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Home , Cysts of the jaws, Nasolabial cyst

07/01/2012

Lecture Objectives

CYSTS OF At the end of this lecture you should be able to: THE JAWS & • Define & classify of the jaws & oral cavity • Describe pathogenesis, features, differential THE ORAL diagnosis and management of cysts in general CAVITY • Explain the following of common cysts – Aetiopathogenesis – Clinical features – Radiographic appearance (if present) – r v subramanyam – Management [email protected] 2 18 Aug 2009

Definitions Definitions

• Etymology: Derived • True cysts from Greek “Kystis” – Radicular for sac, bladder, pouch, bag from – “Kyso” I hold – • Pathological cavity or • Pseudocysts pouch, containing – Aneurysmal fluid or semi-fluid cyst material, and which may or may not be – Solitary bone cyst lined by epithelium. – Mucus escape phenomenon 3 4 18 Aug 2009 18 Aug 2009

CLASSIFICATION CLASSIFICATION

Developmental I. b) INFLAMMATORY A. Epithelial-lined cysts i. Radicular cyst, apical and SINUS lateral Odontogenic 1. ODONTOGENIC ii. Residual cyst Inflammatory a) DEVELOPMENTAL iii. Paradental cyst and juvenile Epithelial‐ i. Dentigerous cyst paradental cyst lined ii. iv. Inflammatory collateral cyst Non‐ iii. Eruption cyst 2. NON-ODONTOGENIC CYSTS JAWS odontogenic iv. of infants (Developmental) i. Midpalatal raphé cyst of infants So‐called v. Gingival cyst of adults Non‐Epithelial‐ vi. Developmental lateral ii. Fissural cysts periodontal cyst lined iii. vii. Botryoid B. Non-Epithelial-lined cysts SOFT viii. Glandular odontogenic cyst 1. Aneurysmal bone cyst TISSUES ix. Calcifying odontogenic cyst 2. Solitary bone cyst

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CLASSIFICATION Frequency of occurrence

II. Cysts associated Maxillary 5. Oral cysts with gastric or 1. Radicular cyst Antrum intestinal epithelium 2. Dentigerous cyst 1. Mucocele 6. 3. Odontogenic keratocyst 2. Retention cyst 7. Nasopharyngeal cyst 4. Nasopalatine duct cyst 3. Pseudocyst 8. Thymic cyst 4. Postoperative maxillary 9. Cysts of the salivary glands: 5. Paradental cyst cyst Developmental origin mucous extravasation cyst; 6. Solitary bone cyst III. Cysts of the soft tissues of mucous retention cyst; ; 7. Calcifying cystic odontogenic tumour the mouth, face and neck polycystic (dysgenetic) 1. Dermoid & epidermoid cysts disease of the parotid 8. Eruption cyst 2. Lymphoepithelial 10. Parasitic cysts: hydatid cyst; 9. Developmental (branchial) cyst Cysticercus cellulosae; 10. Nasolabial cyst 3. Thyroglossal duct cyst trichinosis 11. Gingival cyst of adults 4. Anterior median lingual cyst 12. Aneurysmal bone cyst 7 8 18 Aug 2009 18 Aug 2009

Pathogenesis of true cysts Sources of epithelial cells

Dental lamina Cell rests of Serres

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Sources of epithelial cells Sources of epithelial cells

Tooth primordium Cell rests of Malassez Cell rests at the line of Sinus lining fusion

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Aetiopathogenesis of Odontogenic Cysts Aetiopathogenesis of true cysts

CYST OE DL DO COM REE PHASE OF INITIATION . Genetic ODONTOGENIC KERATOCYST  . Immunological DENTIGEROUS CYST & ERUPTION CYST  . Inflammatory . Local factors –  O & GINGIVAL CYST OF INFANTS  2 CO2 tension GINGIVAL CYST OF ADULTS  PHASE OF CYST FORMATION LATERAL PERIODONTAL CYST (Dev)  . Death & degeneration RADICULAR CYST / RESIDUAL CYST  of central cells due to  vascularity KEY: OE –ODONTOGENIC EPITHELIUM; DL –DENTAL LAMINA; DO –DENTAL ORGAN; COM –CELL RESTS OF MALASSEZ; REE – REDUCED ENAMEL EPITHELIUM PHASE OF CYST ENLARGEMENT 13 14 18 Aug 2009 18 Aug 2009

