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									                                                                                             Pseudotumors of orofacial region
                                                                                             True tumors of orofacial region
                                                                                                 Odontogenic tumors
                       Tumors of orofacial region                                                Soft tissue and bone tumors
                                                                                                 Epitelial tumors
                                                                                                 Salivary gland tumors

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Pseudotumors of orofacial region                                                                                                          Malassez)
                                                                                         Odontogenic cysts (cysts derivated from rests of Malassez)

    mostly the jaw cysts                                                                     Rests of Malassez – rests of Hertwig's Epithelial Root Sheath
    classified in several categories depending on histogenesis and etiology

    True cysts                                                                                                radicular)
                                                                                             Periapical cyst (radicular)
        Odontogenic cysts (arising from the epithelium involved in the process of
        teeth development)
            From the rests of Malassez (periapical, residual cyst)
                                                 (dentigerous, eruption, paradental)
            From the reduced enamel epithelium (dentigerous, eruption, paradental)
            From dental lamina (rests of Serres) (odontogenic keratocyst, lateral
            periodontal cyst)

        Non-odontogenic cysts
        Non-                                                                                 Residual cyst
           Developmental cyst

        Post-traumatic bone cyst
        Aneuryzmatická bone cyst

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                                                                                         Odontogenic cysts (cysts derivated from reduced enamel
Periapical (radicular) cyst

    Located at the root tips of nonvital teeth
    Activation: necrosis of the pulp, advanced tooth caries, chronic pulpitis
    Remnants of Hertwig’s epithelial root sheath proliferate due to the
                                                                                             Follicular (dentigerous
    inflammatory stimulus                                                                    cyst)
    subsequent liquefaction necrosis in the center of these enlarged
    epithelial nests

    lined by nonkeratinising squamous epithelium
    may be thin and atrophic but
    mucous cells may be present
    inflammatory changes in the underlying                                                   Eruption cyst
    connective tissue
    cholesterol clefts with adjacent giant cells
    hyaline Rushton bodies

    residual cyst is radicular cyst that is
    retained in jaws after removal of the
    associated tooth
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Odontogenic cysts (cysts derivated from reduced enamel
                                                                             Dentigerous (follicular) cyst

                                                                                 Developmental cyst
    Follicular (dentigerous                                                      surrounds the crown of an unerupted tooth and is attached to
                                                                                 the neck of this tooth

                                                                                 Usually associated with the maxillary canine or the mandibular
                                                                                 third molar tooth
                                                                                 accumulation of fluid between the reduced enamel epithelium
                                                                                 and the crown surface or between the layers of the enamel
                                                                                 epithelium itself
    Eruption cyst
                                                                                 no epithelial proliferation is needed to form this cyst

                                                                                 thin epithelial lining - two to three cells thick
                                                                                 resembles the reduced enamel epithelium
                                                                                 mucus-producing cells as well as ciliated cells are more common

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                                                                             Odontogenic cysts (from dental lamina remnants – rests of
Eruption cyst

    type of dentigerous cyst located in the gingival soft tissues
    overlying the crown of an erupting tooth                                     Odontogenic keratocyt

    appear as blue-stained blisters of the oral mucosa
    cyst rupturing with progressive eruption of the associated tooth

                                                                                 Lateral periodontal cyst

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Odontogenic keratocyst
                                                                             Nonodontogenic cysts (developmental cysts)

        Frequently affects mandible – last third of mandible and ramus
                                                                                 Fissural cysts:
        Typically located in bone with possible bone destruction
                                                                                     Median mandibular cyst (median
        Recurrence rate 25-60% (like ameloblastoma)                                  alveolar), Median palatal cyst
        Gorlin-                                     ne
        Gorlin-Goltz syndrome - multiple OKC, AD, nevoid basal cell                  Globulomaxillary cyst
        carcinomas, milias,                                                          Nasolabial cyst (nasoalveolar)
                                                                                     Lateral cysts, Branchiogenic cysts
                              (6- cells)
        thin epithelial layer (6-10 cells) with parakeratosis
        basal cell layer                                                         Remnants of epithelial ducts
        no inflammatory reaction                                                     Nasopalatine duct cyst (incisive canal
                                                                                     cyst, nasopalatine canal cyst)
                                                                                     Ductus thyreo-glossus cyst

