j0305257 Soft tissue tumors and lesions. Bone pathology. Oral pathology j0305257 Soft tissue tumors and lesions û mesenchymal tumor-like lesions û true neoplasms û majority are rare in oral cavity û Diff. dg : hyperplastic lesions j0305257 Fibrous lesion of the oral mucosa ûHyperplastic lesions: û - epulides (fibrous, vascular, giant cell); û - pyogenic granuloma; û - fibroepithelial polyp; û - denture irritation and papillary hyperplasia û ûNeoplastic and neoplastic-like lesions: û - peripheral odontogenic fibroma û - fibrosarcoma û - fibrous histiocytoma û - nodular fasciitis û - fibromatosis û j0305257 Fibrous histiocytoma ûshow both fibroblastic + histiocytic differentiation ûmiddle-aged and older adult ûbuccal mucosa and vestibule ûnodular mass vary in size û û j0305257 Fibrous histiocytoma 1 - fibroblasts 2 - histiocytes 2 1 j0305257 Nodular fasciitis ûrare in oral cavity, superficial ûrapidly growing pseudosarcomatous lesion ûself-limiting growth of a clone of neoplastic cells that contain a fusion gene (USP6 rearrangement) ûpainfull, tenderness ûhistologically may resemble fibrosarcoma û û j0305257 Fibromatosis û non-neoplastic but infiltrative fibrous lesion û do not metastasize!!! û children or young adults (juvenile fibromatosis) û paramandibular soft tissue region û vary in size facial disfigurement û firm mass with rapid growth + destruction adjacent bone ûMicro: proliferation of spindle-shaped cells, no cytonuclear atypia ûTreatment: wild excision û recurrence rate 23% û û j0305257 Fibromatosis proliferation of spindle-shaped cells, without cytonuclear atypia j0305257 Fibrosarcoma û malignant tumor of fibroblasts û rare in oral cavity û have good prognosis û young adults and children ûMicro: fascicles of fibroblasts that forms “herringbone”pattern j0305257 Fibrosarcoma fibroblasts with “herringbone”pattern cytonuclear atypia j0305257 Tumors of adipose tissue û Lipoma – benign tumor of adipose tissue û- 40 yrs or older û- mucosa of cheeks and tongue û!!! ulcerated tumor-like masses of partly necrotic fat in very young children - the result of traumatic herniation of cheek’s mucosa û ûGross: soft, yellowish-colored swelling ûMicro: mature adipose tissue, thin fibrous capsule û û Fibrolipoma – lipoma with increasing amount of fibrous stroma û û û û û û j0305257 Tumors of adipose tissue lipoma fibrolipoma fibrous stroma j0305257 Tumors of adipose tissue û Liposarcoma – malignant tumor of adipose tissue û- peak 40-60 yrs û- cheeks, floor of the mouth, tongue û- resemble benign lipoma + atypical hyperchromatic nuclear û- myxoid, round-cell, well-differentiated, pleomorphic, dedifferentiated û Have a good prognosis in oral cavity û û j0305257 Liposarcoma Iipoblasts with cytonuclear atypia j0305257 Tumors of vascular tissue û Hemangioma – benign hamartomatous tumor û- 1-year-old children, F:M 3:1 û- lips, tongue, cheeks or palate ûGross: solitary, flat or raised, dark reddish-purple in color û typically blanch on pressure û û Hereditary haemorrhagic telangiectasia – AD, multiple telangiectases in skin, mucous membrane, internal organs û Sturge-Weber syndrome – haemangiomatous lesions of the face (n. trigeminus) + heamangiomas and calcification of leptomeninges + limbs affecting û û û û j0305257 Hemagioma û Histological types: capillary, cavernous, mixed û Complications: ulceration û thrombosis û organization û calcification û Treatment: “watchful neglect”, occur regression û ûSublingual varicosities - purplish venous ectasia on the ventral (undersurface) of the tongue after the age of fifty. j0305257 