INTRODUCTION

Lesions

derived from the epithelial and or mesenchymal remnants of the tooth forming apparatus Found exclusively in the mandible and maxilla ( occasionally gingiva) Originate from an aberration from the normal pattern of odontogenesis A complex group of lesions of diverse histopathologic types and clinical behavior

INTRODUCTION

Range

from hamartomatous proliferations to malignant neoplasms with metastatic capabilities Exhibit varying inductive interactions between odontogenic epithelium and odontogenic ectomesenchyme

Presentation
Asymptomatic Jaw

expansion Movement of teeth Root resorption Bone loss

Microscopy
Mimic

cell / tissue of origin Resemble the soft tissues of the enamel organ or dental pulp or may contain hard tissue elements of enamel, dentin, & or cementum

CLASSIFICATION
WHO

(1992)

ODONTOGENIC TUMORS

BENIGN

MALIGNANT

MODIFIED WHO CLASSIFICATION

BENIGN I Odontogenic epithelium without odontogenic ectomesenchyme
Ameloblastoma Squamous

odontogenic tumor Calcifying epithelial odontogenic tumor Adenomatoid odontogenic tumor

CLASSIFICATION

II Odontogenic epithelium with odontogenic ectomesenchyme with or without hard tissue formation
Ameloblastic fibroma Ameloblastic fibrodentinoma Ameloblastic fibro-odontoma Odontoameloblastoma Calcifying odontogenic cyst Complex odontoma Compound odontoma

CLASSIFICATION

III Odontogenic ectomesenchyme with or without included odontogenic epithelium

Odontogenic

fibroma Myxoma (myxofibroma) Cementoblastoma (benign cementoblastoma, true cementoma)

CLASSIFICATION

B. MALIGNANT I Odontogenic Carcinomas

Malignant

ameloblastoma Primary intraosseous carcinoma Clear cell odontogenic carcinoma Ghost cell odontogenic carcinoma

II Odontogenic Sarcomas
Ameloblastic

fibrosarcoma Ameloblastic fibro dentinosarcoma Ameloblastic fibro odontosarcoma

2005 WHO HISTOLOGICAL CLASSIFICATION OF ODONTOGENIC TUMOURS

BENIGN TUMOURS
1. Odontogenic epithelium with mature, fibrous stroma without odontogenic ectomesenchyme Ameloblastoma,
solid

/ multicystic type extraosseous / peripheral type desmoplastic type unicystic type

Squamous

odontogenic tumour Calcifying epithelial odontogenic tumour Adenomatoid odontogenic tumour Keratocystic odontogenic tumour

2. Odontogenic epithelium with odontogenic ectomesenchyme,with or without hard tissue formation Ameloblastic fibroma Ameloblastic fibrodentinoma Ameloblastic fibro-odontoma Complex Odontoma Compound Odontoma Odontoameloblastoma Calcifying cystic odontogenic tumour Dentinogenic ghost cell tumour

3. Mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium Odontogenic fibroma Odontogenic myxoma / myxofibroma Cementoblastoma

MALIGNANT TUMOURS
1. Odontogenic carcinomas Metastasizing (malignant) ameloblastoma Ameloblastic carcinoma Primary intraosseous squamous cell carcinoma Clear cell odontogenic carcinoma Ghost cell odontogenic carcinoma

2. Odontogenic sarcomas Ameloblastic fibrosarcoma Ameloblastic fibrodentino and fibro-odontosarcoma

AMELOBLASTOMA
Introduction Types Incidence Clinical

& definition

features Radiographic features Histogenesis Histopathology subtypes Treatment and Prognosis

UNICYSTIC AMELOBLASTOMA Peripheral Ameloblastoma Pitutary Ameloblastoma Adamntinoma of

long bones

AMELOBLASTOMA
SYNONYMS Adamantinoma Adamantoblastoma Multilocular cyst

Definition (Robinson)
It

is a tumor of odontogenic origin (derived from enamel organ type tissue which does not undergo differentiation to the point of enamel formation) usually unicentric, nonfunctional, intermittent in growth, anatomically benign and clinically persistent and Churchill ameloblastoma (1934)