Aetiopathogenesis of true cysts Factors involved in the growth of the cysts

• Attraction of fluid into the cyst cavity  Retention of fluid within the cavity  Raised internal hydrostatic pressure • Bone resorption with increased size of bone cavity – MMPs 1, 2, 3 and Prostaglandins E2 & E3 • Epithelial proliferation

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General Features of Jaw Cysts General Features of Jaw Cysts

• Slowly enlarging swelling. • Usually painless, unless secondarily infected or impinging on a nerve. • Normally only buccal cortical plate expansion, and not lingual. • "Eggshell crackling" on palpation of large cysts. • Occasionally pathological fracture. • Displacement of adjacent teeth may occur. • Sometimes resorption of roots of adjacent teeth. • Well-circumscribed radioloucency, unilocular (occasionally multilocular) with a radiopaque border. 17 18 18 Aug 2009 18 Aug 2009

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General Features of Soft Tissue Cysts General Features of Cysts

• Slowly enlarging swelling • All true cysts show • Painless usually – a lumen – an epithelial lining, • Fluctuant on palpation and – a connective tissue wall

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ASPIRATION Differential Diagnosis

CONTENT CYST Radiolucent cyst-like features can be seen in

Whitish, pale, cheesy material – keratin • Odontogenic tumours Keratocyst flakes; protein level < 4 mg/100 ml • Non-odontogenic tumours Light straw coloured fluid Dentigerous cyst • Giant cell granuloma

Shimmering straw colour – cholesterol • Hyperparathyroidism Radicular cyst crystals; protein level > 5 mg/100 ml • Serosanguinous - pus Infected cyst • Stafne bone cavity Red - blood Aneurysmal bone cyst • Normal anatomic structures (antrum, incisive canal fossa) Nothing on aspiration Solitary bone cyst 21 22 18 Aug 2009 18 Aug 2009

Management of Cysts Cysts that will be discussed…

1. Enucleation 1. Radicular Cyst Separation of the from the bone, with preservation of bone continuity 2. Dentigerous Cyst 2. Curettage 3. Odontogenic keratocyst (Kerato- Removal by scraping or morcellation odontogenic tumour) 3. Marsupialization 4. Gorlin Cyst Surgical removal of overlying tissue, creating a window in the wall of the cyst  decompresses cyst & s intra-cystic 5. Gingival cyst of infants / Epstein’s Pearls pressure  promotes cyst shrinkage & bone fill 6. Mucocoeles 4. Resection without continuity defect 7. Fissural Cysts 5. Resection with continuity defect 6. Disarticulation 8. Aneurysmal Bone Cyst 7. Recontouring 9. Solitary Bone Cyst 23 24 18 Aug 2009 18 Aug 2009

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RADICULAR CYST Radicular Cyst: Demographics

• Synonyms: Dental Cyst, Apical Cyst, • Most common jaw cyst – nearly 60% of all jaw • Most common cyst of the jaws, which is of inflammatory origin cysts and arises from the epithelial cell rests of Malassez, as a result of • Age predilection – 3rd to 5th decade following death and necrosis of the . • Residual Cyst: Radicular cyst that remains behind in the jaws after • Gender predilection – more in males removal of the offending tooth. – Girls less likely to neglect their teeth • Inflammatory Collateral Cyst: Inflammatory cysts that occur at the – Boys more likely to sustain trauma cervical margin of the lateral aspect of a root as a consequence of an inflammatory process in a periodontal pocket. Main (1970) • Site predilection – more in maxillary anterior • Paradental Cyst: Cyst of inflammatory origin occurring on the region lateral aspects of the roots of partially erupted mandibular third – high prevalence of palatal invaginations in the molars with an associated history of . Craig (1976) maxillary lateral incisors • Mandibular Infected Buccal Cyst: Cyst of inflammatory origin – >> placement of silicate restorations occurring on the buccal aspects of mandibular molars in young – teeth are more prone to traumatic injuries children. Stoneman and Worth (1983) 25 26 18 Aug 2009 18 Aug 2009