Hemangioma sublingual hemangioma capillary type – proliferation of capillary-sized vessels j0305257 Tumors of vascular tissue û Lymphangioma - benign hamartomatous tumor lymphatic vessels û- early childhood û- anterior 2/3 of the tongue (macroglossia) û trauma sudden increase in size ûGross: pebbly surface ûMicro: endothelial-lined spaces containing lymph û ûCystic hygroma – large, fluctuant swelling (>10 cm) of the head and neck region, may extend to oral cavity. j0305257 Lymphangioma dilated lymphatic vessels beneath the epithelium pebbly surface of the tongue j0305257 Tumors of peripheral nerves û1. Neurofibroma û - solitary û - multiple û2. Neurinoma (Schwannoma) û3. Multiple mucosal neuromas (MEN sy) û4. Traumatic neuroma û5. Granular cell tumor j0305257 Tumors of peripheral nerves û Neurofibroma – mixture proliferation of Schwann cell and fibroblasts û - young adults, children û Solitary lesion – tongue, buccal mucosa, well-circumscribed nodules û Multiple lesions associated with neurofibromatosis (von Recklinhause’s disease of nerves) – AD, mutations in NF1 gene, located 17q11.2 û skin pigmentation (coffee and milk) + involving of cutaneous nerves + axillary freckling + oral lesions û!!! May be associated with tumors of CNS, leukemia, RMS, WT… û j0305257 Tumors of peripheral nerves ûOral lesions: û mucosal swelling of the tongue, gingiva û enlargement of the fungiform papilla û enlargement of mandibular foramen û increased bone density û û Risk of malignization MPNST (neurofibrosarcoma) 5-15% ûTreatment: no specific therapy û j0305257 Neurofibromatosis Involving of cutaneous nerves + mucous swelling of the tongue, gingiva j0305257 Tumors of peripheral nerves ûNeurinoma (Schwannoma) - benign neoplasm of Schwann cell origin û- young and middle-aged adults û- tongue, intraosseous in the posterior mandible û û Gross: vary in size, encapsulated tumor û Micro: 2 patterns – Antoni A (palisaded nuclei, Verocay bodies) û Antoni B (less cellular fields) ûTreatment: surgical excision û û j0305257 Neurinoma (Schwannoma) 1 – Antoni A pattern 2 – Antoni B pattern 1 2 j0305257 Traumatic neuroma ûTraumatic neuroma (amputation neuroma) – tumor-like reactive proliferation of Schwann cells ûcause: transection or other damage of a nerve bundle ûmiddle-age adults, F>M ûmental foramen area, tongue, lower lip ûpainful lesion!!! û Gross: small nodule û Micro: proliferation of mature nerve bundles, fibrotic stroma, mild chronic inflammation û ûTreatment: surgical excision (incl. involved nerve bundle) û û j0305257 Granular cell tumor û Granular cell tumor – tumor of unknown origin û (in the past was called the granular cell myoblastoma) û û- wide age range û- dorsal surface of the tongue - û Gross: non-encapsulated lesion û Micro: large cells with granular cytoplasm, û pseudoepitheliomatous hyperplasia of the overlying epithelium - û j0305257 Granular cell tumor non-encapsulated lesion of tongue’s mucosa large cells with granular cytoplasm j0305257 Tumors of muscle û Leiomyoma – benign smooth muscle tumors û ûprobably, leiomyomas of the oral cavity have their origin from vascular smooth muscle û û Leiomyosarcoma and rhabdomyosarcoma are both very rare in oral cavity j0305257 û û ûBone pathology j0305257 Inherited and development disorders of bone û1. Osteogenesis imperfecta û2. Osteopetrosis û3. Cleidocranial dyspasia û4. Achondroplasia û5. Fibro-osseous lesions û6. Cherubism j0305257 Inherited disorders of bone ûuncommon diseases ûjaw involvement variable ûorofacial manifestations include: û - abnormalities in number, form, structure