                                                                                 Dermoid cyst

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   Nasopalatine duct cyst                                                          Nasolabial cyst

    Midline cyst near the incisive foramen                                          in the soft tissue, beneath the ala of
    intraosseous lesion                                                             the nose
    from nasopalatine duct remnants                                                 from parts of nasolacrimal duct

    epithelial lining (squamous,                                                    pseudostratified collumnar epithelium
    respiratory, with or without mucous                                             mucous cells
    cells, cuboidal, ...)
    chronic inflammatory infiltrate

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   Dermoid cyst

        medial cyst in the neck region, base of the oral cavity
        teratoma                                                                    Pseudotumors of orofacial region
                                                                                    True tumors of orofacial region
        epithelium: squamous epithelium                                                 Odontogenic tumors
        fibrous capsule may contain sweat and sebaceous glands, hair                    Epitelial tumors
        follicles, ...                                                                  Salivary gland tumors
                                                                                        Soft tissue and bone tumors

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Odontogenic tumors
                                                                                Tumor grows slowly; typical is locally aggressive behaviour, but do not metastasise
                                                                                most common odontogenic tumour
    group of lesions that arises from the odontogenic tissue. They develop
    from the epithelial part of the tooth germ, the ectomesenchymal part,       mandible (molars) 75%, 20-40y
    or from both.                                                               Deformating the face, loosening of teeth
                                                                                X-ray - as multilocular cyst
                                                                                Rare metastatic – malignant ameloblastoma
    Epithelial odontogenic tumors
                                                                                polycystic amelobalstoma - unicystic ameloblastoma (younger patients)
    Odontogenic tumors from connective tissue
          Odontogenic fibroma
                                                                                anastomosing epithelial strands and fields or discrete epithelial islands
          Odontogenic myxoma
                                                                                peripheral cells at the border with the adjacent fibrous stroma are columnar, with
          Cementoblastoma                                                       nuclei usually in the apical half of the cell body away from the basement membrane
    Mixed odontogenic tumors                                                    tumour infiltrates into the adjacent cancellous bone
          Odontoma                                                              spread into soft tissues is highly unusual
          Ameloblastic fibroma
    Malignant                                                                   follicular type – tumor islands in fibrous stroma
          Malignant ameloblastoma                                                                                           pattern)
                                                                                plexiform type – forming net (the farmer pattern)

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Odontogenic tumors                                                          Odontogenic fibroma
    group of lesions that arises from the odontogenic tissue. They
    develop from the epithelial part of the tooth germ, the
    ectomesenchymal part, or from both.
                                    both.                                       peripheral or central tumor with the dominant fibrous component

                                                                                    odontogenic fibroma (with
    Epithelial odontogenic tumors
                                                                                    odontogenic epithelium)
    Odontogenic tumors from connective tissue
           Odontogenic fibroma
           Odontogenic myxoma
    Mixed odontogenic tumors
           Ameloblastic fibroma
           Malignant ameloblastoma
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Odontogenic fibroma                                                         Odontogenic myxoma

    The lesion is seen within the jaw as well as in the gingiva                 rare tumor, growing in the bone with the locally agressive
    Odontogenic fibroma consists of fibroblasts lying in a background           behaviour
    of myxoid material with collagen fibres that may vary from                  maxilla a mandibula
    delicate to coarse
                                                                                erosion into the surrounding
    Odontogenic epithelium, may occur.                                          area (sinus)
    Only rarely is the epithelial component so conspicuous that                           painless
                                                                                swelling, painless growth
    differentiation between odontogenic fibroma and ameloblastoma
                                                                                X-ray: resembling honeycomb
    may be difficult.

