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Sarcomas clinical and radiographic features /prosthodontic courses


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Sarcomas clinical and radiographic features /prosthodontic courses

  2. 2. HISTORY • cancer is found among fossilized bone tumors, human mummies in ancient Egypt, and ancient manuscripts. • Growths suggestive of the bone cancer called osteosarcoma have been seen in mummies. • Oldest description of cancer dates back to about 3000 BC in egyptian Papyrus. writing says about the • disease, “There is no treatment.”
  3. 3. • Origin of the word cancer is credited to the Greek physician Hippocrates (460-370 BC), “Father of Medicine.” • He used the terms carcinos - non-ulcer forming • carcinoma - ulcer-forming tumors.( refers to crab in greek) • Roman physician, Celsus (28-50 BC), later translated the Greek term into cancer, the Latin word for crab • Galen (130-200 AD), Roman physician, used the word oncos (Greek for swelling) to describe tumors
  4. 4. • Hyperplasias may produce tissue masses referred to as tumors • Neoplasm or tumor is “ a mass of tissue formed as a result of abnormal, excessive, uncoordinated, autonomous, purposeless proliferation of cells”.
  5. 5. Benign Malignant Carcinoma SarcomaHamaratoma Choriostoma Teratoma
  6. 6. BENIGN NEOPLASMS • Dysmorphic proliferations of tissues • They have the capacity for continuous autonomous growth. • They donot elaborate the enzymes and growth factors necessary for metastasis
  7. 7. Hamaratoma • Dysmorphic proliferation of tissue that is native to the area • It does not have the capacity for continuous growth but merely parallels that of the host • They cease grow at some point in their course and they do not infiltrate into surrounding tissues. • E.g: hemangioma , pigmented nevi, odontoma, ameloblastic fibro odontoma.
  8. 8. Choriostoma • Dysmorphic proliferations of tissue that are not native to the site. They have a limited proliferation. • Heterotopic GI cyst, may be found in the tongue or floor of the mouth contains GI glandular structures; • Bone or cartilage in the tongue • Development of thyroid tissue in the posterior tongue • Ectopic sebaceous glands known as Fordyce granules • salivary gland tissue within lymph
  9. 9. TERATOMA • Neoplasias that arise from multiple germ layers • Produce tissues that are foreign to the part in which they develop. • They are distinct from other neoplasias that may also show tissue diversity
  10. 10. • Carcinoma: malignant tumors of epithelial origin • Sarcoma: (sarcos- fleshy) Malignant tumors of mesenchymal origin.
  11. 11. Grading of sarcomas • FNCLCC( FEDERATION NATIONAL DE CENTRES DE LUTTE CONTRE LE CANCER) • Based on tumor differentiation • Mitosis count • Tumor necrosis
  12. 12. TUMOR DIFFERENTION • Score 1 : sa closely resembling normal adult mesenchymal tisssue ( well differentiated) • Score 2 : sa for which hp typing is certain • Score 3 : embryonal & undifferentiated
  13. 13. MITOSIS COUNT • Score 1 : 0-9/10(HPF) • Score 2 : 10-19 • Score 3 : >/= 20 TUMOR NECROSIS • Score 0 : no necrosis • Score 1 : < 50% tumor necrosis • Score 2 : > 50% tumor necrosis
  14. 14. HISTOLOGIC GRADE • Grade 1 : total score 2/3 • Grade 2 : total score 4/5 • Grade 3 : total score 6/7/8
  15. 15. American Joint Committee on Cancer (AJCC) and International Union Against Cancer (UICC) staging system for soft tissue sarcomas • Primary tumor • Tx - Primary tumor cannot be assessed • T0 - No evidence of primary tumor • T1 - Tumor less than 5 cm in greatest dimension (T1a, superficial; T1b, deep) • T2 - Tumor greater than 5 cm in greatest dimension (T2a, superficial; T2b, deep)
  16. 16. • Regional lymph nodes – Nx - Lymph nodes cannot be assessed – N0 - No lymph node invovemnt – N1 - Lymph nodes involvement present • Distant metastases – Mx - Distant metastases cannot be assessed – M0 - No distant metastases – M1 - Distant metastases present
  17. 17. Histopathologic grade • Gx - Grade cannot be assessed • G1 - Well-differentiated • G2 - Moderately differentiated • G3 - Poorly differentiated • G4 – Undifferentiated
  18. 18. • IA (G1-2, T1a-b, N0, M0) - Low-grade, small, and superficial or deep tumor • IB (G1-2, T2a, N0, M0) - Low-grade, large, and superficial tumor • IIA (G1-2, T2b, N0, M0) - Low-grade, large, and deep tumor • IIB (G3-4, T1a-b, N0, M0) - High-grade, small, and superficial or deep tumor
  19. 19. • IIC (G3-4, T2a, N0, M0) - High-grade, large, and superficial tumor • III (G3-4, T2b, N0, M0) - High-grade, large, and deep tumor • IV (any G, any T, N1, M0) - Any metastasis
  20. 20.
