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Malignant connective tissue tumors.pptx
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- 2. FIBROSARCOMA OSTEOSARCOMA HODGKIN’S LYMPHOMA BURKITT’S LYMPHOMA KAPOSI’S SARCOMA PLASMACYTOMA MULTIPLE MYELOMA MALIGNANT TUMOURS
- 3. FIBROSARCOMA Malignant tumourof fibroblasts Can occur as soft tissue mass or as a primary bone tumour or secondary bone tumour Etiology No definite cause is known May arise from pre-existing lesions like Neurofibromas in neurofibromatosis Fibrous dysplasia Chronic osteomyelitis Bone infarcts Paget’s disease of bone Previously irradiated areas of bone
- 4. CLASSIFICATION SOFT TISSUEFIBROSARCOMA: occurring in the soft tissues FIBROSARCOMA OF BONE PRIMARY PERIPHERAL: Arising from the periosteum CENTRAL : Arising from the medullary cavity SECONDARY: Arises from the pre-existing lesions or in previously irradiated areas
- 5. CLINICAL FEATURES Represent10% of musculo-skeletal sarcomas and 5% of all primary tumours of bone Age: In adults 35-55 years Infantile form: occurs in children < 20 years Gender: fibrosarcoma of bone has male predilection Location: most common in the extremities 10% occur in head and neck
- 6. CLINICAL PRESENTATION SOFTTISSUE FIBROSARCOMA Large painless mass deep to the fascia Has ill-defined margins FIBROSARCOMA OF BONE Present with pain and swelling May grow to a large size and can cause pathological fractures PRIOR H/O IRRADIATION AND OTHER DISEASE HAVE TO BE ENQUIRED: COULD BE SECONDARY FIBROSARCOMA Infantile form has excellent prognosis, unlike the fibrosarcoma of adults
- 7. HISTOPATHOLOGY They varyin histologic grade WELL DIFFERENTIATED Multiple plump fibroblasts with pale eosinophilic cytoplasm and deeply stained spindled nuclei Malignant cells are dispersed in rich collagen background Histologically normal mitotic figures are seen in few numbers Cells and nuclei are not pleomorphic INTERMEDIATE GRADE Cellular Have HERRING BONE PATTERN: parallel sheets of cell arranged in intertwining whorls Slight degree of cellular pleomorphism Moderate amount of mature collagen
- 9. HISTOPATHOLOGY HIGH GRADE Very cellular with marked cellular atypia Marked mitotic activity Sparse matrix Multinucleated giant cells may be seen NO MALIGNANT OSTEOID FORMATION SHOULD BE SEEN Extremely anaplastic and pleomorphic cells with bizzare nuclei
- 11. TREATMENT AND PROGNOSIS Radical surgery including removal of invaded muscle and bone Chemotherapy and radiotherapy can also be used in conjunction with surgery Survival rate: 5-year: 65% 10-year: < 30% Secondary fibrosarcoma: < 10% at 10 years
- 12. OSTEOSARCOMA Also knownas osteogenic sarcoma Malignancy of mesenchymal cells that have the ability to produce osteoid or immature bone Etiopathogenesis Retinoblastoma (RB) gene mutation TRAUMA Bone dysplasias: fibrous dysplasia, Paget’s disease of bone Li Fraumeni syndrome Rothmund Thompson syndrome Radiation therapy for some benign lesions like giant cell granuloma
- 13. CLINICAL FEATURES Age:bimodal age distribution is seen First peak: 10 – 25 years (dramatic increase in adolescence corresponds with the growth spurt) Second peak: after 50 years Gender: Slight male predilection M:F = 1.25 : 1 Location: majority occurs in intramedullary location location also corresponds with the areas of bone growth Initial peak is more common in long bones In older patients axial skeleton and flat bones are most frequently involved Paget’s disease and previous irradiation are associated with increased prevalence
- 14. CLINICAL PRESENTATION Swellingand pain; particularly with activity of the involved bone ORAL MANIFESTATIONS Osteosarcomas of jaw are uncommon (6-8%) Most common in 3rd and 4th decades (10 -15 years older than the osteosarcomas of long bones) Mandible and maxilla are involved with equal frequency Mandibular tumours: posterior body and ramus of mandible Maxillary lesions: alveolar ridge, sinus floor and palate Swelling and pain are the most common symptoms Loosening of teeth and paraesthesia are also seen Nasal obstruction is seen with maxillary tumours