                                                                                curettage or block resection

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Cementoblastoma                                                             Odontogenic tumors
        benign, well circimscribed tumor, heavily mineralised cementum
        masses connected to the apical root
                                                                                group of lesions that arises from the odontogenic tissue. They develop
        rare tumor                                                              from the epithelial part of the tooth germ, the ectomesenchymal part,
                                                                                or from both.
        20-30 y patients
        molars a premolars
                                                                                Epithelial odontogenic tumors
        pain – tooth is vital                                                       Ameloblastoma
                                                                                Odontogenic tumors from connective tissue
        vascular, loose-textured fibrous tissue                                     Odontogenic fibroma
        surrounds coarse trabeculae of basophilic                                   Odontogenic myxoma
        mineralised material bordered by plump cells                                Cementoblastoma
        with large but not atypical nuclei.                                     Mixed odontogenic tumors
        Mitotic figures are rare
                                                                                    Ameloblastic fibroma
        Also, osteoclastic giant cells may be present
                                                                                    Malignant ameloblastoma
        Th: tooth extraction

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Odontoma                                                                                  Odontogenic tumors

    70% odontogenic tumors, hamartoma                                                         group of lesions that arises from the odontogenic tissue. They develop
    the differenciation into dontoblast and ameloblast (dentin and enamel                     from the epithelial part of the tooth germ, the ectomesenchymal part,
    production) is present                                                                    or from both.
    benign process
    10-     life,
    10-20 y life, maxilla                                                                     Epithelial odontogenic tumors
    Histologically:                                                                           Odontogenic tumors from connective tissue
    Complex form                                                                                  Odontogenic fibroma
        well delineated mass of dental hard tissues in a irregular arrangement
        (dentin, enamel, cementum)                                                                Odontogenic myxoma
        stroma consists of mature fibrous connective tissue                                       Cementoblastoma
    Compound form                                                                             Mixed odontogenic tumors
        malformation consisting of tiny teeth                                                     Odontoma
        may vary in number from only a few to numerous                                            Ameloblastic fibroma
        do not resemble normal teeth                                                          Malignant
        normal arrangement of centrally placed fibrovascular pulp tissue surrounded
        by dentin with enamel in the crown area and cementum in the root part.                    Malignant ameloblastoma

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    Malignant ameloblastoma (mts)
                             mts)                                                             Pseudotumors of orofacial region
    Ameloblastic carcinoma                                                                    True tumors of orofacial region
        resembling the ameloblastoma, but with cellular atypias
                       ameloblastoma,                                                             Odontogenic tumors
                                                                                                  Soft tissue and bone tumors
                                                                                                  Epitelial tumors
                                                                                                  Salivary gland tumors

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Soft tissue tumors                                                                        Bone tumors

    mesenchymal tumors                                                                               dignity:
                                                                                              by the dignity: benign and malignant
        connective tissue             fibroma             fibrosarcoma                            Osteoma
        cartilage                     chondroma           chondrosarcoma                          Osteoblastoma
        fat tissue                    lipoma              liposarcoma                             Giant cell lesions
        blood vessels                 hemangioma          hemangiosarcoma                         Aneurysmal bone cyst
        lymphatic vessels             lymphangioma        lymphangiosarcoma                   Malignant
        cross-striated m.             rhabdomyoma         rhabdomyosarcoma                                                 sarcoma)
                                                                                                  Osteosarcoma (osteogenic sarcoma)
        smooth m.                     leiomyoma           leiomyosarcoma                          Chondrosarcoma
        ...                                                                                       Ewing's sarcoma

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Benign                                                                                   fibro-
                                                                                  Benign fibro-osseous lesions

    Benign fibro-osseous lesions                                                      nonneoplastic intraosseous lesions that replace normal bone and
    Osteoma                                                                           consist of cellular fibrous connective tissue
    Giant cell lesions                                                                                                         mandible, asymptomatic,
                                                                                      periapical cemental dysplasia – anterior mandible, asymptomatic,
                                                                                      radiolucent areas
                                                                                      florid cemento-osseous dysplasia – nonsymptomatic
                                                                                      fibrous dysplasia – one or many bones, mild malocclusion
                                                                                                            facies, symetric,
                                                                                      cherrubism – distant facies, symetric, severe malocclusion

