Connective Tissue Lesions
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CEMENTOBLASTOMA
Cementoblastoma is a rare benign mesenchymal tumor of odontogenic origin. It most commonly involves the mandibular permanent first molar area. Teeth with associated lesions remain vital. The common clinical presentation includes:
- Pain and associated swelling due to bony expansion.
- Well-circumscribed radiopaque lesion with peripheral radiolucent halo.
- Mass fused to the root of the affected tooth.
- Possible root resorption, invasion of the root canal, and cortical erosion.
Diagnosis is based on the lesion history, clinical presentation, and microscopic findings, which may include:
- Abundant number of plump cementoblasts.
- Irregular network of hard hypocellular cementum-like tissue embedded in a fibrovascular stroma.
- Prominent basophilic reversal lines within the cementum (Pagetoid appearance).
- Histologically similar to osteoid osteoma.
Your differential diagnosis should include:
- Focal osseous dysplasia.
- Ossifying fibroma.
- Osteoma.
Treatment options usually involved tooth removal and enucleation. The prognosis is excellent.
CHEILITIS GLANDULARIS
Cheilitis Glandularis is a rare inflammatory condition that predominantly affects the minor salivary glands and surrounding tissues of the lips. The cause is unknown. The common clinical presentation includes:
- Usually involves the lower lip of adult males (occasionally both lips).
- Symptomatic eversion and enlargement of lip(s).
- Intensive mucoid to purulent secretion at minor salivary gland orifices.
- Development of yellowish plaques (or crusts) that cover the labial mucosa.
Diagnosis is based on the lesion history, clinical presentation, and microscopic findings, which may include:
- Signs indicative of nonspecific inflammation.
- Salivary gland hyperplasia and ductal ectasia.
- Sialadenitis.
Your differential diagnosis should include:
- Granulomatous cheilitis (orofacial granulomatosis).
- Atopic cheilitis.
- Actinic cheilitis.
Treatment options include:
- Surgical excision.
- Suppressive therapy with broad-spectrum antibiotics.
- Intralesional corticosteroid injections.
- Topical corticosteroids or other immunosuppressive medications.
FIBROMAS
Fibromas are benign reactive (hyperplastic) lesions composed of fibrous connective tissue, often as a result of chronic low-grade irritation or trauma. The common clinical presentation includes:
- Pedunculated or broadly based (sessile) fibrous mass with a smooth, elevated surface.
- Normal-appearing surface tissue.
- Asymptomatic and slow growing, soft to firm.
- May become ulcerated or keratotic.
- Typically attributable to sources (trauma).
Diagnosis is based on the lesion history and clinical presentation only. Your differential diagnosis should include:
- Granular cell tumor.
- Neurofibroma.
- Lipoma.
- Schwannoma.
- Salivary tumor.
- Metastatic tumor.
Treatment usually involves removal of the irritant and conservative local excision.
FIBROUS DYSPLASIA
Fibrous Dysplasia is an uncommon disorder where normal bone is replaced with fibro-osseous tissue, resulting in bone weakening and expansion. The condition can range from asymptomatic to severely disabling and can lead to fractures, deformity, functional impairment, and significant pain. A polyostotic form may occur with endocrine hyperfunction and focal cutaneous pigmentation (McCuneAlbright syndrome). The common clinical presentation includes:
- Tumor-like masses or deformity of bones.
- May be monostotic (80%) or polyostotic (20%).
- Slow-growing, painless swelling of affected bone(s) causing facial asymmetry.
- Most commonly seen in the mandibular body.
- Tooth displacement and malocclusion is common.
- “Groundglass” radiographic appearance.
Diagnosis is based on the lesion history, clinical and radiographic presentation, and microscopic findings, which may include:
- Irregular trabeculae of woven bone (“Chinese characters”).
- Fibroblastic vascularized stroma.
- Stellate appearance of osteogenic cells at the surface of bone.
Your differential diagnosis should include:
- Chronic sclerosing osteomyelitis.
- Paget’s disease.
- Osteosarcoma.
- Cemento-osseous dysplasias.
There is no cure. Treatment options include cosmetic recontouring and palliative care as needed.
FIBROUS HYPERPLASIA
Fibrous Hyperplasia (Epulis Fissuratum) describes inflammatory hyperplasia of fibrous connective tissue in response to localized low-grade irritation or trauma. It is commonly associated with ill-fitting dentures. The common clinical presentation includes:
- Rounded folds of broadly based fibrous tissue.
- Ulceration is often noted at depth of tissue folds.
- A polypoid or leaf-like appearance may be noted on the hard palate.
- Commonly seen on vestibular mucosa at the periphery of a denture flange.
Diagnosis is based on the lesion history and clinical presentation only. Your differential diagnosis should include:
- Lymphoma.
- Soft tissue tumor (fibroma, Kaposi sarcoma, liposarcomas etc).
- Metastatic tumor.
Treatment involves lesion excision and denture reline or replacement.
GIANT CELL GRANULOMA
Giant Cell Granuloma is an uncommon benign but locally aggressive lesion of osteoclastic origin that is mostly seen in the craniofacial region. The cause is unclear. The common clinical presentation includes:
- Multilocular radiolucency with scalloped margins.
- Appearance can be described as “honeycomb” or soap bubble-like.
- Locally osteolytic lesion with bony expansion.
