PDQ ORAL DISEASE: 2012

Metabolic and Genetic Disorders

Bilezikian JP, Silverberg SJ. Clinical spectrum of primary hyperparathyroidism. Rev Endocr Metab Disord 2000;1:237–45.
Danesh F, Ho LT. Dialysis-related amyloidosis: history and clinical manifestations. Semin Dial 2001;14:80–5.
Garcia RI, Henshaw MM, Krall EA. Relationship between periodontal disease and systemic health. Periodontol 2000 2001;25:21–36.
Hendy GN. Molecular mechanisms of primary hyperparathyroiodism. Rev Endocr Metab Disord 2000;1:297–305.

Malignant Nonodontogenic Tumors

Antunes NL, Gorlick R, Callaja E, Lis E. Numb chin syndrome in Ewing sarcoma. J Pediatr Hematol Oncol 2000;22:521–3.
August M, Magennis P, Dewitt D. Osteogenic sarcoma of the jaws: factors influencing prognosis. Int J Oral Maxillofac Surg 1997;26:198–204.

Tooth Abnormalities

Aldred MJ, Crawford PJ. Molecular biology of hereditary enamel defects. Ciba Found Symp 1997;205:200–5.
Anavi Y, Kaplinsky C, Calderon S, Zaizov R. Head, neck, and maxillofacial childhood Burkitt’s lymphoma: a retrospective analysis of 31 patients. J Oral Maxillofac Surg 1990;48:708–13.
Ansari G, Reid JS. Dentinal dysplasia type I: review of the literature and report of a family. ASDC J Dent Child 1997;64:429–34.

Inflammatory Diseases

Additional Reading 365
Aitasalo K, Niinikoski J, Grenman R, Virolainen E. A modified protocol for early treatment of osteomyelitis and osteoradionecrosis of the mandible. Head Neck 1998;20:411–7.
Blinder D, Yahatom R, Taicher S. Oral manifestations of sarcoidosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:458–61.

Benign Nonodontogenic Tumors

Al-Ammar AY, Tewfik TL, Bond M, Schloss MD. Langerhans’ cell histiocytosis: paediatric head and neck study. J Otolaryngol 1999;28: 266–72.
Horning GM, Cohen ME, Neils TA. Buccal alveolar exostoses: prevalence, characteristics, and evidence for buttressing bone formation. J Periodontol 2000;71:1032–42.

Odontogenic Tumors

Barker BF. Odontogenic myxoma. Semin Diagn Pathol 1999;16:297–301. Cross JJ, Pilkington RJ, Antoun NM, Adlam DM. Value of computed
tomography and magnetic resonance imaging in the treatment of a calcifying epithelial odontogenic (Pindborg) tumour. Br J Oral Maxillofac Surg 2000;38:154–7.
Dunlap CL. Odontogenic fibroma. Semin Diagn Pathol 1999;16:293–6. Houston GD, Fowler CB. Extraosseous calcifying epithelial odontogenic

Cysts

Aguilo L, Cibrian R, Bagan JV, Gandia JL. Eruption cysts: retrospective clinical study of 36 cases. ASDC J Dent Child 1998;65:102–6.
Barreto DC, De Marco L, Castro WH, Gomez RS. Glandular odontogenic cyst: absence of PTCH gene mutation. J Oral Pathol Med 2001;30:125–8.

Lymphoid Lesions

Buchner A, Hansen LS. Lymphoepithelial cysts of the oral cavity. A clinicopathologic study of thirty-eight cases. Oral Surg Oral Med Oral Pathol 1980;50:441–9.
Cruchley AT, Williams DM, Niedobitek G, Young LS. Epstein-Barr virus: biology and disease. Oral Dis 1997;3 Suppl 1:S156–63.
Kanis JA, McCloskey EV. Bisphosphonates in multiple myeloma. Cancer 2000;88(12 Suppl):3022–32.
Kirita T, Ohgi K, Shimooka H, et al. Primary non-Ho

Salivary Gland Diseases

Biasi D, Caramaschi P, Ambrosetti A, et al. Mucosa-associated lymphoid tissue lymphoma of the salivary glands occurring in patients affected by Sjogren’s syndrome: report of 6 cases. Acta Haematol 2001;105:83–8.
Daniels TE. Evaluation, differential diagnosis, and treatment of xerostomia. J Rheumatol Suppl 2000;61:6–10.
Esch TR. Pathogenetic factors in Sjogren’s syndrome:

Connective Tissue Lesions

Adornato MC, Paticoff KA. Intralesional corticosteroids injection for treatment of central giant-cell granuloma. J Am Dent Assoc 2001;132: 186–90.
Antoniades DZ, Belazi M, Papanayiotou P. Concurrence of torus palatinus with palatal and buccal exostosis: case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85:552–7.