  21. 21. CLASSIFICATION OF SOFT TISSUE SARCOMAS Sharon W Weiss, John R. Goldblum Enzinger & Weiss Soft Tissue Tumors 5th Edition Elsevier 2008
  23. 23. • SMOOTH MUSCLE TUMORS Leiomyosarcoma • Skeletal Muscle Tumors Embryonal Rhabdomyosarcoma Usual , Botryoid, Spindle Cell Alveolar Rhabdomyosarcoma Pleomorphic Rhabdomyosarcoma Sclerosing Rhabdomyosarcoma Rhabdomyosarcoma With Ganglionic Cells
  25. 25. • PRIMITIVE NEUROECTODERMAL TUMORS • Ewings Sarcoma • NERVE SHEATH TUMORS • Neurofibrosarcoma/ schwannoma • EXTRASKELETAL OSSEOUS & CARTILAGINOUS TUMORS • Osteosarcoma • Chondrosarcoma
  27. 27. FIBROSARCOMA • Malignant tumor of fibroblasts • Most common soft tissue sarcoma • Most common in extremities • Only 10% occurs in h & n. m > f • May arise from pre-exisisting lesions such as fibrous dysplasia, chronic osteomyelitis, bone infarcts, pagets disease.
  28. 28. • Occur anywhere in h & n, & at any age • Most common in children, young adults • Can also occur in bone as primary or secondary forms • Primary occurs centrally within medullary canal, peripheral arise from periosteum. • Produce variable amount of collagen • Secondary arise from pre-exsisting lesion or after radiotherapy to bone or soft tissue • More aggressive tumor with poorer prognosis
  29. 29. • Painless fibrous, fleshy masses that are destructive of bone • Peripheral lesions may invade local soft tissues causing bulky clinically obvious lesion • If involve course of nerve…neural abnormalities • Involvement of tmj causes trismus • Cause mobility of teeth if they are located in alveolar bone
  30. 30. • Hp: well differntiated fibrosarcomas consists of spindle cels that classically form a “herring bone pattern” • Poorly differentiated tumors, cells are less organised & may appear rounder/ovoid • Produce less collagen when compared to well differentiated.
  32. 32. • Radiology: • Mostly in mandible • Premolar-molar area is common • Poorly demarcated, non corticated • If soft tissue lesions occur adjacent to bone they may cause saucer like depression similar to scc • Sclerosis may occur in adjacent normal bone
  33. 33. • Internal structure: little • Entirely radiolucent • If lesion is not aggressive either residual jaw bone/ reactive osseous bone may be present • Surrounding strctures: • Alveolar process, inferior border, nv canals are lost • Floor of sinus, wall of maxilla can be destroyed • Loss of supporting bone around tooth.
  34. 34. Dd : • Fibrous dysplasia • Osteosarcoma • Fibrous histiocytoma • Neurofibromas , • Malignant peripheral nerve sheath tumors. “Each one must be distinguished by its unique histopathologic features.”
  35. 35. Fibrosarcoma causing displacement of tooth, tongue
  36. 36. MALIGNANT FIBROUS HISTIOCYTOMA • Sarcoma with both fibroblastic & histiocytic features • Most common soft tissue sarcoma of late adult life. • Many were previously diagnosed as fibrosarcomas, malignant peripheral nerve sheath tumors, and pleomorphic rhabdomyosarcomas. • Its overlap with fibrosarcomas is because both arise from a similar precursor cell within a maturation sequence beginning with the pluripotential mesenchymal stem cell and ending with the mature fibrocyte
  37. 37. • Men> women • Occurs mostly in extremities • Occur in soft tissue or bone • Rare in maxillofacial region • Common complaint is expanding mass that may/ maynot be painful
  38. 38. • Hp : • Most lesional cells are spindled fibroblasts like cells • Arranged in storiform pattern • Some areas contain histiocyte like cells • Which have eosinophilic cytoplasm/ pale foamy cytoplasm • Sub classified into • Myxoid, pleomorphic-storiform, angiomatoid( aneurysmal), giant cell
  39. 39. Spindle cells may be arranged randomly. But most commonly usually present as streaming in interlacing fascicles from a central nidus and intersecting with cells from adjacent aggregates….storiform/ criss crossing
  40. 40. Dd: • Malignant fibrous histiocytomas must be distinguished from the benign fibrous histiocytoma( rl expansion of jaw) • fibrosarcomas, malignant peripheral nerve sheath tumors & rhabdomyosarcomas • Clinical presentation of all these lesions is similar • Distinction is mainly histopathologic, most true fibrosarcomas and rhabdomyosarcomas occur at younger ages.