of teeth û - malocclusion û - abnormal facial appearances j0305257 Osteogenesis imperfecta ûAD, mutations in the genes that code for type-1 collagen (80-90%) ûgeneralized osteoporosis (slender bones) û Clinically 4 main type: û Type I (classic type) – AD, blue sclera, deafness, +/- dentinogenesis imperfecta û Type II (perinatal lethal) – AD û Type III (progressively deforming) – AD/AR, severe osteoporosis, progressive deformities, dentinogenesis imperfecta û Type IV – AD, similar to type I, but more severe û û Micro: immature, woven bones of cortex j0305257 Osteogenesis imperfecta Dentinogenesis imperfecta: note delicate bone trabeculae + obliteration of pulp chambers j0305257 Osteopetrosis (marble bone disease) ûexcessive density of all bones ûobliteration of marrow cavities secondary anemia ûdefect in osteoclastic activity, failure in the remodeling of the developing bone ûbones mechanically week, common fractures!!! û û Symptoms: delayed eruption of teeth, osteomyelitis (after tooth extraction) û Radiography: mandible>>maxilla, invisible roots of the teeth û û j0305257 Cleidocranial dysplasia (cleidocranial dysostosis) ûAD, mutations to the RUNX2 gene ûdisturbance of differentiation of osteoblasts from precursor cells ûabnormalities of the skull, jaws, clavicle (partial/complete absence) ûmaxilla with a high, narrow arched palate ûdelayed or non-eruption of the permanent dentition, supernumerary teeth û ûRadiography: thin teeth’s roots j0305257 Cleidocranial dysplasia Complete absence of clavicles Retention of deciduous teeth + multiple impactions of permanent teeth j0305257 Fibro-osseous lesions ûDivided into: û I. Osseous dysplasia û 1. Fibrous dysplasia (monostotic/polyostotic) û 2. Cemento-osseous dysplasia û II. Benign neoplasia (ossifying fibroma) û ûreplacement of normal bone by cellular fibrotic tissue ûcontain woven bone + acellular islands of mineralized tissue develop û j0305257 Fibrous dysplasia (FD) ûdevelopment disorder, but not inherited ûMonostotic FD: much more common! ûchildhood, adolescence û reactivation of quiescent lesion during pregnancy ûaffected 1 bone: limb, skull bones, particularly the jaws û maxilla>>mandible ûCraniofacial fibrous dysplasia – 1 bone is affected (maxilla) + involvement of adjacent bones û Symptoms: painless swelling of the maxilla (buccally) facial asymmetry û rapid and extensive growth exophthalmos û mandibular lesions fusiform expansion + displacement of teeth û Gross: ill-defined smooth enlargement û Radiography: ground-glass/orange-peel-stippling effect û displaced teeth, separated roots j0305257 Fibrous dysplasia (FD) j0305257 Fibrous dysplasia (FD) ûPolyostotic FD ûaffected several bones, segmentally lesions ûaffected sites: limb (lower), skull bones, vertebrae, ribs, pelvis ûchildhood, F:M 2-3:1 û expansion usually stops with skeletal maturation û McCune-Albright syndrome – bone lesions are accompanied by skin pigmentation, sexual precocity, endocrine abnormalities û ûMicro: delicate trabeculae of woven bone + fibrous tissue ûRemodelling of woven to lamellar bone may occur with increasing age!!! û ûTreatment: not radiosensitive !!! (risk of malignant transformation to fibrosarcoma) j0305257 Fibrous dysplasia (FD) 1 – woven bone 2 – fibrous tissue 1 2 j0305257 Cemento-osseous dysplasia ûosseous dysplasia of jaws, which involves the tooth-bearing areas ûF>>M, over 30 yrs old, mandible>>maxilla û Based on the clinical and radiographic features: û periapical, focal and florid cemento-osseous dysplasia ûClinically: û û û ûMicro: fibrous tissue + bone/calcified