Verrucal-Papillary Lesions

Batsakis JG, Suarez P, el-Naggar AK. Proliferative verrucous leukoplakia and its related lesions. Oral Oncol 1999;35:354–9.
Beham A, Regauer S, Soyer HP, Beham-Schmid C. Keratoacanthoma: a clinically distinct variant of well differentiated squamous cell carcinoma. Adv Anat Pathol 1998;5:269–80.

Pigmentary Disorders

Barker B, Carpenter WM, Daniels TE, et al. Oral mucosal melanomas: the WESTOP Banff workshop proceedings. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:672–9.
Birek C, Main JHP. Two cases of oral pigmentation associated with quinidine therapy. Oral Surg Oral Med Oral Pathol 1988;66:59–61.
Buchner A, Hansen L. Amalgam pigmentation (amalgam tattoo) of the oral mucosa: a clinicopathologic study of 268 cases. Oral Surg Oral Med Oral Pathol 1980;49:139–47.

Additional Reading White Lesions

Additional Reading
White Lesions
Appleton SS. Candidiasis: pathogenesis, clinical characteristics, and treatment. J Calif Dent Assoc 2000;28:942–8.
Axell T, Pindborg JJ, Smith CJ, van der Waal I. Oral white lesions with special reference to precancerous and tobacco-related lesions: conclusions of an international symposium held in Uppsala, Sweden, May 18–21, 1994. International Collaborative Group on Oral White Lesions. J Oral Pathol Med 1996;25:49–54.

Ulcerative Conditions

Bentzen SM, Ruifrok AC, Thames HD. Repair capacity and kinetics for human mucosa and epithelial tumors in the head and neck: clinical data on the effect of changing the time interval between multiple fractions. Radiother Oncol 1996;38:89–101.
Califano J, van der Reit P, Clayman G, et al. A genetic progression model for head and neck cancer: implications for field cancerization. Cancer Res 1996;56:2488–92.
Callen JP. Oral manifestations of collagen vascular disease. Semin Cutan Med Surg 1997;16:323–7.

Vesiculobullous Diseases

Additional Reading 351
Birek C. Herpesvirus-induced diseases: oral manifestations and current treatment options. J Calif Dent Assoc 2000;28:911–21.
Cunningham MW. Pathogenesis of group A streptococcal infections. Clin Microbiol Rev 2000;13:470–511.
Dabelsteen E. Molecular biological aspects of acquired bullous diseases. Crit Rev Oral Biol Med 1998;9:162–78.

Red/Blue Lesions

Boshoff C, Chang Y. Kaposi’s sarcoma-associated herpesvirus: a new DNA tumor virus. Annu Rev Med 2001;52:453–70.
Boshoff C, Weiss RA. Epidemiology and pathogenesis of Kaposi’s sarcomaassociated herpesvirus. Philos Trans R Soc Lond B Biol Sci 2001;356: 517–34.

Tuberculosis

• Systemic therapy (prolonged treatment with at least 2 drugs)
• Isoniazid 300 mg daily × 6 mo • Rifampin 450–600 mg daily × 6 mo • Ethambutol 15 mg/kg daily for first 2 mo • Pyrazinamide 1.5–2.5 mg/kg for first 2 mo

Stevens-Johnson Syndrome

• Topical therapy (compounded rinses)
• Option 1 – Diphenhydramine 200 mg, viscous lidocaine 90 mL,
Maalox suspension 90 mL, distilled water 180 mL – Swish 5 mL for 2 min and expectorate 3–4 times/d
• Option 2 – Dexamethasone 100 mg, viscous lidocaine 60 mL,
diphenhydramine 200 mg, sorbitol 15 mL, Maalox suspension to 275 mL–

Recurrent Herpes Simplex Labialis or Stomatitis

• Topical therapy
• Penciclovir cream (Denavir) 1% 1.5 g tube; apply at the onset of symptoms every 2 h × 4 d
• Docosanol cream (Abreva) 10%; apply topically at the onset of symptoms q2–3h 5 times daily
• Acyclovir ointment 5% 3 g tube; apply at the onset of symptoms 6 times daily × 7 d