  41. 41. LIPOSARCOMA • Liposarcomas are very rare tumors in the oral and maxillofacial area( if occurs- neck) • Thigh, retroperitoneum • Present as a slow‐growing mass from a deep origin • Liposarcomas originate from primitive mesenchymal cells rather than from mature fat cells
  42. 42. • liposarcomas are rare in subcutaneous areas; they most commonly arise in intermuscular fascial planes, which contain residual mesenchymal stem cells. • Liposarcomas do not develop from pre‐existing benign lipomas. All are malignant from their inception.
  43. 43. RHABDOMYOSARCOMA • Rhabdomyosarcomas are malignant tumors of primitive mesenchymal cells that undergo partial rhabdomyoblast differentiation • Unlike other sts which occur in adults • 44% occurs in omf regions • the orbit is the most common location, followed by the nasal cavity, mouth, sinuses, cheek, and neck.
  44. 44. • Three basic histologic types: embryonal, alveolar, and pleomorphic • embryonal- best prognosis • most common type found in omf • Males > f (1.5:1). • Tumors have a peak incidence at age 4 years and another at age 17 years
  45. 45. • The tumor presents as a rapidly growing, fleshy mass, which readily invades and destroys bone. • Rhabdomyosarcomas are not radiographically distinctive but will show primarily the anticipated soft tissue mass and bony destruction
  46. 46. • Dd: • A tumor with rapid growth and destructiveness in a child or young adult should suggest a rhabdomyosarcoma. • Other rapidly destructive lesions in this age group are Ewing sarcoma, neuroblastoma & acute Langerhans cell • histiocytosis, and less commonly, a malignant peripheral T‐cell lymphoma. All of these are also known to invade bone in a destructive manner.
  47. 47. Leiomyosarcoma • Leiomyosarcomas are a relatively uncommon type of sarcoma • 7% of all soft tissue sarcomas. • Most occur in the retroperitoneum and within the abdomen; • others are associated with large blood vessels, such as the inferior vena cava and pulmonary artery, and are often referred to as leiomyosarcomas of vascular origin. • Intraoral leiomyosarcomas are extremely rare because of the paucity of smooth muscle in oral tissues
  48. 48. ANGIOSARCOMA • Angiosarcomas are malignant tumors that arise from either vascular or lymphatic endothelium • malignant hemangioendotheliomas/lymphangiosarcomas • Rarest , accounting for 1% of h& n sts • Can occur at any age, more common in elderly • Males> females • Skin of maxillofacial area, scalp • Io lesions lips, palate, gingiva, tongue, centrally with in maxilla, mandible.
  49. 49. • 10% of angiosarcomas develop in chronic lymphedematous tissues • 10% are believed to be late effects of radiotherapy for previous malignancies of other types.
  50. 50. • Induration and ulceration of angiosarcomas lead to a suspicion of malignancy. • rarity makes them an unusual consideration on a differential list. • More common ulcerating malignant lesions that can occur on facial and forehead skin as well as scalp • primarily basal cell carcinoma, melanoma, skin squamous cell carcinoma, and eccrine tumors of sweat gland origin. • If the tumor is nodular and bulky, a so‐called turban tumor/ cylindroma of the scalp, is another possibility.
