Tumors of the head and neck

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Oral Pathology II
Forth Year

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Tumors of the head and neck

  1. 1. Tumors of the head & neck Odotogenic Tumor Originate from : Epithelium mesenchyme Both ODONTOGENIC TUMORS Epithelium Origin Tumor 1) Ameloblastoma :        Local growth in the maxillofacial area . Ability to produce marked deformity . Recurrence after conservative Tx . Locally aggressive behavior . Can start as malignant. Slight metastatic potential Originate within the mandible or maxilla from epithelium that is involved in the formation of the teeth ( enamel organ, reduced enamel epithelium, rest of malassez & serres ).  The stimulus for this neoplastic transformation is unknown .  Clinical feature : - any age , 50% are in 20-40 years old - No gender predilection . - Site: 80% mandible, 3/4 in the under ramus area,20% in maxilla. - Slow growth with expansion and dental changes . - X-ray: classic multilocular lytic lesion .  Histopathology : proliferating cord , island & srtands of odontogenic epithelium.  Ameloblastoma differentiation : ??  Tx : a- conventional ameloblastoma ( multilocular or infiltrative lesion ) : aggressive therapies such as en bloc , marginal or segmental resection ( partial ) mandiblectomy or maxillectomy may be required. b- Unicystic ameloblastom ( uniocular ) : conservative therapy such curettage . " follow up needed due to it's recurrence – may cause death – "
  2. 2.  Variant of ameloblastoma : a- ameloblastic carcinoma : cytologic malignant epithelium odontogenic tumors with ameloblastic differentiation . Pursue an aggressive clinic course. b- Peripheral ameloblastoma : arising from oral mucosa surface – 1% of ameloblastoma – middle ages adults – occur in gingival & alveolus – some cases reported basal cell carcinoma. c- Malignant ameloblastoma : atypical one that metastasizes commonly in lungs, lymph nodes & bones. Histological similar to itrabony ameloblastoma. 2) Adenomatoid odontogenic tumor :        Age : 5-30 years , mean 18 , 75% in Pts <24 Sex : 2:1 female predilection Site : Appear 80% in the anterior jaw 2/3 in the maxilla & 1/3 in the mandible. 15% in the posterior jaws. X-ray : circumscribed, uniocular lucency radiopaque flecks & 75% associated with impacted tooth. Histopathology : whorled epithelium duct like structure. Tx : nucleation. 3) Squamous odontogenic tumor :        Age : young adult age 20-40, average 37 Sex : no gender predilection . Site : any site of alveolus. X-ray : triangular lytic defect adjacent to tooth root with radiopaque border. Derived from rest of Malassez. Histopathlogy : - similar to ameloblastoma. - lack of columnar peripherally palisaded layer of epithelium cells. Tx : because it's invasive, it reoccur after conservative therapy. It need curettage or excision. 4) Calcifying epithelial odontogenic tumor :      Age : 2nd to 10th decades, average 40. Sex : no gender predilection . Site : mandible is affected twice the maxilla & common at molar-ramus area. Peripheral lesion usually in the anterior gingival. X-ray : associated with impacted tooth, uni or multiocular take honeycomb shape. Tx : enucleation or resection.
  3. 3.  Histopathology : - large polygonal epithelial cells, seen in sheets & islands, contain nuclei that show variation in size & shape. - epithelioid strandnestssheets. - amyloid and calcification - mitotic figure and clear cell variant are rare. 5 ) Clear Cell Odontogenic Tumor       Age : older adults mostly more than 50 years. Sex : possible female predilection. Site : posterior jaws maxilla & mandible. x-ray : lytic lesions with ill defined margin. Histopath : islands and nest of clear odontogenic epithelium in dense fibrous stroma. Complete resection, locally aggressive with metabolic potential & need aggressive therapy. Oral pathology "2" ,, 4th lecture by Dr.Louiy ,, 6/3/2011 ODONTOGENIC TUMORS Mesenchymal Origin Tumor 1) Odontogenic Myxoma - It mimic microscopically the dental pulp. - it's benign tumor. – it may be infiltrative & aggressive. – it may be recur. Clinical Features : - Age from 10 to 50 years. – no gender predilection. - anywhere in the jaw can be seen. - It's gelatinous & sticky so may remain some of it after treatment. X-ray : - Radiolucent lesion , well circumscribed or diffused.