Radicular Cyst: Clinical features Radicular Cyst: Radiographic features

• Usually asymptomatic Radicular cyst DD from Periapical • Discovered when periapical radiographs are Granuloma taken for swelling associated with non-vital teeth • < 5 mm more likely to be • slowly enlarging swellings PA granuloma – – buccal or palatal • > 2 cm more likely to be – – buccal or labial, but rarely lingual PA cyst • Large cysts – may show egg shell crackling Due to many • Pain ± cysts have diffuse margin & lack circumscribed • Aspiration or sinus formation: straw coloured appearance usually fluid that is rich in cholesterol crystals ascribed to radicular cysts

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Periapical – Radiographic appearances Other radiographic appearances

A –Normal Bay Cyst Radicular cyst in dens in B – widening of PDL dente space – acute apical periodontitis C – Loss of Lamina dura – Early apical abscess D – Diffuse ill- defined margins – Apical abscess E-Diffuse radiopaque area – condensing osteitis F- Apical granuloma or cyst 29 30 18 Aug 2009 18 Aug 2009

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Radicular Cyst: Radiographic features Radicular Cyst: Aetiopathogenesis

Residual cyst Residual cyst Dental Caries Trauma + pulp • The lesion is at the site exposure of a previously Deep extracted tooth. periodontal • DD: The lesion must be pocket differentiated from a Periapical granuloma / keratocyst abscess

PERIAPICAL CYST Residual cyst Lateral cyst 31 32 18 Aug 2009 18 Aug 2009

Radicular Cyst: Pathogenesis Radicular cyst: Phase of Initiation

• Phase of Initiation Possible factors that cause proliferation of – sources of epithelium epithelial cells • Cell rests of Malassez • Bacterial endotoxins from the dead pulp • Maxillary sinus lining • inflammatory cytokines – IL-1 & IL-6,

• Fistulous tract • Reduced O2 tension • Periodontal pocket • Increased CO2 tension • Phase of Cyst Formation • Reduced pH • Phase of Cyst Enlargement • Loss of immunological surveillance • Genetic predilection 33 34 18 Aug 2009 18 Aug 2009

Radicular cyst: Phase of Cyst formation Radicular cyst: Phase of Cyst Enlargement

Two possibilities: • ed Hydrostatic Pressure – due to lytic • epithelium proliferates products of epithelial & inflammatory andcovers the bare connective tissue cells in the cyst cavity osmotic surface of a PA pressure of the cyst fluid abscess cavity • cyst cavity forms • Epithelial cell proliferation within a proliferating • Bone resorption  due to PG E2 & E3,, IL- epithelial mass in PA granuloma by 1 & IL-6  intra-osseous cyst expansion degeneration and • Collagenase production by cyst walls – death of cells in the centre MMPs 1, 2, 8, 9, & 13 35 36 18 Aug 2009 18 Aug 2009

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Radicular cyst: Histopathology Radicular Cyst: Histopathology

EPITHELIAL LINING Arcading epithelial lining Arcading epithelial lining • stratified squamous –high power epithelium in an arcading pattern • pseudostratified ciliated columnar if assoc. with maxillary sinus • may be hyperplastic or discontinuous, especially over areas of inflammation • mucous cells and ciliated cells may be seen 37 38 18 Aug 2009 18 Aug 2009

Radicular Cyst: Histopathology Radicular Cyst: Histopathology

Ciliated epithelium in a Mucous cells in epithelial Rushton bodies radicular cyst lining of a radicular cyst • Described 1st by Dewey and Lund • Details by Rushton in 1955 • Rounded, elliptical or hairpin shaped hyaline bodies; central eosinophilic granular area • Origin – Odontogenic epithelium – Haematogenous – NOT keratin 39 40 18 Aug 2009 18 Aug 2009

Radicular cyst: Histopathology Radicular Cyst: Histopathology

Cholesterol clefts + giant Cholesterol clefts + giant CONNECTIVE TISSUE cells + haemosiderin CAPSULE cells pigments • bundles of collagen • inflammatory cell infiltrate • cholesterol clefts • Giant cells & foam cells • foci of haemosiderin deposits 41 42 18 Aug 2009 18 Aug 2009

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Radicular Cyst: Management Dentigerous Cyst

• majority of radicular cysts undergo • Synonyms: Follicular cyst resolution after RCT • The term was coined by Paget in 1863 for • Enucleation – in case of larger cysts cysts of the jaws containing teeth. • Most common developmental odontogenic cyst • Defined as developmental odontogenic cyst which is attached to the cervical portion of an unerupted or impacted tooth.

