondrosarcoma?
    Grade
  39. What are fibrous cortical defect and nonossifying fibroma?
    • FCD are found in 30% to 50% of children older than 2 years.
    • Developmental defects
    • Arise eccentrically in the metaphysis of the distal femur and proximal tibia (like osteosarcoma)
    • Half are bilateral or multiple.
    • If > 5 cm --> NOF
  40. What are the radiological features of fibrous cortical defects and non-ossifying fibromas?
    • elongated, sharply demarcated radiolucencies that are surrounded by a thin rim of sclerosis
  41. What is the histological features of fibrous cortical defects and non-ossifying fibromas?
    • Fibroblasts and macrophages (histiocytes). 
    • Fibroblasts are frequently arranged in a storiform (pinwheel) pattern, and the histiocytes are either multinucleated giant cells or clusters of foamy macrophages
  42. What is the course of NOF and FCD?
    • FCD are asymptomatic and undergo spontaneous resolution within several years, being replaced by normal cortical bone.
    • NOF may present with pathologic fracture.
  43. What is the pathophysiology of fibrous dysplasia?
    localized developmental arrest; all of the components of normal bone are present, but they do not differentiate into their mature structures
  44. What are the types of fibrous dysplasia?
    • (1) involvement of a single bone (monostotic);
    • (2) involvement of multiple bones (polyostotic)
    • (3) polyostotic disease, associated with café-au-lait skin pigmentations and endocrine abnormalities, especially precocious puberty (somatic gain-of-function mutation occurring during embryogenesis in the GNAS gene--> resulting in hyperactive GTP binding protein)
  45. What are the three bone lesions occur in metaphysis near the knee?
    Osteosarcoma, osteochondroma, FCD (NOF), GCT in adolescence
  46. What are the features of monostotic FD?
    • 70% of all cases
    • The femur, tibia, ribs, jawbones, calvaria, and humerus.
    • Asymptomatic but may cause pain, fracture, and discrepancies in limb length or disfigurement
  47. What are the features of Polyostotic fibrous dysplasia without endocrine dysfunction?
    • 27% of all cases
    • Earlier age
    • MC femur, skull, tibia, humerus, ribs, fibula, radius, ulna, mandible, and vertebrae. Craniofacial involvement is present in 50-100% 
    • Propensity to involve the shoulder and pelvic girdles
    • Shepherd-crook deformity of the proximal femur
    • Recurrent Fracture
  48. What are the features of McCune-Albright syndrome?
    • Polyostotic fibrous dysplasia associated with café-au-lait skin pigmentation
    • Sexual precocity (mc), hyperthyroidism, pituitary adenomas that secrete growth hormone, and primary adrenal macronodular hyperplasia
    • Due to somatic gain-function mutation in GNAS
    • The bone lesions are often unilateral and the skin pigmentation is usually limited to the same side of the body.
    • The cutaneous macules are classically large; are dark to café-au-lait; have irregular serpiginous borders (coastline of Maine); and are found primarily on the neck, chest, back, shoulder, and pelvic region.
  49. What are the morphological features of fibrous dysplasia?
    • Well circumscribed, intramedullary
    • Curvilinear trabeculae of woven bone surrounded by a moderately cellular fibroblastic proliferation
    • The shapes of the trabeculae mimic Chinese letters, and the bone lacks prominent osteoblastic rimming
    • Nodules of hyaline cartilage with the appearance of disorganized growth plate
  50. What is the complications of polyostotic fibrous dysplasia
    • Sarcoma
    • Fracture and deformity
  51. What is the radiological hallmark of fibrous dysplasia?
    ground-glass appearance and well-defined margination
  52. What is the difference between Ewing sarcoma and PNET?
    Tumors that demonstrate neural differentiation are labeled PNETs, and those that are undifferentiated are diagnosed as Ewing sarcoma.