  51. 51. • Begin as a flat, ecchymotic‐looking area dark red or bluish red with a firm, indurated edge. • associated with facial edema and are presumably of lymphatic endothelial origin. • As the lesions mature, they become nodular and fleshy and will ulcerate. • Most are painless, but secondary infection of ulcerated lesions may produce pain. • They are not vascular lesions, per se, and therefore do not pose a bleeding risk. Instead, they are of vascular cellular origin
  52. 52. • Hp: Angiosarcomas are infiltrating tumors that usually form irregular vascular channels that often intercommunicate to form a network. • The endothelial cells lining the channels are plump and hyperchromatic and may proliferate to form papillary projections. • Nuclear irregularities • atypical mitoses are usually present
  53. 53. Hp variants: • Epithelial hemangioendothelioma • Spindle cell hemangioendothelioma • Kaposiform hemangioendothelioma • Polymorphous hemangioendothelioma
  54. 54. KAPOSI SARCOMA • Angioreticuloendothelioma • Multiple idiopathic hemorrhagic sarcoma of kaposi • Multicentric proliferation of vascular & spindle components. • Etiology is unknown • Co factor model : host factor- immunosuppression • Infectious agent – human herpes virus 8/KSHV • Environmental: overall geographic distribution
  55. 55. • Classic: • Occurs in late adult life. 70 to 90% occurs in males • It forms violaceous macules, papules, and nodules often symmetrically on the skin of the lower extremities. • It is chronic and slowly progresses through an early patch stage and into plaque and nodular stages • The lesions increase slowly in size and number, spreading proximally and emerging into plaques or vascular nodules. • Oral involvement is unusual. If occurs palatal mucosa/gingiva.
  56. 56. • Endemic/ lymphadenopathic/ african: • Present as localised/ generalised enlargement of lymphnodes • Including cervical • Mainly visceral involvement • Less skin & mucous membrane involvement • Salivary glands are affected
  57. 57. • Transplantation associated: • Seen in renal transplant patients • Manifest 1 or 2yrs after transplantation • Due to loss of cellular immunity • Sarcomatous involvement of skin, internal organs • Oral involvement is rare.
  58. 58. Generalised involvement of alveolar mucosa in a renal transplant patient
  59. 59. • Aids related: • 40% homosexual aids pts develop disease as early sign • Lesions occur in many cutaneous locations, along lines of cleavage • Tip of nose • Oral lesions occur on any mucosal surface • Commonly gingival & palatal mucosa
  60. 60. • HP: • PATCH STAGE: In the early patch stage, changes may be extremely subtle and show only a proliferation of both small and dilated vessels. • A mild infiltrate of lymphocytes and plasma cells may be seen at the periphery. • PLAQUE: Vascular proliferation continues in the plaque stage, and foci of spindle cells, typically related to the vascular component, develop
  61. 61. • NODULAR : • nodular stage, the spindle cell component dominates, encroaching on the previously obvious vascular spaces • picture resembles fibrosarcoma • but Kaposi sarcoma will show the presence of slit‐like spaces between the spindle cells,which contain varying numbers of erythrocytes and some hemosiderin • hyaline globules are
  62. 62. Branching blood vessels Spindle cells KAPOSI SARCOMA
  63. 63. • DD : oral & skin lesions resemble bruising (ecchymosis) or a deeply located low‐grade mucoepidermoid carcinoma. • Papular and nodular lesions appear like hemangiomas, lymphangiomas & hemangiomas. • Even though it is uncommon, bacillary epithelioid angiomatosis may appear ks • On the alveolar ridge, they may resemble a pyogenic granuloma, or a peripheral giant cell proliferation
  64. 64. Hemangiopericytoma • Oral hpc is typically rapidly enlarged red/bluish mass which arises in all age groups. • Soft, rubbery, painless, well demarcatedcfrom surrounding mucosa • Sessile/pedunculated • Surface lobularity/ telengiectasis
  65. 65. • Hp: well circumscribed, greyish white • Less hemorrhagic than vascular tumors • Numerous branching vascular channels • Plump endothelial nuclei • Tightly packed oval & spindle cells • Branching vascular channels are in form of stag horn pattern.
  67. 67. SYNOVIAL SARCOMA • Un common form • Represents 5 to 10% of all soft tissue neoplasms • Occurs near large joints/bursae • Commonly involves extremities • Rare in h&n • Occurs in parapharyngeal/paravertebral areas • Causing dyspnoea, dysphagia, hoarsness, headache.