- 15. RADIOGRAPHIC FEATURES Threeclassic features are seen SUNRAY OR SUNBURST APPEARANCE: Small streaks of bone radiate outward Due to osteophytic bone production on the surface of the lesion Appreciated best on occlusal radiographs Tumour may grow within the PDL space causing resorption of the adjacent bone: resulting in uniform widening of the PDL space This is an early radiographic change CODMAN’S TRIANGLE: In long bones affected with osteosarcoma the periosteum is elevated over the expanding tumour mass in a tent-like fashion At the point where periosteum begins to merge, an acute angle is formed between the bone surface and the periostium This feature is highly suspicious of osteosarcoma
- 17. HISTOPATHOLOGY Proliferation ofatypical osteoblasts Presence of osteoid formed by malignant osteoblasts Stromal cells may be round or spindle shaped and atypical with irregularly shape nuclei In addition to osteoid cells may also produce chondroid or fibrous connective tissue Depending on the relative amounts of the type of tissue produced; osterosarcoma is classified into three types: OSTEOBLASTIC TYPE CHONDROBLASTIC TYPE FIBROBLASTIC TYPE
- 20. TREATMENT AND PROGNOSIS Radical resection is the treatment of choice Neoadjuvant(preoperative) chemotherapy: to shrink the tumour size Adjuvant chemotherapy: to increase the survival rate of the disease Overall survival rate is 63%
- 21. HODGKIN’S LYMPHOMA Alsoknown as Hodgkin’s disease or Malignant Lymphoma It is a malignant lymphoproliferative disorder THOMAS HODGKIN first described Hodkin’s disease in 1832 ETIOLOGY Unknown Infectious agents; especially Epstein Barr virus Patients with HIV infection have higher incidence Genetic predisposition 1% have family history Siblings of patients have increased risk Risk is even more higher in monozygotic twins
- 22. CLINICAL FEATURES Bimodalage distribution is seen 15-34 years >55 years Gender: male predilection; particularly in children Race: more common in whites; less common in Asians Location: always begins in Lymph nodes; any lymph node group may be susceptible Most common ones: cervical and sub-clavicular lymph nodes (70-75%) Followed by axillary and mediastinal nodes (5-10% each) Less than 5% initial presentation in abdominal and inguinal nodes
- 23. CLINICAL PRESENTATION Presentingsymptom: persistently enlarging non- tender, discrete mass or masses in one lymph node region Nodes are firm and rubbery in consistency Overlying skin is normal In the early stages, nodes are movable; with progression they become matted and fixed to surrounding tissues If untreated, condition spreads to other lymph node groups Eventually it involves spleen and other lymphatic tissues like bones, liver and lungs
- 25. CLINICAL PRESENTATION Constitutionalsymptoms: Fever Night sweats Generalised pruritis ORAL CAVITY may be secondarily involved Very rare
- 26. HISTOPATHOLOGY CLASSIFICATION Nodular- Lymphocyte predominant HL Classic HL Lymphocyte rich Nodular sclerosis Mixed cellularity Lymphocyte depletion Unclassifiable
- 27. HISTOPATHOLOGY Common features Effacement of normal nodal architecture by diffuse mixed infiltrate of inflammatory cells Interspersed with large ATYPICAL NEOPLASTIC LYMPHOID CELLS In classic type this atypical cell is known as REED STERNBERG CELL Malignant cell in nodular-lymphocyte predominant HL is POPCORN CELL Reed sternberg cell Typically binucleated (owl-eye nuclei) Sometimes may also be multinucleated (pennies on a plate) with prominent nucleoli Popcorn cell: nucleus resembles kernel of popped corn
- 32. TREATMENT Local radiationtherapy Combination of less extensive radiotherapy and mild multiagent chemotherapy Lymphocyte predominant type has most favourable prognosis Lymphocyte depletion type has worst prognosis
- 33. BURKITT’S LYMPHOMA Namedafter DENIS BURKITT Also called as AFRICAN JAW LYMPHOMA: because it is common in Sub-Saharan Africa It is a malignancy of B-lymphocyte origin One of the fastest growing malignancies in humans
- 34. CLASSIFICATION Endemic Burkitt’s lymphoma Sporadic (non-endemic) form or American Burkitt’s Lymphoma Immunodeficiency associated Burkitt’s lymphoma (HIV – related)