accelular tisue develop ûRadiography: radiolucent/mixed/radiopaque û multiple and small <1 cm multiple and large >1 cm associated with apical areas of the mandibular incisors involve 1 or more quadrants in one or both jaws j0305257 Inflammatory diseases of bone û1. Alveolar osteitis (dry socket) û2. Focal sclerosing (condensing) osteitis û3. Osteomyelitis û4. Chronic periostitis û5. Radiation injury and osteoradionecrosis j0305257 Osteomyelitis û Local factors û trauma û radiation injury û Paget’s disease û osteopetrosis û major vessel disease û ûSystemic factors ûimmune deficiency states ûimmunosuppresson ûDM ûmalnutrition ûextremes of ages - now is a rare disease - polymicrobial infection Predisposing factors: j0305257 Suppurative osteomyelitis ûclinically: acute, chronic (>1 month) ûmandible>maxilla ûsource of the infection – dental abscess, fractures, penetraiting wounds, extractions ûSymptoms: û acute lesion- pain, swelling, pyrexia, malaise, mobility of teeth û chronic – discharge of pus through 1 or more sinuses ûMicro: suppurative inflammation, necrosis of the bones, pus within marrow spaces, vascular thrombosis ûComplication: sequestrum (exfoliated through a sinus) û surgical removing û j0305257 Suppurative osteomyelitis 1 – purulent inflammation 2 – necrosis of the bone 1 2 j0305257 Chronic osteomyelitis with proliferative periostitis ûsyn. Garré’s osteomyelitis, periostitis ossificans ûtype of sclerosing osteomyelitis ûmandible, children and young adults ûGross: swelling on the outer surface of the mandible ûMicro: subperiosteal mass of trabeculae of woven bone + chronic inflammation in fibrous marrow û j0305257 Chronic osteomyelitis with proliferative periostitis Subperiosteal mass in mandible j0305257 Metabolic and endocrine disorders of bone û1. Osteoporosis û2. Primary hyperparathyroidism û3. Secondary hyperparathyroidism û4. Rickets and osteomalacia û5. Acromegaly û j0305257 Osteoporosis ûexcessive bone loss/when the apposition of bone is reduced ûF:M 2:1 ûpostmenopausal women (rate of bone’s loss 1-8% per year) ûedentulous patients (mandible) ûaccentuated in Cushing syndrome, thyrotoxicosis, primary hyperparathyroidism ûosteoporotic bone is reduced in quantity û Radiography: increased radiolucency, thin cortex û û û j0305257 Hyperparathyroidism ûPrimary û secretion of parathormone û (adenoma/Ca, hyperplasia PG) ûhypercalciemia, hypercalciuria + ûpathological metastatic calcification û Micro: brown tumor (haemosiderin + fibrotic tissue + multinucleated, osteoclast-like giant cells) û ûSecondary ûresponse to chronic hypocalciemia (CRI) û may associated with rickets and osteomalacia ûMicro: uncalcified osteoid + brown tumor may affect jaws j0305257 Paget’s disease of bone ûform of osteodystrophy, disorganized formation and remodeling of bone ûaetiology - unclear û genetic and environmental factors, paramyxovirus infection û >40 yrs, more common in maxilla û Phases: û1. Osteolytic û2. Mixed osteolytic and osteogenesis û3. Osteoblastic û j0305257 Paget’s disease of bone ûSymptoms: bone pain, cranial nerve compression, facial û deformity, difficulties in wearing dentures û hypercementosis, ankylosis difficulty in extraction û root resorption (1 phase) û increased alkaline phosphatase ûMicro: criss-crossing reversal lines, mosaic bone ûComplication: risk of malignant transformation (osteosarcoma) û û j0305257 Paget’s disease of bone criss-crossing reversal lines, mosaic bone j0305257 Tumors of bone û1. Bone-forming tumors û Benign: Osteoma û Osteoblastoma û Malignant: Osteosarcoma û2. Cartilage-forming