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Bone tumors

BENIGN AND MALIGNANT PRIMARY JAW BONE TUMORS..

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Bone tumors

  1. 1. PRESENTER: DR. KALPAJYOTI BHATTACHARJEE
  2. 2. CONTENTS • Introduction • Classification • Benign tumors of bone • Malignant tumors of bone • Conclusion • References
  3. 3. Introduction • Primary bone tumors are rare; • Non-neoplastic conditions, metastatic disease, and lymphohematologic malignancies, which may simulate primary bone tumors, by far outnumber genuine bone tumors.
  4. 4. • The classification of bone tumor is based on histologic criteria, particularly on the type of differentiation shown by tumor cells and the type of intercellular material they produce, as seen by conventional light microscopy. • However, it is recognized that electron microscopy and especially immunohistochemical techniques may be relevant for precise classification and diagnosis in specific instances.
  5. 5. • The final diagnosis of bone tumors should be based on a synthesis of histopathologic findings, clinical presentation, and imaging characteristics.
  6. 6. Predominant tissue Benign Malignant Bone forming Osteoma Osteoid osteoma and osteoblastoma Osteosarcoma -central -peripheral -parosteal Cartilage forming Chondroma Osteochondroma Chondroblastoma Chondromyxoid fibroma Chondrosarcoma -Juxtacortical chondrosarcoma -Mesenchymal chondrosarcoma -Dedifferentiated chondrosarcoma -Clear cell chondrosarcoma -Malignant chondroblastoma Marrow tumors -Ewing sarcoma -Primitive neuroectodermal tumor of bone (PNET) -Malignant lymphoma of bone -Myeloma WHO Histologic Classification of Bone Tumors 1993
  7. 7. Chondroma • A benign central tumor composed of mature cartilage, is a well- recognized entity in certain areas of the bony skeleton • Considerable clinical importance because of the propensity of the tumor to undergo malignant degeneration in some instances, even after remaining quiescent for long periods of time. • Types: A) Central chondroma / Enchondroma- develop within medullary cavity • B) Periosteal chondroma / Ecchondroma - develops on the surface • C) Soft tissue chondroma.
  8. 8. Clinical feature • Develop at any age and • Shows no apparent gender predilection • Site: maxilla: anterior portion of the maxilla mandible: posterior to the cuspid tooth, involving the body of the mandible, the coronoid or condylar processes. • c/p: arises as a painless, slowly progressive swelling of the jaw, may cause loosening of the teeth
  9. 9. Radiographic Features • Destructive lesion • Irregular radiolucent or mottled area in the bone • Root resorption of teeth adjacent to it
  10. 10. Histological Features • Chondroma is made up of a mass of hyaline cartilage which may exhibit areas of calcification or of necrosis. • The cartilage cells appear small, contain only single nuclei and do not exhibit great variation in size, shape or staining reaction. • vary considerably in appearance from area to area.
  11. 11. A, Chondroma. Strands of epithelium-like cells with abundant eosinophilic cytoplasm reside in a blue-gray mucinous stroma. B, Area of chondroma with tumor cells showing cytoplasmic vacuolation with the formation of multivacuolated physaliferous cells.
  12. 12. A, Enchondroma shows small, uniform chondrocytes whose nuclei are densely hyperchromatic (ink dot) without a visible chromatin pattern. Cells are well separated from each other. B, An island of hyaline cartilage in an enchondroma is separated from the adjacent bone trabeculae by a zone of normal marrow tissue
  13. 13. Osteoma • Osteoma is a benign neoplasm characterized by a proliferation of either compact or cancellous bone, usually in an endosteal or periosteal location. • Described as a specific entity by Jaffe in 1935 • Occurs almost exclusively in the head and neck region • Not a common oral lesion.
  14. 14. Types: Compact osteoma: consists of compact bone, which has a dense lamellae of bone Cancellous osteoma: consisting of trabeculae of bone Periosteal, peripheral or exophytic osteoma: arise on the surface of bone as sessile mass Endosteal or central osteoma: located in the medullary bone Osteoma cutis: extraskeletal lesion of soft tissue seen in the dermis of the skin
  15. 15. Clinical features • Age: second to fourth decades of life, • males> female • c/p: slow growing tumor. • Periosteal origin → circumscribed swelling → obvious asymmetry. • Endosteal origin → expansion of the cortical plates. • Multiple osteomas of the jaws, as well as of long bones and skull, are a characteristic manifestation of Gardner syndrome.