- 35. ETIOLOGY Exact causeand mechanism are not known Probable causes c-myc oncogene mutation Epstein- Barr virus EBV is more commonly associated with the African form
- 36. CLINICAL FEATURES AGE Endemic form usually affects children (peak prevalence = 7yrs) Sporadic form tends to affect patients over a greater age range LOCATION 50% - 70% of the endemic type present in jaws Involvement of abdominal organs is slightly less common Sporadic form usually involves the abdominal organs Endemic form Male predilection Posterior segments of the jaws are more commonly involved Maxilla : mandible = 2:1 Sometimes all four quadrants of the jaws may show
- 37. CLINICAL PRESENTATION Facialswelling and proptosis Pain, tenderness and paraesthesia are minimal Tooth mobility is common : because of aggressive destruction of the alveolar bone Premature exfoliation of the deciduous teeth Enlargement of the gingiva and alveolar processes are other common symptoms
- 38. RADIOGRAPHIC FEATURES Radiolucentdestruction of the bone with ragged and ill-defined margins Radiolucency may begin as several small areas which eventually enlarge and coalesce Patchy loss of the lamina dura is one of the early sign of Burkitt’s lymphoma
- 39. HISTOPATHOLOGY Monoclonal proliferationof B lymphocytes Lesion invades as broad sheets of tumour cells Tumour cells exhibit round nuclei with minimal cytoplasm Each tumour nucleus has prominent nucleoli Numerous mitoses are seen At low power magnification: STARRY SKY PATTERN is often appreciated
- 42. HISTOPATHOLOGY STARRY SKYPATTERN Phenomenon that is caused by the presence of macrophages within the tumour tissue Macrophages have abundant cytoplasm which microscopically appears less stained in comparison with the surrounding cells These macrophages also contain phagocytic debris THE MACROPHAGES THUS, STAND OUT AS STARS SET AGAINST THE NIGHT SKY OF DEEPLY HYPERCHROMATIC NEOPLASTIC LYMPHOID CELLS
- 43. TREATMENT Intensive chemotherapy Survival rate has increase upto 75% - 85% with recent advancements in the treatment modalities
- 44. KAPOSI’S SARCOMA Synonyms: Angioreticuloendothelioma Multiple Idiopathic Hemorrhagic sarcoma of Kaposi Kaposi’s sarcoma is a multicentric proliferation of vascular and spindle cell components First described in 1872 by Moritz Kaposi. He described the lesion as “ Idiopathic multiple pigmented sarcoma of skin”
- 45. ETIOPATHOGENESIS Human Herpesvirus 8 (KSHV) Has increased propensity to develop in individuals infected with HIV; however HIV doesn’t seem to be the direct cause of the tumorous proliferation
- 46. CLASSIFICATION CLASSIC ORCHRONIC KAPOSI’S SARCOMA ENDEMIC OR AFRICAN KAPOSI’S SARCOMA IATROGENIC IMMUNOSUPPRESION ASSOCIATED AIDS RELATED
- 47. CLINICAL PRESENTATION Classictype Associated with altered immune status as well as lymphoreticular and other malignancies Presents as cutaneous multi-focal blue-red macules, nodules and plaques on lower extremities Slowly increase in size and number Some lesions regress while new ones develop on adjacent or distant skin ORAL INVOLVEMENT IS RARE; if occurs it is seen on palatal mucosa and gingiva
- 49. CLINICAL PRESENTATION Endemictype: endemic to African children 4 types Benign nodular type: similar to classic type Aggressive or infiltrative type: locally invasive Florid form: widely disseminated; rapidly progressive Lymphadenopathic type: tumours of lymph nodes Iatrogenic type Occurs in recipients of organ transplants Related to loss of immunity secondary to immunosuppressive drugs More aggressive Rare oral involvement
- 50. CLINICAL PRESENTATION AIDSrelated Kaposi’s sarcoma Early sign of AIDS Multiple lesions of skin and mucosa 70% of patients demonstrate oral lesions; in 22%,oral lesions are the first manifestation Hard palate, gingiva and tongue are commonly affected Palatal and gingival lesions invade bone and cause tooth mobility Present as brown or reddish purple macular lesions that do not blanch on pressure Later macules develop into plaques or nodules Pain, bleeding and necrosis are seen