tumors û Benign: Chondroma û Malignant: Chondrosarcoma û3. Marrow tumors: Myeloma û û4. Histiocytic and dendritic cell neoplasms Langerhans cell histiocytosis û û5. Vascular tumors: Haemangioma of bone û6. Fibrous tumors: Ossifying (cemento-ossifying) fibroma û7. Metastatic tumors û û j0305257 Bone-forming tumors ûOsteoma – benign, slow-growing tumor ûadults, mandible>maxilla û Gross: solitary, well-circumscribe lesion û multiple osteomas of the jaws occur as a feature of Gardner sy û Micro: compact type: dense lamellar bone û cancellous type: interconnecting trabeculae + fibrous marrow û ûOsteoblastoma – rare tumor in the jaws û Micro: cementoblastoma (!!!not related to the roots of the teeth) j0305257 Bone-forming tumors ûOsteosarcoma – primary malignant Tu of bone û 30 yrs and older û relatively rare in jaws û Intramedullary type - arise centrally within the jaws û Juxtacortical type – peripherally in the relation to the periosteum, better prognosis û ûMicro: malignant osteoblasts + abnormal osteoid ûMetastasis: RLN, lungs, brain ûTreatment: neoadjuvant CT+ surgical removal + adjuvant CT j0305257 Osteosarcoma 1 – malignant osteoblasts 2 – abnormal osteoid 1 2 j0305257 Cartilage-forming tumors ûChondroma – rare benign Tu in the jaws û3-4 decades û Mandible (condylar process, posterior part) û Maxilla (anterior part) û Micro: circumscribed mass of mature hyaline cartilage û cellularity, binucleated cells susp well-differentiate chondrosarcoma !!! û û Prognosis: better for mandibular lesions j0305257 Chondrosarcoma Binucleated atypical chondrocytes j0305257 Marrow tumors ûMyeloma – plasma cells neoplasm û Multiple myeloma – disseminated disease involving many bones û Solitary myeloma (plasmocytoma) – solitary lesion û50-70 yrs ûskull, vertebrae, sternum…(sites with red marrow) ûabnormally high levels of single homogenous type Ig in serum (paraprotein) û Radiography: osteolytic lesions (punched-out radiolucencies) û Micro: cellular sheets of Tu cells resemblance to plasma cells û IHC: positivity of CD20, CD138, kappa, lambda j0305257 Myeloma Tumour’s cells resemblance to plasma cells IHC: positivity of CD138 j0305257 Histiocytic and dendritic cell neoplasms ûLangerhans cell histiocytosis – clonal proliferation of Langerhans-type cells û Solitary lesion in bone (unifocal eosinophilic granuloma) û Multifocal eosinophilic granuloma (bone + other organs) û Disseminated multiorgan disease (Litterer-Siwe disease) û Unifocal/multifocal eosinophilic granulomas: û- <20 yrs, M:F 2:1 û- cranuim and jaws (mandible) û Radiography: solitary/multiple osteolytic lesions û Micro: histiocytes + variable numbers of eosinophils û EM: Birback granules û IHC: positivity of CD1α, S100 û j0305257 Langerhans cell histiocytosis Histiocytes + variable numbers of eosinophils IHC: positivity of CD1α j0305257 Fibrous tumors ûOssifying (cemento-ossifying) fibroma – benign well-demarcated !!! neoplasm û wide age range, F>M û rapid growth in children/adolescence - juvenile ossifying fibroma û ûMicro: well circumscribed cellular fibrous tissue + trabeculae of bone ûDiff.dg: fibrous dysplasia û ûJuvenile ossifying fibroma – richly cellular + high mitotic activity + immature-looking woven bone (recc. rate 30-60%) ûDiff. dg: osteosarcoma û û û û j0305257 Metastatic tumors û1% of malignant Tu of oral cavity ûMandible>>maxilla û gingiva, alveolar mucosa, tongue û Ca of breast, bronchus, kidney… û Mts may cause: osteolytic changes û osteoblastic changes j0305257 û û Thank you for your attention... 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