  16. 16. GARDNER SYNDROME • It is an autosomal dominant disorder. • Mutation in APC gene • characterized by the triad of colonic polyposis, multiple osteomas and mesenchymal tumors of the skin and soft tissues including epidermal inclusion cyst, lipoma, fibroma, and fibromatosis
  17. 17. Oral manifestations • Multiple odontomas, Compound odontomas • Supernumerary teeth • Impacted permanent teeth • One or more osteomas of the jaws • Occult radiopaque lesions of the jaws are common • Various incidental findings include hypercementosis, root resorption, ankylosis and persistent primary teeth.
  18. 18. Histologic Features. • Composed either of extremely dense, compact bone or of coarse cancellous bone. • Bone formed appears normal • Well circumscribed. • In some tumors foci of cartilage may be found, in which case the term ‘osteochondroma’ is often used. • Myxomatous tissue also may be intermingled on rare occasions.
  19. 19. Compact and trabecular bone is present beneath intact mucosa at the left of the field. B, Compact cortical-type bone of the osteoma shown in A contains haversian systems
  20. 20. Treatment and Prognosis • Symptomatic lesions→ local excision. • No recurrence after surgical removal.
  21. 21. Osteoid osteoma • Benign tumor of bone, seldom been described in the jaws. Etiology: • True nature → unknown. • Jaffe and Lichtenstein have suggested “A true neoplasm of osteoblastic derivation”. • Trauma • Inflammation
  22. 22. Classification Cortical Cancellous Intra- articular Subperiosteal
  23. 23. Clinical Features. • Age: young persons, under the age of 10 years • Sex: males:female - 2:1. • Site: Frequently in the femur or in the tibia. • In head and neck→ Cervical spine > mandible and maxilla. • Chief symptoms → severe pain → unrelenting and sharp, worse at night. • Classically, the pain is relieved by aspirin.
  24. 24. Pathologic Features • In its active growth phase considerable vascularity. • Grossly appears as a discrete, round to oval lesion marked by a cherry-red or reddish-brown color. Quite granular and friable and easily displaced from the adjacent bone. • In its mature phase → more calcification and bone production, the lesion is hard and gritty and blends with the bone around it.
  25. 25. Histologic Features • Characteristic and consists of a central nidus composed of compact osteoid tissue, varying in degree of calcification, interspersed by a vascular connective tissue. • Formation of definite trabeculae occurs, particularly in older lesions, outlined by active osteoblasts. • Osteoclasts and foci of bone resorption are also usually evident. • Overlying periosteum exhibits new bone formation, and in this interstitial tissue collections of lymphocytes seen.
  26. 26. A, Nidus of osteoid osteoma abuts thickened mature bone. B, Osteoid trabeculae, some partially calcified, within the nidus of an osteoid osteoma. The trabeculae are rimmed with plump osteoblasts with occasional osteoclasts. The stroma is hemorrhagic.
  27. 27. Ultrastructural investigation by Steiner, • The morphology of the osteoblasts to be similar to that of normal osteoblasts. • Although atypical mitochondria could be seen. • Neural staining → many axons throughout an osteoid osteoma, which probably accounts for the pain (the nidus). • ↑Levels of prostaglandin E2 in the nidus; this is presumably the cause of pain and vasodilatation.
  28. 28. Treatment • NSAIDS- Aspirin relieves the pain • Surgical curettage • Lasers
  29. 29. Benign Osteoblastoma (Giant osteoid osteoma) • Osteoblastoma accounts →1% of primary bone tumors. • It is typically a slow-growing, benign bone tumor. • Incidence in the head and neck → 13% to 26%. • Osteoblastoma frequently lacks the characteristic pain and the halo of sclerotic bone associated with osteoid osteoma. • Benign osteoblastoma → Jaffe and by Lichtenstein in 1956.