  53. Which sarcoma has the youngest age of the diagnosis?
    Ewing
  54. Which bone tumor has a striking predilection for whites?
    Ewing
  55. What is the most common translocation in Ewing sarcoma?
    t(11:22)
  56. What is the genetic basis for Ewing sarcoma?
    A translocation involving the EWS gene on chromosome 22 and a gene encoding an ETS family transcription factor; the most commonly involved ETS gene is FLI1, as part of a (11;22) (q24;q12) translocation
  57. What is the histology of Ewing sarcoma?
    • Uniform small, round cells that are slightly larger than lymphocytes.
    • Scant clear cytoplasm due to glycogen.
    • The presence of Homer-Wright rosettes (tumor cells arranged in a circle about a central fibrillary space) is indicative of neural differentiation. 
    • Little stroma
  58. What is the mc place for Ewing sarcoma?
    diaphysis of long tubular bones, especially the femur and the flat bones of the pelvis
  59. What are the manifestations of Ewing Sarcoma?
    • Painful enlarging masses + tender, warm, and swollen
    • Fever, increased ESR, WBC, anemia
  60. What is the radiological hallmark of Ewing sarcoma?
    • Destructive lytic tumor that has permeative margins and extension into the surrounding soft tissues.
    • The characteristic periosteal reaction produces layers of reactive bone deposited in an onion-skin fashion
  61. Which modality has improved the prognosis of Ewing sarcoma?
    Chemotherapy
  62. What is the hallmark of cells in GCT?
    Mixture of mononuclear cells (neoplastic express RANKL) and a profusion of multinucleated osteoclast-type giant cells (reactive, formed via RANK/RANKL pathway)

  63. What are the mc locations for GCT?
    • Adults--> epiphyses and metaphyses
    • Adolescents -->confined proximally by the growth plate and are limited to the metaphysis.
    • Knee is most common location
  64. What is aneurysmal bone cyst?
    • Multiloculated blood-filled cystic spaces that may present as a rapidly growing expansile tumor.
    • Benign
    • 17p13 translocations that result in up-regulation of USP6, a deubiquitinating enzyme.
  65. What are the histological features of ABC?
    • Approximately one third of cases contain an unusual cartilage-like matrix, called “blue bone.”
    • Multiloculated blood-filled cystic spaces 
  66. What are the manifestation of ABC?
    • Metaphyses of long bones and the posterior elements of vertebral bodies.
    • The most common signs and symptoms are pain and swelling
    • Xray --> aneurysmal bone cyst is usually an eccentric, expansile lesion with well-defined margins. Most are completely lytic and often contain a thin shell of reactive bone at the periphery.
    • CT and MRI --> internal septa and characteristic fluid-fluid levels 
  67. What are the mc tumors that metastasize to the bone
    • Adult--> prostate, breast, kidney, and lung
    • Children--> neuroblastoma, Wilms tumor, osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma
  68. Metastasis from which tumor to the bone are solitary?
    kidney and thyroid
  69. What is the mc place for bone metastasis?
    axial skeleton (vertebral column, pelvis, ribs, skull, sternum), proximal femur, and humerus in descending order of frequency
  70. How is implantation and growth of the tumor cells facilitated in marrow of axial bones?
    The red marrow in these areas (axial), with its rich capillary network and slow blood flow, facilitates implantation and growth of the tumor cells
  71. What is the cause of lytic metastasis?
    • The metastatic cells secrete substances such as prostaglandins, cytokines, and PTH-RP that stimulate osteoclastic bone resorption; the tumor cells themselves do not directly resorb bone.
    • Lysis of bone tissue rich in growth factors enhance tumor growth.
    • Carcinomas of the kidney, lung, and gastrointestinal tract and malignant melanoma produce lytic bone destruction
  72. Which tumor produce blastic metastasis and through which mechanism?
    prostate adenocarcinoma, which may do so by secreting WNT proteins that stimulate osteoblastic bone formation

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