  4. 4. - Multiocular and has a honeycomb pattern. - cortical expansion or perforation & root displacement can be seen. Histopath : - Myxomatous CT. - benign fibroblasts with collagen fibers in mucopolysaccharide matrix. - odontogenic rest are typically absent. Differential Diagnosis : central hemangioma ( honeycomb pattern ). Tx : surgical excision. 2) Central Odontogenic Fibroma ( within the bone ) - it's considered as the central counterpart to peripheral odontogenic fibroma. - appear in all age groups - appear at any location in the jaw. X-ray : multiocular radiolucent lesion with cortical expansion. Histopath : - simple type : mass of fibrous tissue containing epithelial rests. Tx : enucleation or excision ( compared to myxoma is conservative therapy ) 3) Cementoblastoma - it's rare benign neoplasm of cementoblast origin. - it occur mostly in 2nd & 3rd decades of life preferably in the mandible posterior lesion. - no gender predilection. - associated with the tooth root but the tooth remain vital. - cortical expansion. – low grade intermittent pain. X-ray : - this neoplasm is an opaque lesion that replaces the root of the tooth. - it's usually surrounded by a radiolucent ring representing the periodontal ligament space. Hostopath :
  5. 5. - dense mass of mineralized cementum like material. - intervening well vascularized soft tissue containing cementoblast. Differential Diagnosis : - odontoma, osteoblastoma, focal sclerosing osteomyletis & hepercementosis. Tx : removal with associated tooth + bone relief is required. 4) Periapical Cementossous Dysplasia ( PCOD ) - it represents a reactive or dysplastic process. - it appear to be unusual response of periapical bone and cementum to undermined local facrors. Clinical Features : - it occurs in the apex of vital tooth. -middle age black women ( 40 years ) represent the typical group of patients. - anterior mandible region & it's asymptomatic. - discovered during routine examination. X-ray : - appear first as periapical radiolucency ( similar to periapical granuloma ). - as a lesion develop, it develop into mixed or mottled pattern because of bone repair. - the final stage appear as a solid opaque mass. 5) Fluoride Cemento-Osseous Desplasia – FCOD - it appears to be an exuberant form of a PCOD. - there no clear cause. - occur bilaterally + asymptomatic except if there is complication of osteomylitis. X-ray : diffuse, radiopaque mass. Histopath : - mixture of benign fibrous tissue, bone & cementum. Differential diagnosis : - paget diseases, chronic diffuse sclerosing osteomylitis.
  6. 6. Tx : no treatment just follow up. ODONTOGENIC TUMOR ( Mixed Origin : Epithelium + mesenchyme ) 1) Odontoma - is composed from both epithelium & mesenchymal dental hard tissue ( enamel & dentin ). - compound odontoma : numerous miniature teeth. - complex compound : amorphous hard tissue. Clinical Feature : - it's lesion of children and young adults ( age : 2nd decades of life ) - maxilla > mandible. - anterior jaw ( mostly compound odontoma ) & posterior jaw ( mostly complex odontoma ). - signs that are suggested of odontoma presence : 1- impacted tooth 2-retained primary tooth 3-alveolar swelling. X-ray : - numerous tiny teeth in a single focus. - location between root of impacted teeth. - amorphous mass ( complex one ) in same previous location. - early stage of tumor development " radiolucent with focal area of pacification ". Histopath : - enamel, dentin, cementum & pulp can be seen. - ghost cell keratinization can be seen. Differential diagnosis : - focal sclerosing osteitis, osteoma, periapical cemental dysplasia, ossifying fibroma & cementoblastoma. Tx : - odontoma have limited growth potential. - enucleation.