  30. 30. Etiopathogenesis • Jaffe and Lichtenstein stated this lesion to be “a true neoplasm of osteoblastic derivation”. • Trauma, • Inflammation, • Abnormal local response of the tissues to injury, and • Local alteration in bone physiology
  31. 31. Clinical Features • Age: in young persons, 20-30 years. • Sex: Males>Females. • C/P: characterized clinically by pain and swelling. pain → more generalized and less likely relieved by salicylates. • Most common site → vertebral column. • Mandible > Maxilla • Occurs in Periosteal, cortical, or medullary location
  32. 32. Pathologic Features. On gross examination, • Well delimited within either the cortex or cancellous bone. • Hemorrhagic • Gritty or granular consistency with cystic regions.
  33. 33. Histologic Features The hallmark of the benign osteoblastoma consists of: The vascularity of the lesion with many dilated capillaries scattered throughout the tissue The moderate numbers of multinucleated giant cells scattered throughout the tissue, and The actively proliferating osteoblasts which pave the irregular trabeculae of new bone It may or may not have a central sclerotic nidus
  34. 34. • In the less mature lesion → abundance of connective tissue stroma in which osteoclast-type giant cells and small foci of osteoid are present, some in a lacelike pattern. • With maturation → progressive mineralization of the osteoid with conversion to trabeculae of coarse woven bone, rimmed by plump osteoblasts. The trabeculae may fuse to form an anastomosing, netlike pattern. • The osteoblasts usually lack any significant atypia, having round to oval regular nuclei, often with prominent nucleoli. Mitotic activity is infrequent. • The combination of bone production and resorption → pagetoid - appearing bone with prominent cement lines
  35. 35. A, Nidus of osteoblastoma shows active production of osteoid trabeculae, some in the early stage of bone formation. The trabeculae are lined with enlarged osteoblasts with occasional osteoclasts. Numerous dilated capillaries are present in the stroma. B, Large epithelioid osteoblasts, in osteoblastoma, have abundant cytoplasm and large nuclei containing prominent nucleoli. Formation of lacelike osteoid is seen.
  36. 36. Osteoblastoma comprises interanastomosing trabeculae of woven bone lined by a single layer of osteoblasts within a loosely textured fibrovascular stroma The nidus is formed by dense sclerotic woven bone
  37. 37. irregular or mosaic-like reversal lines indicative of active remodeling similar to that seen in Paget disease Tumor trabeculae frequently connect with the surrounding bone
  38. 38. Extensive intralesional hemorrhage Parallel arrays of sclerotic bone that radiate away from center
  39. 39. Aggressive Osteoblastoma It is primarily defined by epithelioid osteoblasts, cells with abundant eosinophilic cytoplasm twice the size of conventional osteoblasts. These cells are frequently arranged in sheets with little or no intervening osteoid Cytologically, the neoplastic osteoblasts have abundant basophilic, finely granular cytoplasm with a perinuclear holo of less dense cytoplasm and an eccentric vesicular nucleus with a solitary prominent nucleolus
  40. 40. Differential Diagnosis • Osteoid osteoma • Aneurysmal bone cyst • Osteoblastoma like Osteosarcoma
  41. 41. Treatment and Prognosis: • Conservative surgical excision • Recurrence is rare.
  42. 42. OSTEOCHONDROMA • Osteochondroma or solitary osteo-cartilaginous exostosis is an exophytic lesion that arises from the cortex of bone and is capped with cartilage. • 35%-50% of all benign bone tumors • 8%-15% of all primary bone tumors • Occurs frequently in the metaphyseal region of the long bones • Osteochondroma can eventually transform into a secondary peripheral chondrosarcoma in 1–3% of patients with multiple osteochondromas.
  43. 43. Etiology: • Different theories of etiopathogenesis proposed:  Developmental,  Reparative, and  Traumatic  Radiation-induced osteochondroma  Stress
  44. 44. Clinical features • Age- 13-78 years, mean age- 38.4 years • Sex: females> males • Site: coronoid process and the mandibular condyle are the affected. Especially the medial aspect of the mandibular condyle. • slow growing. Clinical presentation: • facial asymmetry, malocclusion, cross-bite on contra-lateral side and lateral open-bite on the affected side, deviation on opening, hypomobility, pain and clicking
  45. 45. A, Peripheral portion of osteochondroma shows a cartilage cap covered by a layer of periosteum (perichondrium). Active enchondral ossification is present, with widely dilated capillaries present at the base of the cartilage. The marrow is filled with fat. B, Bone within osteochondroma shows persistence of partially ossified hyaline cartilage within the centers of the trabeculae.