Ivy

Types
Certain

behaviour patterns, anatomic locations, histologic and radiologic features

Ameloblastoma

Central (Intraosseous)

Peripheral (Extraosseous)

Solid / multicystic (86%)

Unicystic (13%)

Desmoplastic

Incidence
Common

odontogenic tumor 1% of all tumors and cysts of jaws 18 % of odontogenic neoplasms

Conventional Solid / multicystic Intraosseous Ameloblastoma

Most

frequently encountered Demographics : Age :

wide age range 3-7th decades of life

Sex

: no gender predilection Race : blacks

Clinical features
Site : 85 % - mandible molar ascending ramus area 15 % - maxilla posterior region Usually asymptomatic Pain & paresthesia uncommon Smaller lesions only on routine R/F

painless swelling / expansion of the jaw ; tends to expand the bone rather than perforate it Very thin bone EGG SHELL CRACKLING on palpation Tooth mobility Unhealed extraction sites aggressive

Maxilla Facial swelling, nasal obstruction Sinus involvement, extension into the orbit or nasopharynx Sinusitis, pre auricular pain Foul smelling discharge

Radiographic features
Often

multilocular radiolucent lesion Rarely unilocular Large loculations soap bubble appearance Small loculations honey combed appearance

Radiographic features

Buccal

& lingual cortical expansion and thinning Resorption of roots Displacement of teeth Association with an unerupted tooth (mandibular 3rd molar) Irregular scalloping of the margins of R/L lesions Sinus antral clouding / opacity

Radiographic features

Differential diagnosis OKC ABC Central hemangioma Brown tumor

HISTOGENESIS
Tumor may be derived from Cell rests of enamel organ Cell rests of Serre Cell rests of Malassez Epithelium of Odontogenic cysts Disturbances in developing enamel organ Basal cells of surface epithelium Heterotopic epithelium in other parts of the body (extragnathic ameloblastoma)
E.g. pituitary gland and long bones

Pathology
Macroscopy / gross Grayish white or grayish yellow cylindrical or fusiform mass Small or large cystic spaces

Microscopy (h/p)
6 subtypes / variants Follicular Plexiform Acantomatous Granular cell Basal cell Desmoplastic

Microscopy

Follicular pattern
Most

common and recognizable pattern Discrete islands or follicles of epithelial cells in a mature connective tissue stroma Epithelial islands resemble enamel organ of the developing tooth germ ; :

Consist of:
Peripheral

cells (ameloblast like cells) basal cells with hyperchromatic

Coumnar

nuclei
Nuclear

palisading with polarization vacuolation

Cytoplasmic Central

cells loosely arranged and resemble stellate reticulum

Microscopy

Cyst

formation

common Microcyst large macroscopic cysts

Hyalinization

around the follicles because of induction phenomenon

Stroma Mature fibrous connective tissue in variable amounts Plentiful or very minimal

Microscopy

Plexiform pattern Epithelium Long, anastomosing cords or larger sheets bounded by single layer columnar or cuboidal ameloblast like cells surrounding more loosely arranged epithelial cells Stroma Loosely arranged and vascular Cyst formation -- uncommon

Microscopy

Acanthomatous pattern Extensive squamous metaplasia, often with keratin formation in the central portions of a follicle Mistaken for
Squamous

cell carcinoma Squamous odontogenic tumor

Microscopy

Granular cell pattern Transformation of groups of lesional epithelial cells to granular cells with abundant cytoplasm filled with eosinophilic granules Cells round / polyhedral Small, darkly staining nucleus Granules similar to lysosomes (U/S & H/C) Higher recurrence rate

Microscopy

Basal cell pattern Least common type Nests of uniform basaloid cells H/p similar to BCC of skin No stellate reticulum in the central portions of the nests Peripheral cells cuboidal rather than columnar

Desmoplastic ameloblastoma
Eversole

et al, 1984 as a separate type:

Considered
Different

clinical features Different radiological features Different histopathological features