  7. 7. - a rare variant is odontoblastoma ( little is know but to be considered as ameloblastoma ). Oral pathology "2" ,, 5st lecture by Dr.Louiy ,, 13/3/2011 2) Ameloblastic fibroma and ameoblastic fibro-odontoma:  The difference between these two lesions is the preaence of odontoma.  They are benign lesions.  They are composed of neoplastic epi. and mesenchme.  the biological behavior are the same for both. Xray:   Well circumscribed (usually surround by sclerotic margin) ameloblastic fibroma (lucent) and ameloblastic fibro-odontoma (compound lucent-opaque) Clinical features:  No gender prediction  They are predominatly in children and young adults  Mandibular molar ramus are the preferred location Histopathology:  The lesion is lobulated & surrounded by fibrous capsule.  primaitive appearing myxoid connective tissue.  strands of odontogenic epithelium.  one or more foci of enamel and dentin (ameloblastic fibro-odontoma). Differential diagnosis " ameoblastic fibroma": i. Ameloblastoma ii. Odontogenic myxoma iii. Dentigerous cyst iv. OKC v. Central giant cyst granuloma
  8. 8. Differential diagnosis " ameoblastic fibro-odontoma": i. CEOT ii. CEOC iii. Adenomatoid odontogenic tumer Tx: Conservative surgical procedure (encapsulation) A malignant counterpart of ameloblastic fibrosarcoma can arise from pre-existing or recurrent ameloblastic fibroma.      fibrisarcoam : appears at any age. Maxilla <Mandible. Pain and paresthesia. locally aggressive with metastatic potential. resection is the treatment of choice. 3) Ceratocystic odontogenic tumer:       Unique cyst histopathology and aggressive clinical behavior. Anterior-posterior growth pattern without obvious clinical expansion. Typically 2-4th decade, slight male predilection. Most common in mandibular ramus/3rd molar region. Other findings include thooth displacement, mobility, root resorption, root divergence, pain and swelling. Peripheral variants have been reported. Key histology & treatmen/prognosis        Corrugated parakerathinized stratified squamous epithelium. Usually 6-8 cells thik, hyperchromatic, palissaded basal cell layer. Inflammation may focally. Alter architecture. Recurrence rate of approximately 30%. Enucleation [] resection . Long term clinical and radiographic follow-up. 4) Ossifying fibroma
  9. 9. It’s a benign neoplasm of bone that has the potential for excessive growth, bone destruction and recurrence. It is composed of fibrous connective tissue stroma in which a new bone is formed. Clinical features:  Age: third and fourth decades.  F < M.  Slow growing & expansile.  Asymptomatic.  It arise in the tooth-bearing regions.  Mandibular molar-premolar area.   X-ray: well circumscribed & well defined border. The lesion presents a variable appearance depending on the density of calcification present . Histopathology:  Fibrous connective tissue & fibroblasts.  Bone is immature surrounding by osteoblasts. Differential diagnosis:  Fibrous dysplasia.  Osteoblastoma.  Focal cementoosseous dysplasia.  Focal osteomyelitis.  Periapical cementoosseous dysplasia. Treatment: Surgical removal: enucleation or curettage. 5) Fibrous Dysplasia  A condition where a normal medullary bone is replaced by an abnormal fibrous connective tissue. Etiology: Genetic alteration may ultimately affect the proliferation and differentiation of fibroblasts/osteoblasts that make up the lesion.