  46. 46. Treatment: • Surgical excision • Condylectomy
  47. 47. OSTEOSARCOMA / OSTEOGENIC SARCOMA • Osteosarcoma is the third most common cancer in adolescence, occurring less frequently than only lymphomas and brain tumors. • It is thought to arise from a primitive mesenchymal bone- forming cell and is characterized by production of osteoid
  48. 48. Etiology • Irradiation : 2% of osteosarcomas. • pre-existing benign bone disorders – bone dysplasia, fibrous dysplasia, Pagets disease • Trauma • Disturbance of bone growth and maturation - corresponds with growth spurt
  49. 49. • Environmental factors:  Ultraviolet and ionising radiation  Viral origin: simian virus 40 (SV40) • Transcription Factors  Excess production of transcription factors, or the production of a new overactive transcription factor, may result from gene rearrangement.  Overexpression of Myc • Growth Factor  Dysregulated expression of growth factors such as TGF, IGF, and CTGF leads to the accelerated proliferation of cells.
  50. 50. • Genetic predisposition Alterations in genetic pathways including Rb, p53, SAS (sarcoma amplified sequence) Protein expression of the defective/amplified genes results in loss of control of cell proliferation and differentiation • Syndromes – Li-Fraumeni syndrome - Rothmund-Thompson syndrome
  51. 51. Classification of osteosarcoma Primary osteosarcomas • Conventional-sub-typed as:  Osteoblastic (50%)  Chondroblastic (25%)  Fibroblastic (25%) • Small cell • Telangiectatic • Intraosseous well differentiated and Intracortical • Surface osteosarcomas:-Parosteal, Periosteal, High grade surface
  52. 52. • Secondary osteosarcomas  Paget’s disease and after radiation exposure. • Unusual forms of osteosarcoma subtypes of conventional osteosarcoma because their biological behavior is similar.  Osteoblastic osteosarcoma-sclerosing type  Osteosarcoma resembling osteoblastoma  Chondromyxoid fibroma-like osteosarcoma  Chondroblastoma-like osteosarcoma  Clear-cell osteosarcoma  Malignant fibrous histiocytoma-like osteosarcoma  Giant cell rich osteosarcoma
  53. 53. Clinical features • Sex- M>F • Age – 3rd and 4th decade • Site - metaphysial growth plates of extremities of long bones • femur>tibia>humerus>skull or jaw>pelvis • Mandible : Maxilla = 1.5:1 • Mandible: body of the mandible > symphysis > angle of the mandible > ascending ramus >TMJ.
  54. 54. • Maxilla : alveolar ridge and maxillary antrum, palate • C/P: Painless or painful bony swelling • Facial deformity, loose teeth, toothache, • Numbness & limited mouth opening • Suppuration
  55. 55. • By their site of origin:  the conventional type - arising within the medullary cavity  juxtacortical tumors - arising from the periosteal surface  extraskeletal osteosarcomas - rare.
  56. 56. Sunray or sunburst pattern Codmans triangle
  57. 57. Gross pathology • Osteoblastic - white-tan, yellow in color, firm in consistency • Chondroblastic - translucent lobules • Fibroblastic - tan colored with soft or firm in consistency
  58. 58. Histological features • Presence of osteoid formation by malignant osteoblasts • Stromal cells are spindle shaped and atypical with irregularly shaped nuclei • The amount of matrix material produced in the tumor varies considerably. • Mitotic activity with frequent abnormal forms
  59. 59. • Depending on the relative amounts of osteoid, cartilage. or collagen fibers produced by the tumor.  Osteoblastic  Chondroblastic  Fibroblastic
  60. 60. Osteoblastic type • Atypical neoplastic osteoblasts that exhibit variation in size and shape • large deeply staining nuclei arranged in disordered fashion about trabaculae of bone . • Irregular pattern or solid sheets of new tumor osteoid and bone formation.