Recurrent Aphthous Stomatitis (Aphthosis)

• Classify disease into simple versus complex • Simple aphthosis
• Amlexanox paste 5 g (Aphthasol); apply to ulcers after meals and at bedtime
• Fluocinonide 0.05% gel/cream 60 g – Apply to early lesions after meals and at bedtime. – Do not apply to ulcers.
• Compounded rinse option 1 – Diphenhydramine parenteral (or 12.5 mg/5 mL non-

Plasma Cell Gingivitis

• Identify contact allergen(s) and avoid exposure. • Topical therapy: fluocinonide gel/cream 0.05% 60 g; apply
after meals and at bedtime • Systemic therapy: griseofulvin 250 mg tablets #150; take 1
with each meal for 7 wk Pyostomatitis Vegetans
• Seek the underlying inflammatory bowel disease. • See “Crohn’s Disease.”

Pemphigus Vulgaris

• Coordinate overall management with patient’s internist/primary
care physician since treatment of this disease requires systemic immunosuppression and/or use of anti-inflammatory drugs.
• Management of oral lesions will consist of systemic immunosuppressive agents.

Melkersson-Rosenthal Syndrome

• See “Fissured Tongue.” • Orofacial granulomatosis—see “Cheilitis Granulomatosa”
Nevus • All pigmented nevi should be excised, if reasonable from a

Lupus Erythematosus

• Topical therapy
• Fluocinonide gel/cream 0.05% 60 g; apply after meals and at bedtime
• Tacrolimus (Protopic) ointment 0.1% 30 g; apply after meals 3 times daily, do not eat or drink for 30 min

Lichen Planus

• Topical therapy
Therapeutics 343
• Betamethasone cream (0.1%) 60 g; apply after meals and at bedtime
• Fluocinonide gel/cream 0.05% 60 g; apply after meals and at bedtime

Hairy Tongue

• Brush tongue surface 10–15 times with dentifrice after meals
and at bedtime to remove debris that causes halitosis. • Topical therapy: dilute H 2
O2(1 part 3% H2O2:1 part HO); brush tongue after meals and at bedtime for black hairy tongue
Hand-Foot-and-Mouth Disease • Fluids

Erythema Multiforme

• Topical therapy (compounded rinses) • Option 1
– Diphenhydramine 200 mg, viscous lidocaine 90 mL, Maalox suspension 90 mL, distilled water 180 mL
– Swish 5 mL for 2 min and expectorate 3–4 times/d. • Option 2
– Dexamethasone 100 mg, viscous lidocaine 60 mL, diphenhydramine 200 mg, sorbitol 15 mL, Maalox suspension to 275 mL
– Swish 5 mL for 2 min and expectorate 3–4 times/d. • Systemic therapy

Drug-Induced Stomatitis (Stomatitis Medicamentosa)

Crohn’s Disease

• Challenging to treat • Trials of therapy
• Intralesional corticosteroids such as triamcinolone acetonide 5–10 mg/mL; inject 1–3 mL per session with sessions at 3–4 wk intervals
• Systemic antibiotic: tetracycline 500 mg tid • Systemic corticosteroid: prednisone 5 mg tablets #80
– Take each morning with breakfast for 16 d as 8/d × 4 d, 6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop

Therapeutics Actinomycosis

• Systemic therapy: penicillin or tetracycline in large doses for
3–6 mo • Wide excision of infected tissue
Acute Herpetic Gingivostomatitis • Systemic therapy
• Valacyclovir 500 mg #20; 1 tablet twice daily × 10 d • Acyclovir 400 mg #50; 1 tablet 5 times daily × 10 d
• Fluids • Analgesia

Etiology • Primary form usually due to parathyroid adenoma • Secondary form related to altered renal vitamin D metabolism
with secondary hypocalcemia • Excessive parathormone secretion common to all forms
Clinical Presentation • Classic triad in patients over 60 years includes the following:

Cleidocranial Dysplasia

Etiology • Autosomal-dominant trait with high penetrance and variable
expressivity • Mutations in SH3-binding protein on chromosome 4p16.3 • Widespread membranous and endochondral defects in cranio-
facial complex
Clinical Presentation • Chief head and neck manifestations include the following:

Etiology • Autosomal-dominant, fibroblast/giant cell–containing
condition • May be secondary to somatic mutation, mapping
to chromosome 4p16.3 • No associated metabolic or biochemical alterations noted • Possible linkage/association with Noonan’s syndrome

Metabolic and Genetic Disorders Amyloidosis

Etiology • May be primary (idiopathic), secondary to systemic disease, or
familial • Formation of a fibrillar protein deposited in soft tissues and
visceral organs with associated levels of dysfunction
Clinical Presentation • The primary form may produce obvious tongue enlargement

Etiology • May be associated with pre-existing bone disease such as the
following: • Paget’s disease (10 to 15%) • Fibrous dysplasia (0.5%)
• Mutation/amplification of p53, c-myc, c-JUN, c-fos, MOM2, CDK4, SAS
Clinical Presentation • May present with pain paresthesia, trismus, nasal or paranasal

Etiology • Spread of a primary malignancy to the oral cavity structures or
jaws (usually from lung, breast, prostate, colon, kidney) • Accounts for < 1% of oral malignancies
Clinical Presentation • Usually manifests in the jaws with pain and swelling • Not uncommon is loosening of teeth or pathologic jaw fracture • Soft tissue location is rare. • Most frequent sites of primary neoplasms are kidney, lung,

Malignant Nonodontogenic Tumors Ewing’s Sarcoma

Etiology • Unknown • Chromosomal translocations t(11;22), t(7;22), t(7;21) noted • Gene rearrangement often noted, that is, (22;q12) and expres-
sion of the MIC2 gene • Genetically related to primitive peripheral neuroectodermal
tumor via translocations t(11;22), (q24;q12)

Etiology • Usually indicates prematurely erupted deciduous teeth
Clinical Presentation • Erupted teeth at birth • Almost always are incisors • 85% appear in mandible
Treatment • If mobile, extraction

Etiology • Merging of two tooth germs to create a single tooth
Clinical Presentation • A single large tooth (macrodont) is noted. • One less tooth will be present in the dental arch.
Radiographic Findings • Common or separate pulp canals and roots
Diagnosis • Radiographic evaluation • One less tooth present in dental arch

Etiology • Excessive dietary levels of fluoride: greater than one part per
million in drinking water (can lead to fluorosis in a dosedependent relationship) • Childhood ingestion of fluoride dentifrice on a chronic basis • Tooth enamel hypomaturation resulting from prolonged
ingestion of abnormally high levels of fluoride during tooth development, usually between 2 and 3 years of age
Clinical Presentation • Enamel alterations ranging from local pitting to white opacity
or deeper brown mottling • Distribution is symmetric and bilateral and occurs in all quad-

Etiology • Acid dissolution of enamel and dentin • Loss of enamel and, less commonly, dentin secondary to chemical
(usually acids) action/demineralization • Intrinsic sources relate to stomach acid presence within the
oral cavity. • May be due to the following:
• Occupational exposure to acids • Diet with acid exposure (phosphoric acid– containing
beverages; sucking on lemons) • Chronic regurgitation/gastroesophageal reflux • Bulimia-related vomiting
Clinical Presentation • Loss of enamel initially along lingual surfaces of anterior teeth

Etiology • Hereditary disorder (autosomal dominant) of dentin (1:8,000
frequency in population) • May be seen in association with osteogenesis imperfecta • Altered dentin matrix is related to the defective degradation of
dentin phosphoprotein during dentinogenesis.
Clinical Presentation • Primary and permanent dentition exhibit gray to brownish
opalescence • Normal enamel fractures easily from defective underlying
dentin • Severe tooth abrasion related to exposed dentin following
enamel loss • Radiographically, roots are slender to spiked with pronounced

Etiology • An inherited disorder (autosomal dominant) of circumpulpal
dentin with associated alterations of root morphology; no other organs affected
Clinical Presentation • Premature tooth loss • All teeth affected • Two forms, as follows:
• More severe form (type I) characterized by “rootless teeth,” with normal-colored crowns, obliterated pulp chambers, multiple periapical radiolucencies (periapical granulomas/cysts)

Etiology • A compulsive-eating disorder characterized by repeated
episodes of binge eating followed by vomiting or another form of purging, including laxative abuse
• Cause may be biologic (neurometabolic disturbance), psychological (societal pressure for extreme thinness), or combined (biopsychosocial)
• Nearly 1.2 million adolescent and young adult females are affected in the United States; males are considerably less affected. Up to 20% of college-age women are affected.
Clinical Presentation • Oral signs include erosion of teeth, gingivitis, xerostomia,

Etiology • Defined as a physiologic wearing of teeth secondary to normal
function/mastication • Can involve all surfaces of teeth including interproximal,
incisal-occlusal, buccal, and lingual • Abnormal occlusal-incisal relationship can predispose to accelerated rates of attrition
Clinical Presentation • Primary and permanent dentitions can be affected. • Flattened occlusal surfaces and reduction of incisal height • The loss of interproximal tooth surface (usually enamel only)
leads to gradual dental arch shortening.