- 53. HISTOPATHOLOGY SIMILAR HISTOPATHOLOGICAPPEARANCE IS SEEN IN ALL THE CLINICAL TYPES Kaposi’s sarcoma evolves through 3 stages Patch stage Plaque stage Nodular stage Patch stage: Proliferation of small veins and capillaries around pre- existing dilated vessels Slit-like vessels are seen which are lined by plump, mildly atypical endothelial cells Scattered erythrocytes and hemosiderin deposits are seen Perivascular spindle cell proliferation with minimal cellular atypia
- 54. HISTOPATHOLOGY Plaque type Increased number of small capillaries and dilated vascular channels Slit-like vascular channels without visible endothelial lining Large number of extravasated RBCs and abundant hemosiderin deposits Proliferating sheets of sarcomatous or atypical spindle cells Lesional cells have enlarged hyperchromatic nuclei with mild to moderate pleomorphism Minimal mitotic activity Nodular stage Spindle cells increase to form nodular tumour-like mass
- 60. TREATMENT FOR ORALLESIONS Small and localised lesions Wide surgical excision Low dose irradiation and intralesional chemotherapy Sclerosing agents For larger and multifocal lesions: systemic chemotherapy
- 61. MULTIPLE MYELOMA Malignancyof plasma cell origin Plasma cells are a subset of B-cells which are the producers of humoral immunity factors called antibodies It is the most common primary neoplasm of the skeletal system If metastatic lesions are excluded, multiple myeloma accounts for 50% of all malignancies that involve the bone It constitutes 1% of all malignancies and 10-15% of all hematologic malignancies
- 62. ETIOPATHOGENESIS Results frommutation of terminally differentiated B cells The abnormal plasma cells that constitute the tumour are monoclonal; hence all the cells produce same proteins These proteins are the immunoglobulins but they are not normal or functional PREDISPOSING FACTORS (suggested but not proved…) Radiation exposure Occupational exposure: chemical, metallurgical, rubber plant, pulp and paper workers and leather tanners Chemical exposure: benzene, formaldehyde, hair dyes,
- 63. CLINICAL FEATURES Age:60-70 years; rare before 40 years Gender: male predilection Race: blacks are more commonly affected than whites with a ratio of 2:1 It is the most common hematological malignancy among black persons in The United States Location: Diffuse disease of bone marrow Predominant sites of involvement are within the axial skeleton and include vertebral column, ribs, skull, pelvis and femur bone
- 64. CLINICAL PRESENTATION Bonepain Pathological fractures Anaemia Azotaemia Hypercalcemia Recurrent infections Fatigue Petechial haemorrhages of skin Fever Metastatic calcifications of soft tissues Extension of lesions to lymph nodes, skin and viscera The underlying pathology of multiple myeloma is expansion of a single line of plasma cells that replace the normal bone marrow and produce monoclonal immunoglobulins Renal failure and amyloid depositions can be seen as a result of increased immunoglobulins
- 65. RADIOGRAPHIC FEATURES Multiplewell-defined punched out radiolucencies Especially evident on the skull radiograph Jaws are involved in 30% LABORATORY FINDINGS Presence of Bence Jones protein in the urine
- 67. ORAL MANIFESTATIONS Mandibleis involved more frequently than maxilla Furthermore, the ramus, angle and molar region of the mandible are most frequently involved Signs and symptoms of jaw involvement include: Pain and swelling Expansion of the jaw Numbness and mobility of the teeth EXTRASKELETAL Amyloid deposits can be seen in the tongue Lesions can occur in gingiva and resemble gingival enlargements or epulis
- 69. HISTOPATHOLOGY Composed ofsheets of closely packed cells resembling plasma cells They are round or ovoid cells with eccentrically placed nuclei The nucleus exhibits chromatin clumping in a “cartwheel” pattern; perinuclear halo is seen Russell bodies are common
- 74. TREATMENT AND PROGNOSIS Chemotherapy to reduce the number of malignant plasma cells Management of specific complications Only 5-10% of patients live longer than 10 years Infections, anaemia and kidney failure are the most common causes of death