  61. 61. • Fibroblastic type- varying degrees of proliferation of anaplastic fibroblasts, absence of tumor osteoid. • Occasional areas of neoplastic myxomatous tissue and cartilage. • Comprised about 34%
  62. 62. • Chondroblastic- currently believe that even though a lesion is composed chiefly of malignant cartilage, it should be diagnosed as osteosarcoma, if significant malignant osteoblasts and tumor osteoid or bone can be identified since the course of the lesion will probably be that of an osteosarcoma rather than of a chondrosarcoma
  63. 63. Lacelike streamers of pink osteoid produced by malignant stromal cells (osteoblasts). Area in a conventional osteosarcoma shows a combination of osteoid, malignant cartilage, and spindle cell fibrous zones
  64. 64. Telangiectatic osteosarcoma resemble an aneurysmal bone cyst. Blood filled cystic spaces are separated by delicate septa. Benign giant cells resembling osteoclasts are seen in about 25% of osteosarcomas.
  65. 65. Small round cell osteosarcoma
  66. 66. Stages • Stage I – Low-grade lesions • Stage II – High-grade lesions • Stage III – Metastatic disease Substages • A – Intramedullary lesion • B – Local extramedullary spread Site of primary • Distal extremity – Best prognosis • Distal femur – Intermediate prognosis • Axial skeleton – Worst prognosis
  67. 67. Biochemical markers • ↑Alkaline phosphatase - due to increased osteoblastic activity • ↑ Lactate dehydrogenases (LDH)
  68. 68. Emerging prognostic factors Ezrin P53 VGEF Chemokines Micro ribonucleic acid (miRNA) RANKL MMP’s
  69. 69. Differential diagnosis • Malignant fibrous histiocytoma • Fibrosarcoma • Giant cell tumours • Ewing's sarcoma • Osteoblastoma
  70. 70. Treatment • Long bone involvement→ amputation is a prime requisite. • Radical resection • Primary X-ray radiation is of no avail. • Preoperative chemotherapy →facilitate subsequent surgical removal by shrinking the tumor. • Adjuvant chemotherapy in combination with surgery, including resection of pulmonary metastases, has appeared to offer promise of increased survival from this disease • Overall 5 years survival – 63%
  71. 71. CHONDROSARCOMA • Chondrosarcoma is a malignant tumor characterized by the formation of cartilage. • Comprise about 10% of all primary tumors • 1 % to 3% arise in the head and neck area. Types: • Primary: arise directly from the cartilage • Secondary: develop in a pre-existing benign cartilaginous tumor.
  72. 72. Clinical features • Age: 6th- 7th decade • No significant sex predilection • Site: In head and neck→ maxilla, mandible, base of the skull, cervical vertebrae, nasal cavity and nasal septum. • c/p: painless mass or swelling, loosening of teeth. • Maxillary tumors may cause nasal obstruction, congestion , epistaxis, photophobia, or visual loss.
  73. 73. Gross examination • On sectioning→ lobular, blue-gray to gray-white, translucent, glistening surface, although firm, they are usually easily cut with a scalpel, except for areas with dense calcification or ossification. • Necrosis within the center of the lobules is common.
  74. 74. Histopathologic Features • Chondrosarcomas are composed of cartilage showing varying degrees of maturation and cellularitywith typical lacunar formation. • Lobular growth pattern, with tumor lobules separated by thin fibrous connective tissue septa. • Central areas of the lobules →greatest degree of maturation. • Peripheral areas → immature cartilage & round or spindle shaped cells. • Neoplastic cartilage may be replaced by bone in a manner similar to normal endochondral ossification.
  75. 75. • Grade I chondrosarcomas closely mimic the appearance of a chondroma, composed of chondroid matrix and chondroblasts • Large, plump chondroblasts and binucleated chondrocytes seen. • Calcification or ossification prominent, and mitoses are rare. • Grade II chondrosarcomas show a greater proportion of moderately sized nuclei and increased cellularity. More myxoid with a less prominent hyaline matrix. • Grade III chondrosarcomas are highly cellular and may show a prominent spindle cell proliferation. Mitoses may be prominent .
  76. 76. Low-grade chondrosarcoma. The tumor cells are larger than normal chondrocytes, with larger, open-faced nuclei that have a uniform, fine chromatin pattern. Several mitotic figures are present, an uncommon finding in most chondrosarcomas High-grade chondrosarcoma. Hypercellular tumor contains pleomorphic cells, some with large, bizarre nuclei. A few cells are spindle shaped
  77. 77. Variants • Clear cell chondrosarcoma • Myxoid chondrosarcoma • Mesenchymal chondrosarcoma • Dedifferentiated chondrosarcoma
  78. 78. infiltration between existing normal bone, resulting in trabeculae that are closely abutted and surrounded by tumor. Mesenchymal chondrosarcoma. showing sheets of small basophilic cells with focal areas of cartilaginous differentiation (right).