  68. 68. • It so named because of its resemblance to developing synovial tissue • Arise from pleuripotent mesenchymal stem cells near joint surface, tendon, tendon sheats, juxta articular membranes, fascial aponeurosis
  69. 69. • Radiograhic findings: • Plain radiographs aid in diagnosis • Produce spotty calcification ( snow
  70. 70. WHO CLASSIFICATION OF BONE SARCOMAS Louis B Harrison, Roy B Sessions, Waun Ki Hong Head & Neck Cancer- A Multidisciplinary Approach 2nd Edition Lippincott Williams & Wilkins Usa 1998
  74. 74. Osteosarcoma • Malignancy of mesenchymal cells that have ability to produce osteoid or immature bone. • Most common primary tumor to orginate in bone after multiple myeloma • arises from undifferentiated cells &
  75. 75. Etiology • UNKNOWN • Risk factors are radiation exposure • Genetic predisposition( 13q14) causing inactivation of RB gene leding to dev of retinoblastoma, os • Bone dysplasias fibrous dysplasia, pagets disease increase risk • Li-fraumeni syndrome ( germline t53 mutation) predispose to os
  76. 76. • Can occur in any bone • Common in long bones • Extragnathic os shows bimodal age distribution • 10-20yrs( period of active growth) & >50yrs • Site: distal femoral & proximal tibial metaphyses. • Older pts axial skeleton & flat bones are involved
  77. 77. Shoulder Pelvic Distal femur and proximal tibia Jaw
  78. 78. • Os of jaws 6 to 8% of all os. • Occurs mostly in 3rd & 4th decade of life • Slight male predominance. • Maxilla < mandible • Mandible: posterior body, hori.ramus > ascending ramus • Maxilla : inferior portion( alveolar ridge, sinus floor,palate) > superior( zygoma, orbital rim) • Os of jaws are better differentited , better prognosis, less metastasis, than extragnathic os
  79. 79. Clinical features: • Pain, swelling of involved area – facial deformity • Pt c/o sprain,arthritis or so called growing pain • Loosening of teeth • Paresthesia • Nasal obstruction in max. tumors • Epistaxis, hemorrhage • h/o recent tooth extraction with a nodular/polypoid some what reddish granuloma like growth growing from tooth socket.
  80. 80. • As tumor grows, eroding cortical plates expansion is very firm because of dense fibrous tumor tissue produced. • Initially swelling is smoothly contoured, covered with normal appearing mucosa • When expansion becomes chronically traumatised mucositis develops on surface • Surface ulcerates and grayish white necrotic surface results which can be removed with tongue blade.
  81. 81. juxta cortical os • Paraosteal: lobulated nodule attached to cortex by a stalk • No elevation of periosteum/its reaction • Hp: mass contain spindle cell fibroblast like proliferation • Well dev trabeculae of bone • They coalesce and form large solid mass of bone
  82. 82. • Periosteal : • Sessile lesion • Arise with in cortex • Elevates overlying periosteum • Provokes production peripheral periosteal bone • Leading edge of tumor mass perforates periosteum extends into soft tissue
  83. 83. Histopathology • Cells of tumor may producoe osteoid, chondriod, or fibrous connective tissue • Vary in shape uniform round/ spindle shaped to highly pleomorphic • Depending on relative amounts of osteoid, cartilage/collagen os is div into osteoblastic( 50%), chondroblastic, fibroblastic
  84. 84. • Chondroblastic os are more common • Composed entirely of malignant cartilage growing lobules with only small foci of direct osteoid production by tumor cells • Other less common histologic variants are • Malignant histiocytoma like • Smallcell, epitheloid, telangiectatic, giant cell rich • Low grade well differentiated os – minimal cellular atypia , abundant bone formation. • May be misdiagnosed as fibrous dysplasia/ fibro osseous lesion
  85. 85.
  86. 86. Telangiectatic • Seen in adolescence & early adulthood • rare variant of central highgrade • Gross examination reveals a blood-filled cavity • Hemorrhagic & necrotic areas are seen in tumor • Microscopically dilated vascular channels lined with multinucleated giant cells and an anaplastic sarcomatous stroma with evident bone formation
  87. 87. • Radiographically : large lytic defect, • usually expansile and accompanied by an extensive soft tissue component. • Mri- fluid levels similar to those seen in an aneurysmal bone cyst • Occasionally, this lesion may be very difficult to distinguish from an aneurysmal bone cyst.
  88. 88. Radiology Internal structure: • Entirely rl/ osteolytic • Mixed rl-ro • Quite ro/ osteoblastic • Osseous structure may be granular/sclerotic apparing bone • In form of cotton balls, wisps, honey coomb internal structure with adjacent destruction of pre-exisisting osseous architecture. • What ever may be presentation, normal trabecular pattern is lost.