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Cherrubism                                                                        Osteoma / exostosis / torus palatinus

    loss of bone in the mandible replaced by fibrous tissue                                   palatinus,
                                                                                      torus – palatinus, mandibularis
    usually the condition fades as the child grows
    deform the affected person's face
    causes premature loss of the primary teeth and uneruption of
    the permanent teeth.

    rare autosomal dominant disease of the maxilla and mandible
    Mutations in the SH3BP2 gene have been identified in about 80
    percent of people with cherubism
    Orthodontic treatment

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Osteoma / exostosis / torus palatinus                                             Osteoblastoma

    torus palatinus                                                                    cellulary“ osteoma,
                                                                                      „cellulary“ osteoma,
        20% older patients                                                            osteoblastoma (2cm and more)
        osseous structure lined by epithelium                                                          (0,5-
                                                                                      osteoid osteoma (0,5-2cm)
        ulcerations                                                                          patients,
                                                                                      young patients, swelling a pain
    torus mandibularis                                                                osteoblasts a osteoclasts
        bilateral                                                                     activation + cellulary bone +
        dentures irritation                                                           osteoid areas
        solitary – multilocular (AD, Gardner syndroma ~ + intestinal polyps,
        cysts a skin fibromas)

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Giant cell lesions                                                        Malignant

    many lesions of the mandible and                                          Ewing's sarcoma
    maxilla contain giant cell tissue
    peripheral giant cell granuloma
        extraossueous nodule in jaws                                                                   sarcoma)
                                                                              Osteosarcoma (osteogenic sarcoma)
        from periosteum and periodontal                                           1:100.000 people
                                                                                  cca. 7% in the neck and face area
        30-40 y women
        focal prple nodule on the gigiva                                                                          gene)
                                                                                  retinoblastoma association (Rb1 gene)
    aneurysmal bone cyst                                                          pain?/face deformation/
                                                                                  pain?/face deformation/teeth lost
        vessels resembling hemangioma
        10-20 y pacienti                                                          normal a abnormal osteoid
        madibla – ramus
                                                                                  pleomorfic a hyperchromic
        face deformation / bone fracture
                                                                                  sarkomatous cells

    dif.dg.traumatic bone cyst

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Ewing's sarcoma                                                           Epithelial tumors

    highly malignant bone tumor (10% of malignant bone tumors)            From squamous epithelium
    neuroektodermal cells                                                    Benign
    ESFT - t (11.22) - EWS/FLI1                                                   Keratoacanthoma
    human adenovirus type 5                                                   Benign pigmented lesions
                                                                                  Peutz-Jeghersov syndróm
    children and adolescents (not older than 30 years)                        Leukoplakia
                                                                              Epithelial dysplasia
    fever, leukocytosis, FW
    pain and swelling, focal ulceration                                       Malignant
                                                                                  Squamous cell carcinoma
    sensitive and chemo-and radiotherapy
                  chemo-                                                          Basal cell carcinoma

    recurrence rate (30% per 5 years)
                                                                          From glandular epithelium
                                                                             Salivary gland tumors

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Papilloma                                                                 Keratoacanthoma

                                                                              predilection for the face, hands
                                                                              solitary nodule, fast growth, possible spontaneous regression

                                                                              solid papule with a central hollow, filled with keratin

                                                                              central keratinization, hyperkeratosis seems embedded in the
                                            HPV (not allways)                                                    epidermis;
                                                                              epidermis, but bounded by normal epidermis; at the base of the
                                            keratinisating /                                               present,
                                                                              infiltrative growing may be present, possibly mitotic activity but
                                            nonkeratinizating                 no atypical mitoses, small abscesses in the epithelium, the
                                            basal layer / spinosa /           inflammatory infiltrate
                                            keratinocytes                     elderly