  10. 10. Clinical features:  Asymptomatic-painless.  Slow enlargement of the involved bone.  Involvement of single (monostotic).  Involvement of multiple bone (polyostotic).  Maxilla < Mandible.  Unilateral swelling.  Onset during the 1st and 2nd decades of life. X-ray:  It ranges from radiolucent to uniformly radiopaque mass.  Poorly defined radiographic and clinical margins. Histopathology:  Fibrous connective tissue.  Trabeculae of immature bone.  Capillaries are prominent and uniformly distributed. Differential diagnosis:  Ossifying fibroma.  Chronic osteomyelitis (symptomatic). Treatment & prognosis:  It stabilizes after a variable period of prepubertal growth .  Small lesion my require no treatment (biopsy confirmation).  Larg lesion: surgical recontouring. 6) Osteoblastoma/Osteoid Osteoma  It is thought that osteoid osteoma represent a smaller variation of osteoblastoma. Clinical features  Osteoblastoma < 1.5 cm in diameter.  Osteoid osteoma > 1.5 cm in diameter.  Posterior tooth-bearing region are the usual site.  Most cases occur during the 2nd decades of life.
  11. 11.   Male < Female 2:1. Pain relived by NSAID (Osteoid osteoma) , no pain relief with Osteoblastoma. X-ray " Osteoblastoma" :  Well circumscribed.  Mixed radiolucent-radiopaque lesion. Histopathology " Osteoblastoma" :  Irregular Trabeculae of immature bone lined by osteoblasts. Differential diagnosis: i. Cementoblastoma. ii. Ossifying fibroma. iii. Fibrous dysplasia. iv. Osteosarcoma. Treatment & prognosis: Conservative surgical approach. 7) Osteoma     It is a benign lesion tumer composed of mature compact or cancellous bone. When arise on the surface of bone: Periosteal osteomas. When arise centrally: Endosteal osteomas Cause is unknown (trauma, infection & developmental abnormalities have been suggested). Clinical features:  Age: 2nd to 5th decades of life.  Male < Female.  Solitary except in Gardner`s syndrome.  Asymptomatic - Headache, recurrent sinusitis & ophthalmologic complaints may occur when skull bone are .  Slow growing hard mass. Gardner`s syndrome: It is characterized by:   Intestinal polyposis (very high rate of malignant transformation) Multiple osteomas
  12. 12.      Fibromas of the skin Epidermal cyst Impacted permanent & supernumerary teeth Odontomas Osteomas Histopathology:  One dorm composed of dense compact bone.  Other form composed of trabeculae of cancellous bone. Treatment:  Surgical excision.  In some instances periodic observation of small, asymptomatic osteomas is appropriate. Oral pathology "2" ,, 6th lecture by Dr.Louiy ,, 20/3/2011 8) Desmoplastic Fibroma : Clinical Features : - it's benign locally aggressive bone lesion. - cause is unknown. - age 30 years. - most common area : body ramus region. - asymptomatic, eventually cause swelling of the bone. X-ray : - uni or multi ocular. - either well demarcated or poorly defined. - cortical proliferation & root resorption may be seen. Histopathology : - densely collagenous fibers. - spindle shape fibroblast & collagenous fibers. - bone isn't produced. DD : Odontogenic cyst, odontogenic tumor, non-odontogenic lesion.