  89. 89. Osteolytic: • Unicentric, ill defined borders • Moth eaten appearance • Perforation & expansion of cortical margins into sub periosteal bone
  90. 90. Mixed • Ragged, ill defined borders • Ro due to excess bone formation intermingled with resorption leading to rl areas • In some sequestra appear , appear as well defined ro • Rl destruction may be in form of strands…honey comb appearance • Sun burst appearance • Cumulus cloud • Codmans triangle are seen
  91. 91. Periphery and shape • ragged, Ill defined border • When viewed against normal bone Lesion is usually rl • No peripheral sclerosis / encapsulation • If it involves periosteum: • Typical sun ray spicules/hair on end trabeculae are seen • This occurs when periosteum is displaced, partially destroyed & disorganised.
  92. 92. Sun ray appearance
  93. 93. Cortical bone expansion and destruction…
  94. 94. Garringtons sign
  95. 95.
  96. 96. Effects on surrounding tissues • Widening of pdl space occurs ( garrington sign) • Band like widening along complete length of pdl space unilaterally/bilaterally • Also seen in other malignancies, osteoblastoma, • Pts undergoing ortodontic treatment • Unilateral bone resorption seen in periodontal disease.
  97. 97. • Antral/nasal wall cortices are lost in maxillary lesions • Mand lesions destroy cortex of neurovascular canal, nv canal is symmetically widened & enlarged • Lamina dura of involved tooth is completely destroyed.
  98. 98. • Dd: • If internal structure is minimal fibrosarcoma/ osteoblastic metastatic ca • Osseous structure visible Chondrosarcoma (affects older age group, more often involves maxilla) • Spiculated periosteal reaction Prostate/breast metastasis • Benign tumors & conditions like ossifying fibroma, fo lesioms mimic os radiographically.
  99. 99. • Ossifying subperiosteal hematoma( a h/o recent trauma to the bone) • Peripheral fibroma with calcification( slow, benign growth) • Chronic osteomyelitis ( but infection is absent in os) • An important clinical differential feature is neurosensory loss. • rare osteomyelitis or neural loss from a previous biopsy or surgery, only malignancies can produce objective paresthesias.
  100. 100. • radiographs or CT scans at right angles to the cortex should show extracortical bone and a destroyed cortex. • Fibrous dysplasia and ossifying fibroma will not have extracortical bone. The • extracortical bone seen in osteomyelitis with proliferative periostitis will be associated with an intact • cortex. • Even when other osteomyelitides produce extracortical bone, it is parallel to the cortex rather • than at right angles as is seen in osteosarcoma
  101. 101. Chondrosarcoma • Malignant tumor characterised by formation of cartilage by tumor cells • Comprise 10% of all tumors of skeleton • Rare in jaws( 3% of all cs) • Extragnathic it is primarily neoplasm of adulthood with peak prevalance in 6th, 7th decades of life • Tumors arising in younger age group is uncommon • No sex or race predilection • Site: ileum, femur, humerus
  102. 102. • In h& n : cs common in maxilla ( anterior areas where cartilage tissue is present. • mandible: ccoronoid process, condylar h& n, symphyseal region.less common in body, ramus, nasal septum, pns • Chondrosarcomas most often develop in osseous locations • But 1/3rd originate in laryngeal cartilage/soft tissue • Cs arise directly from cartilage or may occur within benign cartilagenous tumors….secondary cs
  103. 103. • A painless mass, swelling • Associated with separation or loosening of teeth • Pain is unusual • Maxillary tumors may cause nasal obstruction, congestion, epistaxis, photophobia, or visual loss. • If cs occur near tmj trismus, abnormal joint function occurs. • When metastasis occurs lung is organ most commonly involved.
  104. 104. • Hp: composed of cartilage with varying degrees of maturation & cellularity • Lobular pattern with lobules separated by thin fibrous connective tissue • Central area demonstrates lobules with greatest degree of maturity • Peripheral area contains immature cartilage & mesenchymal tissue consisting of round/spindle cells • Calcification/ossification may occur in chondroid matrix
  105. 105. • Some times at periphery of lobules of high grade cs, a few fs like spindling tumor cells are present • Osseous trabeculae when present are seen at periphery of lobules & appears to be rimmed by osteoblasts. • When malignant cells produce osteoid lacework or trabeculae even in small foci, tumor is graded as os
  106. 106. • Four histological subtypes: • Clear cell • Dedifferentiated • Myxoid • Mesenchymal • Occur centrally within bone or less commonly in soft tissue.
