270 PDQ ORAL DISEASE

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

Prognosis
• Generally good
• Can be locally invasive
• May metastasize in 5 to 25% of cases
Benign Nonodontogenic Tumors 271
272 PDQ ORAL DISEASE

Exostosis
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

Diagnosis
• May appear radiographically as homogeneous opacities

Differential Diagnosis
• Peripheral fibroma
• Periostitis
• Periosteal/parosteal osteosarcoma

Treatment
• None required
• May need to be removed for prosthesis (denture) construction

Prognosis
• Excellent
Benign Nonodontogenic Tumors 273
274 PDQ ORAL DISEASE

Juvenile Ossifying Fibroma

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.

Microscopic Findings
• Prominent stromal cellularity
• Woven bone and/or psammomatous calcifications
• Plump osteoblast rimming

Diagnosis
• Correlation of histologic and radiographic findings

Differential Diagnosis
• Osteosarcoma
• Central giant cell granuloma
• Odontogenic tumor

Treatment
• Wide local excision or resection
• Reconstruction

Prognosis
• Recurrence rate of 30 to 50%
Benign Nonodontogenic Tumors 275
276 PDQ ORAL DISEASE

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
(Letterer-Siwe disease)
• Most arise in childhood; eosinophilic granuloma often arises in
adolescents and adults.
• Jaw lesions noted in up to 20% of cases with tenderness, loose
teeth (focal to segmental), gingival inflammation, and friability
Radiographic Findings
• Bone lesions often punched out, sharply circumscribed
• “Floating teeth” appearance with alveolar bone involvement
• Skeletal survey should be performed to rule out multiple bone
involvement
Diagnosis
• Radiographic demonstration of lytic bony lesions
• Infiltrate of mononuclear cells, often with clefted nuclei
• Often accompanied by a variety of other cell types, including
eosinophils, lymphocytes, giant cells, plasma cells
• Immunohistochemical demonstration of CD1a staining
• Langerhans’ cells also stain for S-100 protein, although the
antibody is less specific.
• Ultrastructural demonstration of cytoplasmic racquet-shaped
Birbeck granules
Differential Diagnosis: Clinical
• Cat-scratch disease
• Juvenile xanthogranuloma
 Benign Nonodontogenic Tumors 277

Differential Diagnosis: Radiologic

• Juvenile periodontitis, endocrinopathies, hypophosphatasia,
leukemia, bony malignancy (primary/metastatic)
• In adults: myeloma
Treatment
• Localized variant
• Surgical curettage of bony lesions
• Low-dose radiation therapy of inaccessible lesions
• Widespread variants
• Chemotherapy including methotrexate, vincristine,
cyclophosphamide
• Bone marrow transplantation for resistant/recurrent disease
Prognosis
• Varies with form of disease, as follows:
• Localized variant: very good
• Disseminated variant: fair to poor
278 PDQ ORAL DISEASE

Ossifying Fibroma
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

Microscopic Findings
• Fibrovascular stroma
• Islands/trabeculae of osteoid, woven bone
• Cementum droplets may be present.

Diagnosis
• Correlation of histologic and radiographic findings

Differential Diagnosis: Radiographic

• Odontogenic cyst
• Giant cell lesion
• Odontogenic tumor

Differential Diagnosis: Histologic

• Fibrous dysplasia (must have clinical-pathologic correlation)

Treatment
• Conservative excision
• Enucleation with peripheral bony curettage
 Benign Nonodontogenic Tumors 279

Prognosis
• Excellent
280 PDQ ORAL DISEASE

Osteoma
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

Differential Diagnosis
• Tori, exostoses
• Ossifying fibroma
• Osteoblastoma
• Focal sclerosing osteitis

Treatment
• Usually none
• Local resection, if compromising

Prognosis
• Excellent
• Little recurrence potential
• When associated with Gardner’s syndrome, malignant conver-
sion of intestinal polyps is assured.
Benign Nonodontogenic Tumors 281
282 PDQ ORAL DISEASE

Peripheral Ossifying Fibroma

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

Diagnosis
• Central islands or trabeculae of bone/cementum
• Fibroblastic proliferation in a sheet-like configuration
• Usually ulcerated with granulation tissue base

Differential Diagnosis
• Pyogenic granuloma
• Peripheral giant cell granuloma
• Peripheral fibroma
• Peripheral odontogenic tumor
• Osteosarcoma/chondrosarcoma
• Metastatic neoplasm

Treatment
• Excision including underlying periosteum or associated peri-
odontal ligament

Prognosis
• Recurrence occasionally seen; believed to be related to incom-
plete excision
Benign Nonodontogenic Tumors 283