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Dentigerous cyst: Aetiopathogenesis Dentigerous cyst: Aetiopathogenesis

• Follicular theory of origin • Extrafollicular theory of origin

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Dentigerous cyst: Demographics Dentigerous cyst: Clinical Features

• 2nd most common cyst. • Asymptomatic • Most common developmental • A tooth is usually odontogenic cyst missing from the arch • Age predilection: 2nd to 3rd decade • Slowly growing • Gender predilection: Males >Females swelling • Site predilection: Mandibular 3rd ; • Egg shell crackling Max 3rd molar; Maxillary canine may be elicited

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Dentigerous cyst: Radiological Features Radiographic DD

• Unilocular or multilocular radiolucency around • Dilated dental the crown of an unerupted tooth follicle • central – If width is >>3 mm, • lateral then cyst • circumferential/envelopmental • AOT, Am , Unicystic

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Dentigerous cyst: Radiological Features Dentigerous cyst: Radiological Features

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Dentigerous cyst: Histopathology Dentigerous cyst: Histopathology

• Lining – Stratified squamous epithelium which is usually non- keratinised – Thin lining (3-4 layers) with flattened or cuboidal cells resembling the reduced enamel epithelium – mucous metaplasia may be seen – Flat epithelial-connective tissue junction; rete pegs absent • Capsule – The connective tissue capsule is thin, composed of cellular fibrous tissue, with few inflammatory cells – Cholesterol clefts – if secondarily infected 53 54 18 Aug 2009 18 Aug 2009

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Dentigerous cyst: Histopathology Dentigerous cyst: Complications

• Ameloblastoma • Squamous cell carcinoma • Mucoepidermoid Carcinomas

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Dentigerous cyst: Management Eruption Cyst

• Enucleation + removal of impacted tooth • Also called Eruption • Marsupialization haematoma or blue- domed cyst of Seward • Radiation to be avoided, as it may • Soft tissue counterpart cause malignant transformation of dentigerous cyst in the gingiva above an erupting tooth • lined by non- keratinizing stratified squamous epithelium

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Eruption Cyst Developmental Lateral Periodontal Cyst

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Developmental Lateral Periodontal Cyst Comparison

GINGIVAL CYST OF ADULTS LAT PERIODONTAL CYST Age 5th and 6th decades 5th to 7th decades Gender >> Females Equal Site Mandible –premolar‐canine Mandibular premolar area; region Anterior maxilla Clinical Gingiva –facial aspect, Gingival swelling ±pain; Assoc attached or interdental papilla teeth vital Radiological None (soft tissue) lesion or Well‐circumscribed unilocular faint round shadow radiolucency with radiopaque margin, between apex & cervical margin

Pathogenesis REE – after tooth eruption REE – before tooth eruption 62 18 Aug 2009

Botryoid Odontogenic Cyst Odontogenic Keratocyst

• The term ‘odontogenic keratocyst’ was introduced by Philipsen (1956) • Keratocyst by Pindborg and Hansen (1963) • Also called “Primordial cyst” in 1960s • Defined as developmental odontogenic cyst which is distinguished by its characteristic histological features, high rate of recurrence and aggressive behaviour. • Only cyst that can be diagnosed solely by its histological appearance . 63 64 18 Aug 2009 18 Aug 2009

New terminology Odontogenic Keratocyst: aetiopathogenesis

• Keratocystoma – Shear, 2003 Sources of epithelial lining: • Keratocystic odontogenic tumour – • Dental lamina Philipsen, 2005 • Basal layer of oral epithelium • keratinising cystic odontogenic tumour – • Cell rests of Serré Reichart & Philipsen, 2004 • Liquefaction degeneration of stellate No consensus as yet!! reticulum