Etiology • Intrinsic enamel defect that affects all teeth of both dentitions • Results from defective amelogenin genes on X and Y chromosomes and also chromosome 4 (tuftelin gene) • At least 16 variants noted based upon inheritance pattern,
enamel qualities, and radiographic features • Frequency of 1:14,000 to 1:16,000 of population
Clinical Presentation • One of three basic alterations of enamel may be seen: hypopla-

Tooth Abnormalities
Abrasion
Etiology • Excessive or abnormal wearing of teeth • Commonly associated with use of smokeless tobacco, abrasive
dentifrices, cigars, and pipes, habitual grinding, improper tooth-brushing techniques
• Pathologic wear of teeth associated with abnormal habits or function or consumption of abrasive to coarse diets

Etiology • Unknown; granulomatous disease process • May represent a systemic response to a single provoking agent;
mycobacteria has been suggested but not proven • Possible role of genetic factors coupled with disordered reac-
tions to foreign antigens
Clinical Presentation • Mucocutaneous
• Red to brown nodules/plaques with erythema nodosum features
• Minor salivary glands of the lips and palate may be involved. • Erythematous, hyperplastic gingiva
• Salivary/lacrimal • Parotid, submandibular, and lacrimal glands may be
enlarged. • Multiple organ systems, such as the following, may be involved:

Etiology • A mass of inflamed granulation tissue • Forms secondary to pulp necrosis of the associated tooth • May develop following periapical abscess formation or may
form as pulpal death eventuates without abscess precursor
Clinical Presentation • Usually asymptomatic • With acute exacerbation, pain and sensitivity develop. • Tenderness at root apex on palpation • Pain on biting or percussion of tooth
Radiographic Findings • Radiolucency at apex of tooth • Size ranges up to 1 to 2 cm in diameter • Root resorption not uncommon
Diagnosis • Radiographic features • Demonstration of nonvital pulpal component

Etiology • A serious complication of tumoricidal doses of radiation to
the head and neck, usually > 60 Gy (6,000 rads) • Radiation produces damage to the microvasculature, permit-
ting a hypoxic state, which, in turn, leads to a hypocellular bony environment.
• Minor damage to the irradiated bone produces a nonhealing wound, forming dead bone—necrosis.
Clinical Presentation • Usually affects the mandible • Bone pain • Exposed necrotic bone within radiation portal • External fistula formation • Pathologic fracture

Etiology • An acute or chronic inflammatory process within the
medullary space or along the cortical surface of bone • Usually due to extension of a periapical abscess • Other common causes include physical trauma (fracture) or
bacteremia. • Most common organisms include staphylococci and streptococci
Clinical Presentation • Pain, swelling, fever, lymphadenitis • Sequestrum formation • Lower lip paresthesia, occasionally with acute disease in

Median Rhomboid Glossitis
Etiology • A benign, inflammatory condition • Often related to yeast colonization (erythematous candidiasis) • Inflammatory process noted in response to overlying Candida
population • Exact mechanism is unclear
Clinical Presentation • Well-defined, asymptomatic erythematous patch on dorsum of
tongue • Paramedian erythema, usually with focal atrophy of filiform

Etiology • Variable • Most are microbiologic or plaque associated (simple marginal
gingivitis). • Some are modified by hormonal changes, such as those in
pregnancy (pregnancy gingivitis). • Fusospirochetal gingivitis plus poor oral hygiene and poor nutri-
tion are associated with acute necrotizing ulcerative gingivitis. • Rarely, some forms are associated with contact allergy (“plasma
cell gingivitis”).
Clinical Presentation • Dependent on etiology, as follows:

Etiology • Chronic, low-grade, dentoalveolar infection • Resultant bony inflammation extends to the periosteum, pro-
ducing a reduplication of the cortex (“onion skin” effect).
Clinical Presentation • Usually an asymptomatic, unilateral, mandibular, bony hard
asymmetry • Limited to children and young adults
Radiographic Findings • Medullary mottling with (lucent and opaque) ill-defined margins • Periosteal-cortical expansion • Occlusal radiograph shows concentric or parallel layering of
cortex

Drug-Induced Stomatitis (Stomatitis Medicamentosa)

Etiology • Oral changes found in approximately 5% of those with cuta-
neous reaction to drugs • Mucosal alterations may result from the following:
• Myelosuppression • Direct cytotoxic or cytostatic effect(s) on dividing epithelial
cells • Xerostomic effects • Alterations of oral microbial flora
Clinical Presentation • Painful, erythematous, erosive, or ulcerative lesions • Nonkeratinized locations often affected initially • Fixed form of drug-associated eruptions relatively uncommon

Etiology • Isolated, idiopathic, and chronic lip enlargement • May be an incompletely expressed or oligosymptomatic form
of Melkersson-Rosenthal syndrome
Clinical Presentation • One or both lips may be diffusely enlarged and nontender. • Episodic swelling initially, with progression to a persistent
enlargement • Less often, superficial labial exfoliation or surface

Inflammatory Diseases Angioedema

Inflammatory Diseases
Angioedema

Etiology • Usually triggered by ingested antigens (eg, shellfish, nuts,
fruits, medications) • Mechanism associated with immunoglobulin E (IgE)-mediated
mast cell degranulation with subsequent histamine release • Drug reactions resulting in release of inflammatory mediators
(bradykinin) • Some cases have a genetic basis: C1 esterase inhibitor deficien-
cy or inhibitor dysfunction (autosomal recessive) • May be correlated with disease states characterized by the

Etiology • A reactive hyperplasia of the gingiva; may be related to chronic
irritation • Periodontal ligament/membrane origin postulated
Clinical Presentation • Exclusive gingival location; commonly interdental • Nodular, sessile to pedunculated, usually ulcerated mass • Slow growing; may rarely displace teeth • Usually in young adults and adolescents • Early lesions may bleed easily. • Anterior maxillary arch is favored site

Etiology • Sporadic form is idiopathic • May be a component of Gardner’s syndrome • Excludes maxillary and mandibular tori
Clinical Presentation • Sporadic form with frontal and sphenoid sites predisposed • May be multiple • Solitary lesions rare in jaws
Radiographic Findings • Well circumscribed, dense, sclerotic • May be subperiosteal or medullary
Diagnosis • Radiographic features • Microscopic features: normal cortical and trabecular bone

Etiology • A benign fibro-osseous lesion of bone • Cause unknown
Clinical Presentation • Expansile lesion of bone • Cortices intact • May produce deformity, malocclusion, dysfunction • Mandibular lesions are more common than are maxillary.
Radiographic Findings • Well-delineated, smooth contours • Quality varies from lucent to opaque • Margins may be sclerotic. • Can resorb roots and displace teeth • May displace mandibular canal

Langerhans Cell Disease (“Histiocytosis X,” Idiopathic Histiocytosis)

Etiology • Unknown • Proliferation of Langerhans’ cells (immune surveillance cells)
normally found in skin, mucosa, bone marrow, and lymph nodes Clinical Presentation
• A broad spectrum, typically divided into three subsets, as follows: • Unifocal or multifocal chronic disease of bone (eosinophilic
granuloma) • Widely disseminated chronic disease of bone and soft tissue
(Hand-Schüller-Christian disease) • Acute, disseminated disease with bone marrow involvement

Etiology • A rapidly evolving variant of ossifying fibroma of the young • Cause unknown
Clinical Presentation • Onset between 5 and 15 years of age • Rapid growth over several weeks • Maxilla and paranasal areas predominate • Tooth displacement common
Radiographic Findings • Well-defined radiolucency • Focal mineralization may be noted. • Adjacent bone may be eroded or destroyed.