Desmoplastic ameloblastoma
Desmoplasia

extensive stromal collagenization( hyalinization) Hypocellular Tendency to grow in thin strands and cords of epi rather than in an island like patteren. Epithelium compressed and fragmented Scant central cells with peripheral flat cells. Site : maxilla > mandible Histogenesis : cell rests of Malassez

RADIOLOGICAL FEATURES
Mixed

radiolucent & radio-opaque

lesion Unilocular or Multilocular Borders poorly defined

DIFFERENTIAL
Fibro-osseous

DIAGNOSIS:
lesions

HISTOPATHOLOGY
STROMA:
Desmoplasia Thick

collagen bundles squeeze the epithelial islands New bone formation

HISTOPATHOLOGY
ODONTOGENIC
Islands

EPITHELIUM:

compressed by the collagen bundles (ANIMAL-LIKE; KITE-LIKE) Peripheral cells: cuboidal No ameloblast-like cells Central cells: spindle / polygonal No stellate reticulum-like cells

Treatment and prognosis

A

variety of treatment modalities Simple enucleation and curettage to en bloc resection Curettage higher recurrence rate (50 90 %) Marginal / bloc resection most widely used Recurrence rate -- 15 %

Treatment and prognosis

Conventional
A

ameloblastoma

persistent, infiltrative neoplasm Progressive spread to vital structures Death

Many

of these tumors not life threatening lesions rarely frank malignant behavior

UNICYSTIC AMELOBLASTOMA
A

unilocular cystic lesion whose clinical features resemble those of a non neoplastic cyst A distinct entity based on clinical, radiographic & pathologic features and its response to treatment 10 15% of all intraosseous ameloblastomas

UNICYSTIC AMELOBLASTOMA

Clinical features Age :

Younger

patients 2nd decade

Sex

: no predilection Site : posterior mandible (90 % cases) Presentation :

Asymptomatic Large

lesions painless swelling of the jaws

UNICYSTIC AMELOBLASTOMA

Radiographic features May or may not be associated with an impacted tooth Well defined radiolucencies ; may or may not be demarcated by a perilesional corticated rim

UNICYSTIC AMELOBLASTOMA

Histogenesis Proposed theories Cystic degeneration of solid ameloblastomas Ameloblastomatous change in an preexisting cyst Co existence of non-neoplastic and neoplastic epithelium De novo

UNICYSTIC AMELOBLASTOMA

Association with a cyst Most common -- dentigerous cyst Other cysts - Parakeratinized Radicular

OKC

cyst Residual cyst COC GOC

UNICYSTIC AMELOBLASTOMA

Histopathology 3 distinct patterns 1. Luminal 2. Intraluminal 3. Mural

UNICYSTIC AMELOBLASTOMA

Luminal Unilocular cystic lesion lined by epithelium (basal cells VICKERS-GORLIN CRITERIA) No infiltrating neoplastic epithelium Tumor confined to luminal surface of the cyst luminal unicystic ameloblastoma Cells overlying the basal layer loosely cohesive and resemble stellate reticulum

VICKERS-GORLIN CRITERIA
Columnar

basal cells with hyperchromatic nuclei palisading with polarization

Nuclear

Cytoplasmic

vacuolation with intercellular spacing Subepithelial hyalanization

UNICYSTIC AMELOBLASTOMA

Intraluminal Unilocular cystic lesion in which a nodule arises from the epithelium and projects into the lumen of the cyst Nodules odontogenic epithelium that may sometimes resemble plexiform ameloblastoma Part of the lining V & G criteria No infiltration into the cyst wall

UNICYSTIC AMELOBLASTOMA

Mural Unilocular cystic lesion islands of ameloblastomatous epithelium (follicular / plexiform) in the fibrous cyst wall May or may not be connected to the lining Part of cystic lining V & G criteria
Mural

+ Intraluminal prolierations

UNICYSTIC AMELOBLASTOMA

DIAGNOSIS OF UA ONLY AFTER MICROSCOPIC EXAMINATION Treatment and Prognosis Luminal & intraluminal variants conservative approach Mural -- radical resection Recurrence rates 10 - 20% after enucleation & curettage