- 75. PLASMACYTOMA Solitary massof neoplastic monoclonal plasma cells in either bone marrow or soft tissue Classified into: Solitary bone plasmacytoma Extramedullary plasmacytoma Represents the least aggressive part of a spectrum of multiple myeloma and may ultimately give rise to multiple myeloma
- 76. PLASMACYTOMA vs MULTIPLE MYELOMA Mean age is 55 years; 10 years younger than multiple myeloma Radiologically presents as well-defined radiolucency with no sclerotic borders or may also appear as ragged radiolucency similar to multiple myeloma Amount of abnormal protein produced is much less than that of multiple myeloma No evidence of plasma cell infiltration should be seen by random bone marrow biopsy Patient should not show signs of anemia, hypercalcemia or renal failure
- 77. EXTRAMEDULLARY PLASMACYTOMA 90%of tumours develop at head and neck area without primary bone involvement Most common sites are paranasal sinuses, pharynx, nasal cavity and oral cavity Intraorally gingiva, palate, floor of the mouth and tongue are most commonly involved Lesions occur as sessile or polypoid reddish masses, which become lobulated as they grow but donot ulcerate
- 78. TREATMENT AND PROGNOSIS Radiation therapy Most will eventually develop into multiple myeloma Extramedullary plasmacytoma has much better prognosis with only 30% of lesions progressing to multiple myeloma
- 79.
- 80. CENTRAL GIANT CELLGRANULOMA Uncommon, benign and proliferative lesion Jaffe first introduce the term “CENTRAL GIANT CELL REPARATIVE GRANULOMA” to distinguish this lesion from giant cell tumour of long bones Since reparative response was quite rare and most of the lesions were found to be destructive the word “reparative” was omitted
- 81. CLINICAL FEATURES Age:most of the cases occur before 30 years Gender: more common in females; M:F = 1:2 Location: mandible is affected more often (70% occur in mandible) Lesions are more common in the anterior portions of the jaws Mandibular lesions frequently cross the midline
- 82. CLINICAL PRESENTATION Asymptomatic Noted during routine radiographic examination Sometimes painless expansion of the affected bone may be the presenting complaint Sometimes may be associated with Pain and paresthesia expansion of cortex, perforation of the cortical bone ulceration of the mucosal surface by underlying lesion mobility, displacement and root resorption of the associated teeth
- 83. RADIOGRAPHIC FEATURES Appearsas radiolucent defect which may be unilocular or multilocular The defect is usually well delineated, but margins are non- corticated
- 84. CLASSIFICATION Based onthe clinical and radiographic features, central giant cell granuloma of jaws can be classified into: NON-AGGRESSIVE LESIONS Few or no symptoms Slow growth No cortical perforation or root resorption AGGRESSIVE LESIONS Pain Rapid growth Show cortical perforation and root resorption Have marked tendency for recurrence
- 85. HISTOPATHOLOGY Loose fibrillarconnective tissue stroma with interspersed proliferating fibroblasts, small capillaries and giant cells The connective tissue may sometimes be quite cellular (corresponds to clinically aggressive lesions) Collagen fibres: arranged in the form of whorls Mesenchymal cells may be ovoid to spindle shaped and interspersed with round monocyte-macrophages Giant cells: multinucleated with variable size; contain few or several dozen nuclei Numerous foci of extravasated RBCs and associated hemosiderin pigment can be seen Foci of new trabeculae of osteoid or bone may be seen at the periphery of the lesion
- 87. TREATMENT AND PROGNOSIS Curettage or surgical excision X-RAY RADIATION THERAPY IS CONTRAINDICATED Recurrence is rare
- 88. PERIPHERAL GIANT CELLGRANULOMA Also called peripheral giant cell epulis or peripheral giant cell reparative granuloma Common tumour of the oral cavity ETIOLOGY Unknown Probable causes: Local irritation due to dental plaque or calculus, periodontal disease, poor dental restorations, ill-fitting dental appliances or dental extractions
- 89. CLINICAL FEATURES Age:first to sixth decades; mean age: 31-42 years Gender: 60% of cases are seen in females Location: occur exclusively on gingiva or edentulous alveolar ridge May develop in either anterior or posterior regions Mandibular tissues are most commonly affected