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Odontogenic Keratocyst: Demographics Odontogenic Keratocyst: Clinical Features

• 3rd most common cyst; 2nd most • Asymptomatic common developmental cyst • Slowly growing swelling • Age predilection: Bimodal: 2nd to 3rd • Aspiration: cheesy material decade • Large cyst with secondary infection may • Gender predilection: Males > Females produce paraesthesia in the region • Site predilection: Mandibular 3rd molar, (Vincent's sign) angle, ramus area • ± Displacement of teeth • Race predilection: More in whites than • Maxillary OKC may extend to involve blacks; Rare in black females base of the skull 67 68 18 Aug 2009 18 Aug 2009

Naevoid Basal Cell Carcinoma Syndrome (NBCCS) Naevoid Basal Cell Carcinoma Syndrome

• Synonyms: Gorlin–Goltz syndrome, Gorlin syndrome – Gorlin & Goltz, 1960 • autosomal dominant disorder – gene mapped to chromosome 9q22.3 – PTCH gene • multiple naevoid basal cell carcinomas • jaw cysts – OKCs • bifid ribs • ectopic calcifications • plantar and palmar pits • frontal bossing • ocular hypertelorism

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Odontogenic Keratocyst: Radiological Odontogenic Keratocyst: Radiological Features Features

Usually multilocular • Replacement • Paradontal • Follicular • Radicular • Ectopic

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Odontogenic Keratocyst: Radiological Odontogenic Keratocyst: Histopathology Features

Multilocular Scalloped margins • Stratified squamous epithelium which is keratinised: either ortho or para (more common) • Corrugated surface • Flat epithelial-connective tissue junction; rete pegs absent • Basal cells tall and oriented like “tomb stones” or “picket fence” • Nuclei oriented away from basement membrane – reversal of polarity • epithelium is often detached from the capsule

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OKC – Metaphors Odontogenic Keratocyst: Histopathology

Picket Fence Tomb stones CONNECTIVE TISSUE CAPSULE • thin and collagen fibres • rich in GAGs and proteoglycans • inflammatory cell infiltrate is uncommon • presence of islands of odontogenic epithelium that have formed daughter or "Satellite" cysts

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Odontogenic Keratocyst: OKC: Histopathology

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OKC: Histopathology OKC: Histopathology

OKC with dysplastic Inflammed Odontogenic changes keratocyst

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Odontogenic Keratocyst: causes for high OKC: Histopathology recurrence rate

1. Presence of Satellite or Daughter cysts 2. Remnants of dental lamina or basal cell hamartias may form new cysts 3. Incomplete surgical removal a) Large cysts b) Surgeon’s ineptitude c) Cyst removal in several pieces d) Thin, friable lining preventing complete removal e) Detachment of lining from capsule 4. Growth into the trabecular spaces 5. High mitotic index of epithelial cells 6. Cysts with parakeratinised lining more likely to recur when compared to cysts with orthokeratinised linings 81 82 18 Aug 2009 18 Aug 2009

Odontogenic Keratocyst: causes for high Odontogenic Keratocyst: Management recurrence rate

Satellite cysts Basal cell hamartias • Enucleation + Peripheral osteoctomy • En bloc osseous resection – marginal resection that preserves continuity, or segmental resection that violates continuity • enucleation and liquid nitrogen cryotherapy or Carnoy’s solution

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Orthokeratinized OKC Orthokeratinized OKC

• Different clinico-pathologically & behaviourally • Young adult males • Posterior portions of jaws, esp mandible • Not assoc with Gorlin-Goltz syndrome • Usually unilocular on radiographs • Orthokeratinized stratified squamous epithelium with prominent granular layer • Does not have prominent palisaded basal cell layer • Treated by enucleation & curettage

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Gorlin Cyst Gorlin Cyst: Classification (Prætorius, 2006)