Etiology • Unknown • Probable reactive phenomenon (stimulus undetermined)
Clinical Presentation • Asymptomatic, bony, nodular masses • Cortical bone enlargement of the jaws; usually bilateral and
symmetric • Usually multiple; slow growing • Most commonly along buccal/facial aspects of the maxillary
and mandibular alveolar ridge • Overlying mucosa intact, unremarkable • Usually develops in adults

Benign Nonodontogenic Tumors Carotid Body Tumor

Benign Nonodontogenic Tumors

Carotid Body Tumor
Etiology • Rare neoplasm arising from nonchromaffin paraganglia in
carotid artery bifurcation • Heredofamilial (autosomal-dominant) form can occur (in less
than 10%) • Can be multiple, bilateral, or multicentric
Clinical Presentation • Typically presents as a mass in the lateral neck • May be associated with bruit, hoarseness, dysphagia
Diagnosis • Ultrasonography as a screening measure • Angiography of both carotid systems
Differential Diagnosis • Metastatic tumor • Vagal nerve sheath tumor
Treatment • Surgical removal • Radiation therapy • Combined surgical and radiotherapy

Etiology • A benign proliferation neoplasm of fibroblastic and odonto-
genic epithelial origin
Clinical Presentation • Asymptomatic, firm, slow-growing mass of the attached gingiva • Overlying mucosa unremarkable and intact • Sessile growth pattern • Usually along facial or buccal aspect of gingiva • Calcifications may be present radiographically. • Underlying alveolar bone is spared. • Uncommon to rare • Also seen centrally (within bone)

Etiology • A hamartomatous or benign mixed odontogenic tumor of the jaw • Composed of enamel, dentin, cementum, and pulp tissue
Clinical Presentation • Two forms, as follows:
• Complex: a randomly arrayed mixture of dental tissues with no gross resemblance to a tooth
• Compound: multiple, tooth-like structures • Mean age of occurrence, 12 to 16 years • Asymptomatic, usually small and discovered incidentally • Jaw expansion may be present with large lesions. • Presence may be heralded by an over-retained primary tooth or

Etiology • A benign odontogenic tumor • Unknown origin
Clinical Presentation • A lesion of adulthood (average occurrence at 30 years) • Equal male:female and mandible:maxilla occurrences • Wide age range: second through sixth decades • Usually asymptomatic • May produce jaw expansion
Radiographic Findings • Well-defined, unilocular to multilocular radiolucency • Loculi range from small “honeycomb” to large “soap bubble”

Etiology • A benign odontogenic tumor of uncertain histogenesis • Stratum intermedium component of enamel organ is favored
cell of origin
Clinical Presentation • Chiefly in posterior mandible • Painless, slow growing • Mean age of occurrence is approximately 40 years • Occasional soft tissue origin (peripheral) noted as a sessile
gingival mass • Jaw expansion a common clinical presentation
Radiographic Findings • Usually noted in association with an impacted tooth • Multilocular; most often with mixed radiolucent and
radiopaque features • Impacted tooth often obscured by tumor-

Etiology • A benign, aggressive jaw tumor of odontogenic epithelial (ecto-
dermal) origin; the most common odontogenic tumor after the odontoma
• Incidence of 0.3 cases per million people
Clinical Presentation • Peak incidence during third to fifth decades • 80% occur in the mandible, chiefly in molar and ramus region • Often presents in association with unerupted third molar teeth • May produce marked deformity, facial asymmetry • Extraosseous or peripheral variant arises in gingival tissues of
older adults (fifth to seventh decades) • Typically slow growing, but persistent

Ameloblastic Fibroma and Ameloblastic Fibro-odontoma

Etiology • Ameloblastic fibroma: a benign mixed odontogenic tumor with
concomitant epithelial and mesenchymal neoplastic proliferation • Ameloblastic fibro-odontoma: as for ameloblastic fibroma with
the addition of an odontoma • Spontaneous; no known cause for either

Adenomatoid Odontogenic Tumor
Etiology • Derivation from epithelial component of the enamel organ • Represents less than 10% of odontogenic tumors • Biologic behavior allows for distinction from ameloblastoma
Clinical Presentation • Narrow age range, 5 to 30 years, with most cases noted during
second decade • Female predilection • Anterior jaw location common • Association with unerupted tooth • Asymptomatic; occasionally produces expansion of alveolar
bone • Rarely occurs in gingival soft tissue (peripheral) • May produce root divergence of adjacent teeth
Radiographic Findings • Well defined, unilocular, often adjacent to crown of unerupted

Etiology • Unknown in most cases • May be due to traumatic injury producing intramedullary
hemorrhage and subsequent clot resorption • Alternative theory suggests degeneration of primary intrabony
pathology
Clinical Presentation • Peaks in second decade • Usually in body of mandible • Painless in most cases • Swelling noted in one-fourth of cases
Radiographic Findings • Clearly defined radiolucency • Margins may be uneven but clear. • May extend between tooth roots creating a scalloped pattern