- 90. CLINICAL PRESENTATION Presentsas red or red-blue nodular mass Smaller than 2cms in diameter, large ones are occasionally seen Sessile or pedunculated and may or may not be ulcerated Ulceration is less common in edentulous conditions In edentulous patients, lesion may be arising from the crest of the ridge or from tissue covering the slope of the ridge RADIOGRAPHICALLY, the lesion may sometimes produce a “cupping” resorption of the underlying alveolar bone
- 92. HISTOPATHOLOGY Non-encapsulated Composedof delicate fibrillar and reticular stroma containing large number of ovoid or spindle shaped connective tissue cells and multinucleated giant cells Giant cells may contain few or several dozens of nuclei Some may have large vesicular nuclei and others demonstrate small pyknotic nuclei Numerous capillaries are found around the periphery of the lesion Foci of hemorrhage and hemosiderin pigment are seen Spicules of osteoid or dystrophic calcification may be seen
- 94. TREATMENT AND PROGNOSIS Conservative surgical excision Prognosis: good 10-15% is the recurrence rate which can be prevented by complete removal of the lesion rather than surgical excision
- 95. ANEURYSMAL BONE CYST Intraosseous accumulation of variable-sized, blood filled spaces surrounded by cellular fibrous connective tissue Seperated as a distinct entity in 1942 by Jaffe and Lichenstein
- 96. ETIOPATHOGENESIS Aneurysmal bonecyst may arise as a primary lesion or as a result of disrupted vascular dynamics in a pre-existing intrabony lesion Various theories have been proposed: Lichenstien: persistent local alteration in hemodynamics Increased venous pressure development of dilated and engorged vascular bed in the transformed bone area
- 97. ETIOPATHOGENESIS Attempt atrepair of a hematoma: a hematoma that maintains a circulatory connection with the damaged vessel would produce a slow flow of blood through the lesion As a secondary lesion: a primary lesion of the bone initiates an osseous, arteriovenous fistula and thereby creates, via its hemodynamic forces, this secondary reactive lesion of the bone
- 98. CLINICAL FEATURES Age:lesion of young persons; predominant under 20yrs of age Gender: no gender predilection Location: most commonly long bones and vertebral column Gnathic ABCs are uncommon; 2% reported in the jaws Mandibular predominance: mostly in the posterior segments
- 99. CLINICAL PRESENTATION Commonpresentation:Swelling that has usually developed rapidly Pain is often reported Parasthesia and compressibility: rare Malocclusion, mobility, migration or resorption of involved teeth may be present Maxillary lesions bulge into the sinus and can cause nasal obstruction, nasal bleeding, proptosis and diplopia
- 100. RADIOGRAPHIC FEATURES Unilocularor multilocular radiolucency Marked cortical expansion and thinning Ballooning or blow-out distension of the contour of the affected bone Small radiopaque foci may be seen within the radiolucency: trabeculae of reactive bone
- 105. HISTOPATHOLOGY Gross: “blood-soakedin sponge” appearance Microscopically Fibrous connective tissue stroma interspersed with cavernous or sinusoidal blood-filled spaces Spaces are filled with unclotted blood; blood filled spaces are not lined with endothelium Surrounding connective tissue contains multinucleated giant cells, trabeculae of osteoid and woven bone Varying amounts of hemosiderin pigment may be seen Approximately 20% of cases are associated with another pathosis like giant cell granuloma or other fibro-osseous lesions
- 106. TREATMENT AND PROGNOSIS Curettage or enucleation Recurrence is rare in the oral cavity
Editor's Notes
- #4 Sarcomas as a group differ from malignant epithelial neoplasms by their typical occurrence in relatively younger persons and their greater tendency to metastasize through the blood stream rather than through lymphatics, thereby producing more widespread foci of secondary tumour growth
- #66 Unusual protein which coagulates when the urine is heated to 40-60˚C and then disappears when the urine is boiled; it reappears when the urine is cooled