  79. 79. A, myxoid chondrosarcoma. Tumor cells are more closely arranged at the periphery of the lobules. B, Radial, cordlike arrangement of tumor cells in myxoid chondrosarcoma. Cells are embedded in grayish myxoid stroma
  80. 80. Differential diagnosis • Chondroma • Chondroblastic osteosarcoma
  81. 81. Treatment and Prognosis • Prognosis in chondrosarcoma depends primarily on the ability to adequately excise the tumor • Radical surgical excision. • Radiation and chemotherapy are less effective • 5-year survival rate →43% to 95%
  82. 82. Primitive neuroectodermal tumor (PNET) • Term used to describe a category of neoplasm of neuroectodermal origin with variable cell differentiation. • “small round cell tumors of childhood” Divided into two categories • Group (I) tumors→ pituitary adenomas and carcinoid tumors, represent tumors that show predominantly epithelial differentiation. • Group (II) tumors→ Olfactory neuroblastoma, Malignant melanoma, Ewing’s sarcoma (EWS) display features that are predominantly neural and non-epithelial in origin
  83. 83. EWINGS SARCOMA • Ewing’s sarcoma is a sarcoma of the bone, classically described under small round cell tumors. • uncommonly involve the head and neck • Incidence →1-3 cases per million of population per year. • Skull tumors constitute about 2% of tumors. • James Ewing (1866–1943) first described the tumor
  84. 84. Cell of origin
  85. 85. Pathogenesis • Trauma • Balanced t(11:22) (q24;q12) chromosomal translocation- 85% • EWS-FLI1 → central player in the pathogenesis of ES • Overexpression of CD99 • Dysregulated signaling of receptor tyrosine kinase. • Altered pathways of RB and p53
  86. 86. Some of the potential molecular targets of Ewing’s sarcoma described in this review include: (a) EWS-FLI1 fusion protein, (b) its target genes, (c) growth factor receptor, cell- surface receptors and (d) molecules involved in cell survival, proliferation and anti- apoptotic pathways
  87. 87. Clinical Features • Age: children and young adults, 5-25 years, • Male: female= 2:1 • uncommon in blacks. • Site: long bones of the extremities, In head and neck region, It involves skull, clavicle, maxilla and mandible. Mandible ˃ maxilla.
  88. 88. • Earliest sign: Pain, usually of an intermittent nature, and Swelling of the involved bone • Facial neuralgia and lip paresthesia • Jaw swelling • Ulcerated intraoral mass • Low -grade fever • Elevated white blood cell count • Extraskeletal form- Ewing’s Sarcoma of soft tissue.
  89. 89. ‘onion skin’ appearance ‘sun-ray’ appearance
  90. 90. Histologic Features • Extremely cellular neoplasm composed of solid sheets or masses of small round cells with very little stroma. • Cells are small and round, with scanty cytoplasm and relatively large round or ovoid nuclei with dispersed chromatin and hyperchromasia. • Cell borders are indistinct.
  91. 91. • Mitotic figures are common. • Cells are positive for glycogen and are diastase resistant • Geographic necrosis with perivascular sparing • Hemorrhage many mitotic figures in the field
  92. 92. A, A lobular arrangement of primitive round cells with cytoplasmic clearing in Ewing’s sarcoma/primitive neuroectodermal tumor. B, The cells of Ewing’s sarcoma/primitive neuroectodermal tumor are usually uniform and small with finely dispersed chromatin and small nucleoli.
  93. 93. Staging for Ewings sarcoma
  94. 94. Differential diagnosis • Small cell osteosarcoma, • Mesenchymal chondrosarcoma. • Metastatic neuroblastoma,
  95. 95. Treatment and Prognosis • Chemotherapy • Radiation therapy • Surgery • Five-year survival with a combination of surgery and chemotherapy is 74%.