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Epithelial tumors                                                        Peutz-
                                                                         Peutz-Jeghers syndrome

From squamous epithelium                                                     AD
   Benign                                                                    formation of benign polyps in the gastrointestinal - hamarthomas
                                                                             (risk intususcepcia!!)
    Benign pigmented lesions                                                 hyperpigmented maculae of the lips and mucosa
        Peutz-Jeghersov syndróm
        Peutz-                                                               dif.dg. hyperpigmentation: Addison's disease (adrenal insuf.)
    Epithelial dysplasia
        Squamous cell carcinoma
        Basal cell carcinoma

From glandular epithelium
   Salivary gland tumors

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Epithelial tumors
                                                                                 "white coatings at the mocous of oral cavity, which can not be
                                                                                 wiped off and classified as other disease"
From squamous epithelium                                                         precancerosis!
   Benign                                                                                                  candidosis,
                                                                                 smoking!, alcohol, chron. candidosis, HPV, EBV ...
        Keratoacanthoma                                                          homogeneous - flat, uniform, white areas
    Benign pigmented lesions                                                     non-homogeneous - irregular, nodular areas + erythroplakia
        Peutz-Jeghersov syndróm
    Leukoplakia                                                                  clinical diagnosis, has a characteristic histologic correlate
    Epithelial dysplasia                                                         acanthosis = thickening of the spinous layer of squamous
    Erythroplakia                                                                epithelium
    Malignant                                                                    hyperkeratosis = thickening of the keratin layer
        Squamous cell carcinoma                                                  atrophy – erythroplakia
        Basal cell carcinoma

                                                                                 may lead to
From glandular epithelium
                                                                                 dysplasia and CIS
   Salivary gland tumors

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Squamous cell carcinoma                                                  Basal cell carcinoma

                                                                             skin tumor (predominantly on the face) – proliferation of the
                                                                             cells similar to the immature cells of the basal layer of the
    malignant epithelial tumors
                                                                             epidermis (but arises from hair follicule)
    smoking, sunlight, alcohol, chron.irritation, viruses
                                                                             variable histological features
    older age, 90% men
                                                                             grows predominantly endophytic, with the dermis infiltration, and
    mostly lower lip and tongue                                              surface exulceration (ulcus rodens)
    firstly, the exophytic growth, slow growth and late metastases           without metastasis (semimalignant), locally destructive, with
    (in tongue the early ingrowth into the blood vessels and                 relapses – the adequate surgical extirpation with the
    metastasis to the lung can be found)                                     histopathological examination of the borders is necessary
    may exulcerate
    The endofytic form does exist!

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Tumors of salivary glands                                                    Tumors of salivary glands

    relatively rare tumors with an incidence 1-3/100.000                         Benign tumors
    Affect in 70% the major glands (parotid gland 70% - benign,                      Pleomorfic adenoma
    sublingual 70% - malignant)                                                      Monomorfic adenoma
    in 30% affect small glands                                                       Adenolymphoma
    Histomorfologically highly variable appearance (glands, ducts -
    Histomorfologically                                                          Malignant tumors
    basal and reserve cells, myoepithelial cells)                                    Mukoepidermoid carcinoma
                                                                                     Adenocystic carcinoma (cylindroma)
                                                                                     Carcinoma from acinous cells

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   Pleomorphic adenoma
                                                                              Monomorphic adenoma
        most common salivary gland tumor, usually affects
                   women, 20-
        parotid of women, 20-60 r                                              The group of benign tumors formed by proliferation of epithelial
             growth, painless,
        Slow growth, painless, face deformating                                cells of one type with a characteristic arrangement
        unfixed, circumscribed, nodular, soft consistancy
        unfixed, circumscribed, nodular,                                       Properties similar to PA, less recidivism