  13. 13. Local perforation may suggest malignancy. Tx : surgical excision. 9) Chondroma : Clinical Features : - benign eartilanous tumor of unknown cause. - painless slowly progressive lesion. - Common site is the anterior region or coronoid process. - age : mostly before 50 years. x-ray : irregular radioleucent area and foci of calcification may be seen. Histopathology : mature hyline cartilage. Tx : surgical excision. 10 ) Osteochondroma ( benign lesion of the jaw ). 11) Central Giant Cell Granuloma CGCG : - it's a benign proliferation of fibroblast and giant cells. X-ray : occur as solitary radioleucent lesion, multioccular, center of the bone. Etiology : - is classified as a benign neoplasm. - tumor fibroblast are believed to be responsible for recruiting of monocytes & Subsequently for transformation into multineucleated giant cells. Clinical Features : - age : children & young adult. – female more 2 : 1 . - mandible > maxilla. – painless expansion. - margin in x-ray are well demarcated. – aggressive CGCG exhibit rapid growth, root resorption & perforation of cortical bone. Histopathology :
  14. 14. - uniform fibroblast in collagenous stroma, capillaries, & multineucleated giant cells. - foci of osteoid may be seen. Deferential Diagnosis : Ameloblastoma, odontogenic myxoma, OKC, adenomatoid odontogenic tumor. Tx : surgical management, more extensive surgery needed if there are aggressive clinical features. 12) Hemangioma of the Bone : - it's mimic odontogenic & nonodontogenic lestion. Clinical Features : - more in the mandible, posterior region. – female > male. - age : 2nd decade. – asymptomatic expansion. – firm slow growing. - spontaneous bleeding of the gingival. - possible vertical mobility of the involved teeth. X-ray : multiocular radioleucencies, angular lobulation or cyst like radioleuncy & resorption root may be seen. of the Histopathology : - it represents a proliferation of blood vessels. - has cavernous type. Differential Diagnosis : Ameloblastoma, myxoma, CGCG, ABC. Tx : - may become a life threating if improperly treated. - needle aspiration is critical. - surgery, radiation therapy sclerosing agent, cryotherapy & presurgical embolization technique. 13) Langerhans Cell Diseases : - it's characterized by a proliferation of cells exhibiting phenotypic characteristics of langerhans cells. - etiology : is remains obscure ( similarities to langerhans cells located in the rpidermis & mucosa ). It's suggested that immunologic defects may affect normal regulatory mechanism with langerhans cell proliferation.
  15. 15. Clinical Features : - age : children, young & older adults. - any bone can be involved. - oral manifestation can be initial manifestation (80%) : a- tenderness, pain & swelling. b- loosening of teeth ( floating in space ) --- Very important --- gingival tissue is inflamed, hyperplastic and ulcerated. x-ray : radioleucent lesion ( solitary or multiple ). - cervical lymphodymopathy, mastoditis & otitis are common in multifocal involvement. Histopathology : - proliferation of large cells with abundant cytoplasm. - eosinophils and other inflammatory cells. - macrophages. - multineucleated giant cells & foci of necrosis. Differential Diagnosis : Juvenile or diabetic periodentitis, leukemia, hypophostaphasia, cystic neutropenia , malignant neoplasm, odontogenic tumor and cyst. 14) Tori & Exostosis : - they are nodular protuberances of mature bone. - they are neoplastic lesion. - they are rarely a source of discomfort. - surgical removal for propose of prosthetic rehabilitation. - Etiology & Pathogenesis : unknown. - parafunctional habit. - high occurance in some population. ( Torous Platinous + Torous Mandibularis + Exostosis + their Histopathology )  read it from the book. 15) Coronoid Hyperplasia : ( enlargement of coronoid process ).
  16. 16. Etiology & pathogenesis : unknown. Clinical features : bilateral, limit mandible movement, painless, no swelling or asymmetry & common in young male patient. Histopathology : - mature & hyperplastic bone which cartilaginous of fibrous CT. Tx : surgical excision + post operative physiotherapy. Malignancies of the Jaw 1) Osteosarcoma : ( 20% of all sarcoma ) - 5% occurs in the jaws. - it occur in different clinical setting including pre exciting bone abnormalities : 1-chronich osteomylitis 2fibrous dysplasia 3-giant cell granuloma 4-multiple osteosarcoma 5-paget's diseases 6-osteogensis imperfecta. - the majority involve long tubular bone ( adjacent to knee ). - can be classified to : a- conventional type ( medullary cavitiy ). b- juxtacortical tumors ( periosteal surface ). c- extra skeletal ( soft tissue ). - some gene have been cited as contributing to osteosarcoma e.g P53. Clinical Features : - male prevalence 60%. – age: 2nd decade for the skeletal osteosarcoma. - 3rd & 4th decade as general. – swelling. - more in the mandible than maxilla (body-symphesis- angle-ascending ramus-TMJ) - localized pain. – loosening, displacement of teeth and parasthesia.