  96. 96. Multiple Myeloma • Most common primary neoplasm of the skeletal system. • Malignancy of plasma cells. • Plasma cells are a subset of B-cells, which are the producers of humoral immunity factors termed antibodies. • Underlying pathology → Expansion of a single line of plasma cells that replace normal bone marrow and produce monoclonal immunoglobulins. • Diffuse disease of the bone marrow.
  97. 97. Etiology • Radiation exposure • Occupational exposure • Chemical exposure
  98. 98. Pathogenesis
  99. 99. • Frequent aberrations of chromosomes 1 and 14 • Other chromosomal abnormalities include 6q-, 7q-, 5q- • Abnormal expression of the bcl-2 protein • Mutations of the ras oncogene and p53 gene mutations • Interleukin-6 (IL-6), considered the most important myeloma growth factor
  100. 100. Clinical features • Age: 60-65 years • Sex: males> females • More common among black people • Site: mandible> maxilla • Number of lytic foci or diffuse demineralization. • Anemia, azotemia, hypercalcemia, recurrent infection. • Extramedullary plasmacytoma- tonsils, nasopharynx, or paranasal sinuses
  101. 101. Macroglobulinemia. • Macroglobulinemia is a proliferation of plasmacytoid lymphocytes secreting an IgM-protein. • Patients often have lymphadenopathy and hepatosplenomegaly, • Bony lesions are uncommon.
  102. 102. Histologic Features • Composed of sheets of closely packed cells resembling plasma cells → round or ovoid cells with eccentrically placed nuclei exhibiting chromatin clumping in a ‘cartwheel’ or ‘checkerboard’ pattern • Two nuclei within a single cell membrane are seen • Perinuclear halo may be present. • Russell bodies are common
  103. 103. Ultrastructurally, • Numerous mitochondria in a perinuclear distribution as well as prominent Golgi complexes. • Golgi complexes are most likely responsible for the perinuclear halo which is observed by light microscopy. (Chen)
  104. 104. Laboratory Features • Hyperglobulinemia (monoclonal gammopathy) • Hypercalcemia • Hyperuricemia • ↑ESR level • Bence Jones protein in the urine → 60–85% • Unusual protein which coagulates when the urine is heated to 40°–60° C and then disappears when the urine is boiled. It reappears as urine is cooled.
  105. 105. Treatment and Prognosis. • Bisphosphonate therapy → reduction of osteoclastic activity and bone mineralization maintenance. • Chemotherapy • Extramedullary plasmacytoma → radiation therapy • Infection, anemia and kidney failure are the most common immediate causes of death
  106. 106. CONCLUSION • The possibility for the pathologist to correctly diagnose a bone tumor depends to a large extent on the completeness of the clinical and imaging information provided. • Care of the patient requires a multidisciplinary approach  Pathologist  Radiologist  Orthopedic surgeon  Medical oncologist  Radiation oncologist
  107. 107. REFERENCES  Rajendran R, Sivapathasundharam B. Shafer’s Textbook of Oral Pathology. 7th edition. Elsevier publication;941-1038  Neville et al. Oral & Maxillofacial Pathology. 2nd edition. Elsevier publication.  Gnepp. Diagnostic Surgical Pathology Of The Head And Neck. 2nd Edition.  Ghoms.  Lars Gunnar Kindblom. Bone Tumors: Epidemiology, Classification, Pathology.  Fritz Schajowicz, M.D. The World Health Organization’s Histologic Classification of Bone Tumors. CANCER March 1,1995, Volume 75, No. 5  David R. Lucas. Osteoblastoma. Arch Pathol Lab Med. 2010;134:1460–1466
  108. 108.  Ferdem N, Manisali M. Osteochondroma of mandibular condyle: A case report. Annals of Oral & Maxillofacial Surgery 2014 Jun 08;2(2):11 Rani PS, Shyamala K, Girish HC, Murgod S. Pathogenesis of Ewing sarcoma: A review. J Adv Clin Res Insights 2015;2:164-168. Jully B, Rajkumar T. Potential molecular targets for Ewing's sarcoma therapy. Indian J Med Paediatr Oncol 2012;33:195-202. Yuwanati MB. Primitive neuroectodermal tumor of the posterior mandible: A case report. J Clin Exp Invest 2013; 4 (1): 101-104 . Kundu ZS. Classification, imaging, biopsy and staging of osteosarcoma. Indian J Orthop 2014;48:238-46.
  109. 109. Thank you

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