  107. 107. Variants : • Clear cell cs: cells with abundant clear cytoplasm • Dedifferentiated cs: high grade malignancy that show admixture of well differentiated cs & malignnat mesenchymal tumor resembling fibrosarcoma. • Myxoid cs: soft tissue tumor. io variant is seen, characterised by clear, vacuolated, eosinophilic cytoplasm with a background of mucoid
  108. 108. • Mesenchymal cs: uncommon tumor of soft tissue & bone • Shows biphasic hp • More common in soft tissue than bone • Chondroid tissue is well differentiated, degree of cellularity & atypia varies from benign chondroma to well differentiated chindrosarcoma • If cartilagenous foci is sparse, tumor may be misdiagnosed as hemangiopericytoma
  109. 109. • Radiographically….internal structure: • radiolucent area with poorly defined borders • Rl area contains scattered ro foci caused by ossification of cartilage matrix • Mixed rl- ro appearance • Some times it appears as moth eaten bone alternating with islands of residual bone unaffected by tumor
  110. 110. • Central ro structure appear as flocculent implying snow like features • Diffuse calcification may be superimposed on a bony background that resembles granular/ground glass appearing normal bone • Examination of this flocculence may reveal central rl nidus which is probably cartilage surrounded by calcification.
  111. 111. • Periphery : • Generally roun/ovoid/lobulated • Well defined corticated borders • Occasionally peripheral periosteal reaction in form of sunray, hair on end appearance is seen • Uncommonly aggressive lesions ill defined, invasive , non corticated borders seen
  112. 112. Effect on surrounding tissues: • Relatively slow growing so expands cortex, rather than rapidly destroying them • Mand cases, ian expanded still maintaining corical covering • Maxillary lesions push wall of sinus or nasal fossa & impinge on infratemporal fossa • Lesions of condyle cause expansion, remodelling of articular fossa & eminence.
  113. 113. • If lesion is in articular disk region, widening of joint space, remodelling of condylar neck occurs. • Erosion of articular fossa occurs • If lesion occurs near tooth, root resorption, widening of pdl space, tooth displacement may occur.
  114. 114. • Dd; • Os : typical calcifications of cs are absent • Fibrous dysplasia: periphery is well defined. ro portion of fibrous dysplasia is abnormal bone & not the calcifications • Because of their slow growth and especially their intact overlying mucosa, most cases will initially resemble a benign odontogenic tumor or a benign tumor of bone
  115. 115. • Some punctate radiopacities are identifiable, the lesion will resemble a • calcifying epithelial odontogenic tumor • an ossifying fibroma • an immature osteoblastoma • a cavernous hemangioma of bone. • The more obviously aggressive presentations with irregular radiolucencies and perhaps neurosensory loss would be consistent with an intraosseous carcinoma, an osteosarcoma, and a malignant fibrous histiocytoma
  116. 116. Ewings sarcoma • Notoriously aggressive and destructive malignancy of bone arising from marrow mesenchymal stem cells • Genetically and histologically distinctive small round cell sarcoma of bone • 85% to 90% cases, tumor cells show reciprocal translocation b/n chromosomes 11 & 22. (q24;q12) • Third most common osseous neoplasm than os, cs • It was first described by James Ewing in 1920 as a "diffuse endothelioma of bone.“
  117. 117. • In both the jaws and long bones,peak age of occurrence is in the teenage years (50%). Young men slightly > young women • mandible's posterior body, the angle and ramus regions are common. Cause bony expansion , mobile teeth, extensive destruction of bone and necrosis…fever occurs • present a picture similar to that of an osteomyelitis. • rapid growth rate. Metastasis is
  118. 118. • Hp : Ewing sarcomas are composed of densely packed, rather uniform cells with little intercellular stroma • The nuclei are rounded to oval with defined nuclear borders and a • finely granular chromatin pattern • The cytoplasm is indistinct and may be vacuolated. • The cells are two to three times the size of a lymphocyte. • Mitoses are infrequent. • Rapid growth, undergo considerable necrosis, sometimes resulting in a perivascular pattern of viable tumor cells
  119. 119. Tumor cells are arranged in broad sheets…filigree pattern in which infiltratin strands of tumor cells are separated by thin fibrovascular septae Well defined nuclei Indistinct cytoplasm
  120. 120. • Hp dd: includes other small round cell tumors, including • neuroblastoma,lymphoma, small cell osteosarcoma& embryonal rhabdomyosarcoma. • Ewing sarcoma will usually have intracytoplasmic glycogen granules demonstrated by periodic acid‐Schiff (PAS) and diastase staining. • neuroblastoma and embryonal rhabdomyosarcomas may also yield positive staining.( for glycogen)
  121. 121. Rf: • Internal structure: • Es is a destructive process with little unduction of bone. • It commences on internal aspect of bone & involves endosteal & periosteal surfaces later , usually entirely rl. • Periphery: rl poorly demarcated & never corticated. • Advancing end destroys bone in an uneven fashion. • May cause pathological fractures. • Extends into adjacent soft tissue.