                                                                            bas ocellular
                                                                            basocellular adenoma
        encapsulates proliferation (fibrous capsule)
                                                                              nests of tumor cells separated by fibrous stroma
        ductal and myoepithelial cells supported by stroma with
                                                                              in the centre are the cuboidal cells resembling strat.basale of the
        mucoid, myxoid or chondroid appearance                                epidermus
        Myoepithelial cells and epithelial cells form areas or              canalicular adenoma
        sheets, may be rarely cribriform or tubular                         •  anastomosing
                                                                               anastomosing sheets forming the canalicules – formed by a layer of
                                                                               cylindrical to cuboidal cells
                     capsule, recurrency,
        growing into capsule, recurrency, possible malignant                •  myxomatous
                                                                               myxomatous hypocellular stroma with dilated capillaries
        variant – the biological behaviour is difficult to analyze
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  Adenolymphoma (Warthin's Tumor, Papillary Cystadenoma
  Lymphomatosum)                                                                                  Oncocytoma)
                                                                               Oxyphilic adenoma (Oncocytoma)

        benign, slowly growing tumor
        lower pole of the parotid, accounting 2-10% of all SGT
                                                                                     rare benign tumours
        males, over 50y                                                              predominantly tumours of those over middle age, women, usually in the
        unfixed, circumscribed
        unfixed,                                                                     seventh or eight decade
                                                                                     parotid glands
        On palpation a solid, painless, smooth surface, elastic,
                                                                                     slow-growing and may rarely be bilatera.
                                                                                          circumscribed, red-
                                                                                     Well circumscribed, red-brown color
        Papillary proliferation (epithelial part) with inner                         From large oxyphilic cells with granular cytoplasm – oncocytes
        and outer layer of epithelial cells
        lymphoid stroma organized in follicules

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                                                                                      Mucoepidermoid Carcinoma
Tumors of salivary glands

                                                                                      most common, form 4.8% of all salivary gland tumours, most common
    Benign tumors
                                                                                      types of post-irradiation salivary gland tumours
         Pleomorfic adenoma
                                                                                      parotid, peak age incidence is in the fifth decade.
         Monomorfic adenoma
                                                                                      locally invasive growth, destructive, metastases, often quickly, painfully
                                                                                      less malignant variants – recurrency
    Malignant tumors
         Mukoepidermoid carcinoma
                                                                                      Well differentiated adenocarcinoma G1
         Adenocystic carcinoma (cylindroma)                                                                                          mucus-
                                                                                           consists of squamous epithelial cells and mucus-producing cells
         Adenocarcinoma                                                                                               mucin-
                                                                                           cystic formations lined by mucin-producing cells
         Carcinoma from acinous cells                                                                squamous-
                                                                                           sheets of squamous-epithelial cells
                                                                                      Poorly differentiated adenocarcinoma G3
                                                                                           consists predominantly from epithelial squamous cell
                                                                                           w ithout cystic formations, pleomorphic cells

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                                                                                      Acinic cell carcinoma
   Adenoid Cystic Carcinoma ('Cylindroma')

                                                                                       Arises from acinic cells
        More common in minor salivary glands, the palate is by far the most
                                                                                       Parotide, women
        peak age incidence is in the sixth decade (range 12-72 years) and the
        female to male ratio is 1.3
                                                                                       Low-      malignancy
                                                                                       Low-grade malignancy, but often recurs
        Slow growth, but painful and facial nerve palsy
                                                                                       Well-circumscribed, unfixed
        late appearance of recurrences, but frequent, metastases, worse
        Perineural dissemination - PAIN                                                Cells:                   seromucinous
                                                                                       Cells: mucinous, serous, seromucinous
                             gray-      color,
        Poorly encapsulated, gray-white color, growing into the surrounding                                                         papillary-
                                                                                       in different structures (solid, mikrocystic, papillary-cystic,
  Typical cribriform adenoid cystic carcinomas                                         Cells with abundant basophilic granular cytoplasm
                                                                                       Cells with vacuolated cytoplasm
        Consists of cylindrical cavities filled with hyaline masses and
                                cavities                                               Eosinophilic cells with centrally located nucleus
        connective tissue with capillaries
        Myoepith elial
        Myoepithelial cells

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