• Synonyms: • Group 1: ‘Simple’ cysts – ameloblastic fibro- – Calcifying odontogenic cyst – Calcifying odontogenic – Keratinizing & Calcifying Odontogenic Cyst cyst (COC) – odontomeloblastoma – Calcifying Cystic Odontogenic Tumour • Described 1st by Gorlin (1962) • Group 2: Calcifying Cystic – odontogenic myxoma Odontogenic Tumours (CCOT) • Histological resemblance to the cutaneous • Group 3: Solid benign calcifying epithelioma of Malherbe associated with: odontogenic neoplasms – • Not actually a cyst but a neoplasm with cystic – Odontome Dentinogenic ghost cell tendencies – adenomatoid tumour • Defined as an odontogenic lesion that arises from odontogenic tumour • Group 4: Malignant dental lamina which may be intraosseous or – Ameloblastoma odontogenic neoplasms – extraosseous and characterized by basal palisading – ameloblastic fibroma Ghost cell odontogenic and large masses of keratinized ghost cells. carcinoma 87 88 18 Aug 2009 18 Aug 2009

COC – Demographics COC – Clinical Features

• Incidence – less than 1% • Asymptomatic • Age predilection – 2nd decade • Swelling ± pain • Gender predilection – Equal • Lingual expansion may sometimes be • Site predilection – More in Maxilla, esp. observed canine-premolar region; Peripheral • Displacement of the teeth also anterior to molars • Extraosseous lesions – pink to red, circumscribed, elevated masses < 4cm in diam

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COC – Radiological Features COC – Radiological Features

• Radiolucent – may or may not have regular outline with well-demarcated margins • Unilocular or multilocular • Irregular radio-opacities of varying sizes may be seen in the radiolucent area • Displacement of teeth often seen • Resorption of the roots of adjacent teeth frequently observed • Extra-osseous lesions show localised superficial bone resorption 91 92 18 Aug 2009 18 Aug 2009

COC – Histopathology COC – Histopathology

• Epithelial Lining 6–8 cells thick • Presence of ghost cells • Prominent basal layer having palisaded columnar – enlarged, ballooned, ovoid or elongated or cuboidal cells and hyperchromatic nuclei elliptoid epithelial cells polarised away from the basement membrane – Eosinophilic with cell outlines are usually • Budding from the basal layer into the connective well-defined tissue seen • Epithelial proliferations into the lumen are – Only nuclear remnants or faint outline of the frequently observed original nucleus seen • An atubular dentinoid is often found in the wall – represent an abnormal type of keratinisation close to the epithelial lining – have affinity for calcification

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COC – Histopathology COC – Histopathology

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COC – Histopathology Aetiopathogenesis of Ghost cells

GHOST CELLS GHOST CELLS • Abnormal keratinization ed expression of Laminin (Gorlin, 1964) 5 in Ghost Cells. Marker • Coagulative necrosis of for CCOT. odontogenic epithelium (Hong et al, 1991) • Enamel-related proteins (amelogenin, enamelin, sheathelin etc) accumulate in cytoplasm during pathologic transformation (Takata et al, 2000) • Matrix glycoprotein Calcifications DENTINOID tenascin-C 97 98 18 Aug 2009 18 Aug 2009

COC – Treatment Gingival Cysts in the infants

• Surgical enucleation • small and white or cream coloured • Wider excision if associated with a • Seen < 3 mths • found only on the crests tumour component of maxillary & mandibular dental ridges • undergo involution and disappear, or rupture • Bohn’s nodules are mucous gland cysts found on the buccal or lingual aspects of the dental ridges 10 99 18 Aug 2009 18 Aug 2009 0

Gingival Cysts in the infants Gingival Cysts in the infants

Pathogenesis Pathology • epithelial remnants • The cysts are lined of the dental lamina by dental lamina – the glands of with the formation of Serres  proliferate, multiple areas of keratinise and form microcyst formation small cysts & keratin production

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Gingival cysts is an infant Midpalatal raphé cyst

• Also called Epstein’s pearls • Non-odontogenic origin – arise from epithelial inclusions at the line of fusion of the palatine shelves and the nasal processes • thin lining of stratified squamous epithelium with a parakeratotic surface • keratin fills the cyst cavity, • usually in concentric laminations containing flattened cell nuclei. • The basal cells are flat. 10 10 18 Aug 2009 3 18 Aug 2009 4

Midpalatal raphé cyst THANK YOU

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