Etiology • Cystic change associated with thyroglossal duct remnants that
failed to involute (tenth week of development) • Rarely may be hereditary in origin (autosomal dominant or
recessive)
Clinical Presentation • Soft, painless, and slowly enlarging mass in anterior midline of
the neck of children and young adults • Usually unilocular as seen by ultrasound examination • Mass is usually mobile • Most occur above the hyoid bone. • May involve the tongue
Microscopic Findings • Cyst lining of squamous, transitional, ciliated columnar epithelium composite • The cyst wall may contain residual thyroid tissue.

Etiology • Preceded by periapical granuloma; arises as follows:
• Secondary to necrosis of dental pulpal tissue • Stimulation of epithelial network (Malassez’s rest) at tooth
root apex results in cystification • Cyst growth continues secondary to effects of osmotic gradient
across epithelial lining layers, mediators of inflammation, and epithelial proliferation
Clinical Presentation • Asymptomatic unless there is an acute exacerbation • Usually a limited process at root apex or lateral to root surface • Radiograph shows a round and well-defined lucency, usually

Etiology • A developmental odontogenic cyst arising from cystic degener-
ation of the enamel organ prior to formation of hard tissue
Clinical Presentation • Invariably has the microscopic appearance of odontogenic
keratocyst • Rare • Radiolucent lesion of jaw • Occurs in place of a tooth, usually a third molar
Radiographic Findings • A well-defined radiolucency • Most commonly in the posterior mandibular quadrants
Diagnosis • Radiograph shows a cyst instead of a tooth • Histologically an odontogenic keratocyst

Etiology • A benign, aggressive developmental odontogenic cyst; may be
associated with mutation of PTCH tumor suppressor gene
Clinical Presentation • 5 to 15% of odontogenic cysts • Usually occurs sporadically as an isolated finding • Approximately 5% are associated with nevoid basal cell
carcinoma. • 5% of patients have multiple odontogenic keratocysts (OKCs)
and no syndrome

Etiology • Autosomal-dominant condition • Loss of heterozygosity at chromosome 9q22.3 • Mutation of PTCH tumor suppressor gene
Clinical Presentation • Multiple jaw cysts (odontogenic keratocysts) • Numerous cutaneous basal cell carcinomas, which arise earlya
in life and are independent of sun exposure • Bifid ribs • Calcification of falx cerebri • Ocular hypertelorism • Mandibular prognathism • Broad nasal bridge • Medulloblastoma • Palmar and plantar pits

Etiology • Developmental, nonodontogenic cyst • Cystic degeneration of epithelial remnants of the vestigial
nasopalatine duct
Clinical Presentation • Usually develops in adulthood • May be incidental on routine dental radiographs • Palatal mass with tenderness and drainage • Adjacent teeth are vital.
Radiographic Findings • Well-defined, medianparamedian radiolucency in anterior

Etiology • Stimulus unknown • Dental lamina remnant proliferation within the alveolar
segment of the jaw, separate from the periodontal ligament
Clinical Presentation • Asymptomatic • Usually occurs in fourth decade and beyond • Usually in mandibular canine/premolar region (65%) • In the maxilla, the lateral incisor area predominates.
Radiographic Findings • Well delineated, round to ovoid lucency with thin, opaque
(corticated) margin • Located lateral to vital tooth roots • Usually unilocular; may be multilocular (botryoid odontogenic
cyst)

Etiology • A developmental odontogenic cyst • A unique jaw cyst with microscopic evidence of glandular differentiation
Clinical Presentation • Slow growing; may be expansile • Located chiefly in the anterior mandible • May present a lateral periodontal relationship
Radiographic Findings • Usually a multilocular cystic radiolucency • Sharply defined with hyperostotic margins • May be extensive, locally invasive; may perforate cortical bone
Diagnosis • Radiographic qualities • Incisional biopsy results show cystic epithelium with mucous
cells and pseudoduct formation

Etiology • Soft tissue cyst of attached gingiva secondary to fluid accumu-
lation within the follicular space of an unerupted tooth
Clinical Presentation • Gingival swelling on the alveolar crest • Usually soft, translucent to bluish (“eruption hematoma”)
Diagnosis • Location • Radiographic demonstration of erupting tooth

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