  122. 122. Effect on surrounding tissues: • adjacent normal structures ian canal, lower border of mandible, alveolar cortical plates are destroyed. • It doesnot characteristically cause root resorption, it destroys supporting bone adjacent to tooth.
  123. 123. • Panoramic radiographs and a CT scan • an ill‐defined, irregular resorption of bone with focal areas of residual bone resembling sequestra. • Pathologic fractures are common, attesting to the degree of bone destruction. • Multilayered periosteal reaction that has been described as an "onion skin" appearance, • (Similar to proliferative periostitis in osteomyelitis) • such a radiographic appearance is almost never seen when Ewing sarcoma arises in the jaws.
  124. 124. • Ewing sarcoma in the jaws will produce a destructive radiolucency with resorbed tooth roots and displaced teeth. • On rare occasions, a Ewing sarcoma may produce a periosteal new bone formation perpendicular to the cortex and thereby create the "sun‐ray" appearance more frequently seen in osteosarcomas
  125. 125. • Dd : • Pain , fever, leukocytosis suggest a suppurative osteomyelitis. • Reinforced by radiographs showing destructive bone pattern , bone foci resembling a sequestrum, layered periosteal "onion skin“ appearance…resembling proliferative periostitis.
  126. 126. • Other aggressive malignancies that occur in this young age group include • Rhabdomyosarcoma , • Osteosarcoma, fibrosarcoma, and neuroblastoma • Eosinophilic granuloma of jaw is also a destructive process, but is associated with laminar periosteal reaction, where as in jaws ewings sarcoma doesnot produce.
  127. 127. Periosteal reactions
  128. 128. • periosteum is a membrane several cell layers thick that covers almost all of every bone. • only parts not covered by this membrane are the parts covered by cartilage. • Besides covering the bone and sharing some of its blood supply with the bone, • it also produces bone when it is stimulated appropriately. • Practically anything that breaks, tears, stretches, inflames, or even touches the periosteum.
  129. 129. •With slow-growing processes, the periosteum has plenty of time to respond to the process. •It can produce new bone just as fast as the lesion is growing. • solid, uninterrupted periosteal new bone along the margin of the affected bone.
  130. 130. •In case of rapid growing tumors, new bone is not formed at the rate of tumor. •An interrupted pattern is formed •This may result in a pattern of one or more concentric shells of new bone over the lesion. This pattern is sometimes called lamellated or "onion-skin" periosteal reaction.
  131. 131. •If the lesion grows rapidly but steadily, the periosteum will not have enough time to lay down even a thin shell of bone, •In such cases, the tiny fibers that connect the periosteum to the bone (Sharpey's fibers) become stretched out perpendicular to the bone. •When these fibers ossify, they produce a pattern sometimes called "sunburst" or "hair-on-end" periosteal reaction, depending of how much of the bone is involved by the
  132. 132. • Codmans triangle : • When a process is growing too fast, it penetrate through the cortex causing separation of the periosteum and formation of lamellated new bone. If the periosteum elevates to a significant degree, it can break forming an acute angle
  133. 133. • When this little bit of ossification is seen tangentially on a radiograph, it forms a small angle with the surface of the bone, but not a complete triangle..
  134. 134. • References 1. Robert E Marx, Diane Stern Oral And Maxillofacial Pathology: A Rationale For diagnosis & Treatment. 1st edition, Quintessence.2003. 2. Stuart C White , Michael J Pharoah , Textbook Of Oral Radiology Principles And Interpretation 3. Norman k wood paul w goaz. Dd of oral and maxilllofacial lesions. 5th edition elsevier 2007
  135. 135. • R Rajendran B Sivapathasundharam Shafer’s Textbook Of Oral Pathology. 5th Edition, Elsevier, 2008 • Brad W Neville Douglas D Damm Carl M Allen Jerry E Bouquot. Oral And Maxillofacial Pathology. 3rd Edition Elsevier, 2009
  136. 136.