  17. 17. - epistaxis. – nasal obstruction & eye problems. - mucosal ulceration ( not occur until late stage ). X-ray : - at early tumor stage is widening of PDL. - in late stage, mouth eaten irregular radioleucencies. Histopathology : All histological variant reflect the multi potentionality of the neoplastic mesenchymal cells in producing osteoid, cartilage & fibrous tissue. DD : chronic osteomylitis, clacifying epithelium odontogenic tumors. Oral pathology "2" ,, 7th lecture by Dr.Louiy ,, 27/3/2011 2) Parasteal osteosarcoma : - arise at periosteal surface. slow grow swelling or palpable mass. Age :39 , Male predominance for jaw involvement. Dull or aching sensation. X-ray : radio-dense and attached to the external surface of bone. A radiolucent clear space corresponding to the periosteum can be identified. Histopathology : - spindle cell stromae separating irregular trabecular. - periphery is less ossified than base. - invasion of underlying bone can be seen. Differential diagnosis : Osteoma , osteochondroma ,extosis. Treatment : #periosteal osteosarcoma : -enblock resection. -recurrence rate is high if cortical bone is not removed. - 5 year survival rate is 80%. #osteosarcoma : -surgery and chemotherapy. -when cortex is affected , surrounding tissue will be invaded. - 3 cm margin resection.
  18. 18. Pre-operative chemotherapy : -elimination of micro-metastasis. -size reduction. -induction of necrosis. -increase chance for tumor free margins. 3) Osteosarcoma : -intramedullary sarcomas showed no response to radiation therapy. -5 years survival rate is 25-40%. -prognosis of mandible is better than maxilla. -recurrence rate 40-70%. -areas of metastasis : lung and brain. - survival rate : 6 months when metastasis occur. - can manifestation in the jaw at early stage as widening of PDL. -2nd decade = skeleton osteosarcoma. -3rd to 4th decade for jaw involvement (8 - 85 years). 4) Chondrosarcoma : -well differentiated chondrogenic neoplasms to be considered potentially malignant. - Maxilla more than mandible. - adulthood disease. -painless swelling loosening of teeth. - signs can occur from surrounding bone invasion. - X-ray : range from mouth eaten radiolucency to diffusely opaque lesion. Histopathology : - Grade I : increase number of chondrocyte in chondroid to myxomatous storma (0% metastasis). - Grade II : myxoid storma with enlarged chondrocyte nuclei (10% metastasis). - Grade III : markedly cellular with spindle cell component and mitotic figure (70% metastasis ). - Grade I > II > III in prognosis. Differential diagnosis : Osteosarcoma , pleomorphic adenoma. Treatment : -Lesion is radio-resistant. -local or radical surgical excision is best.
  19. 19. 5) Ewing’s sarcoma: -it is highly lethal round cell sarcoma. -cause is unknown. -the cell of origin is uncertain ( related to primitive neuroectodermal tumor ). - 1% occur in jaws. - ramus of mandible is preferred site. Clinical features : - Age : 5 – 30 years ( average of 11 years in head and neck ). - Male > Female - Pain and swelling ( facial deformation ). - Mucosal ulcers. - Destruction of alveolar bone with loosening of teeth and mucosal ulcers. - Non-specific radiographic features. - Mouth eaten radiolucency of medullary bone , cortical erosion and expansion. Histopathology : - proliferation of uniform , closely packed cells ( round nuclie ) and fibrous bands. Differential diagnosis : Lymphoma , leukemia , chondrosarcoma , osteosarcoma. Treatment : -surgery and radiation for local control. -chemotherapy for systemic micro-metastasis.

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