- Near exclusivity in mandible or maxilla.
- Slow growing and asymptomatic.
- Typically does not pierce cortical bone and there is no root resorption.
- Can become aggressive, progressing rapidly.
Diagnosis is based on the lesion history, clinical and radiographic presentation, and microscopic findings, which may include:
- Osteoclast-type of giant cells and loose bundles of polygonal to spindle-shaped cells.
- Focal collections of lymphocytes and neutrophils.
- Proliferative fibroblasts and endothelial cells.
You differential diagnosis should include:
- Ameloblastoma.
- Odontogenic myxoma.
- Odontogenic keratocyst.
- Hemangioma.
- Aneurysmal bone cyst.
- Traumatic bone cyst.
Treatment options include:
- Thorough curettage and marginal resection.
- If recurrent, aggressive marginal resection.
- Calcitonin.
- Cryotherapy.
- Chemical cauterization.
- Intralesional corticosteroid.
LINGUAL BONE DEFECT
Lingual Bone Defect (Stafne Bone Cyst or Static Bone Cyst) is not a true cysts but a round-to-oval radiolucency observed in the posterior aspects of the mandible. This developmental depression on the lingual side of the mandible “fills” with submandibular gland or adipose tissue. The cause is unknown. The common clinical presentation includes:
- Asymptomatic lesion (usually discovered incidentally).
- Uniform radiolucency with a well-defined dense hypercorticated margin.
- Located inferior to the alveolar canal and anterior to the angle of the mandible.
Diagnosis is based on the lesion history and clinical presentation only. No treatment is required.
LINGUAL THYROID
Lingual Thyroid is a rare lesion in the oropharyngeal region caused by the failure of thyroid primordium to descend from the foramen cecum into the anterior neck. This results in thyroid tissue seen within the tongue. The exact mechanism is unclear. The common clinical presentation includes:
- Dark well vascularized mass on the midline of the tongue around the foramen cecum.
- May interfere with swallowing and breathing in infancy.
- Bleeding may occur.
Diagnosis is based on the lesion history, clinical presentation, and a positive radionuclide scan. Your differential diagnosis should include:
- Thyroglossal duct cyst.
- Squamous cell carcinoma.
- Lymphoma.
Treatment options include:
- Removal so long as it isn’t the only functioning thyroid tissue.
- Move/transplant lesion.
LIPOMA
A lipoma is a benign tumor made of adipose tissue. The etiology is generally unclear, but risk factors include family history, obesity, and trauma. The common clinical presentation includes:
- Asymptomatic, slow-growing well circumscribed nodular lesion.
- Sessile or pedunculated base.
- Soft and fluctuant with dough-like consistency.
- Most common sites include buccal mucosa and tongue.
- May have a yellowish tinge if superficially located.
- Larger lesions often are covered by telangiectatic vessels.
Soft tissue diagnostic imaging (ultrasonography, MRI) may be used to help confirm the identification. Diagnosis is based on the lesion history, clinical presentation, and microscopic findings, which may include:
- Lobules of mature white adipose tissue with possible bone formation.
- Mature adipocytes without atypia.
- Spindle cells with enlarged, hyperchromatic nuclei.
- Fibrous septa containing thin-walled capillary-sized vessels.
Your differential diagnosis should include:
- Other soft tissue tumors (Fibroma, Epulis Fissuratum, Neurofibroma etc.).
- Minor salivary gland neoplasm.
- Metastatic disease.
Treatment usually involves either observation only or surgical excision. Recurrence is rare.
MUCOSAL NEUROMA
Mucosal Neuroma is a benign tumor consisting of neural tissue that is strongly associated with the hereditary syndrome multiple endocrine neoplasia type III (MEN III). Patients with MEN are at an elevated risk of developing medullary thyroid carcinoma and pheochromocytoma, as well as various skeletal and ophthalmologic anomalies. Oral lesions may be the initial sign of a syndrome. The common clinical presentation includes:
- Prominent thick lips and frenula.
- Possible ocular neuromas.
- Oral neuromas most commonly seen on the tongue, lips, cheeks, and commissures.
- Medullary thyroid carcinoma.
Diagnosis is based on genetic testing, lesion history, clinical presentation, and microscopic findings, which may include:
- Plexiform bundles of neural tissue.
- Neural axons within bundles.
Your differential diagnosis should include neurofibromatosis. Treatment options include:
- Thyroidectomy.
- Periodic review for pheochromocytoma development.
- Genetic counseling.
NEUROFIBROMA
Neurofibroma is a benign peripheral nerve sheath tumor that can be found as a stand alone lesion (sporadic neurofibroma) or as multiple lesions, which is highly suggestive of a syndrome (neurofibromatosis type I). Neurofibromas result when Schwann cells exhibit inactivation of the NF1 or NF2 genes. Many additional types of cells (perineural fibroblasts) and structural elements are involved. Neurofibromas can be divided into two broad groups. Dermal neurofibromas are associated with a single peripheral nerve, while plexiform neurofibromas are associated with multiple nerve bundles. The common clinical presentation includes:
- Discrete nodules or diffuse lobular lesions.
- Sessile or pedunculated base.
- Often symptomatic and can lead to stinging, itching, pain, and disfigurement.
- Plexiform neurofibromas can grow to significant size.
- Most commonly seen on the tongue, buccal mucosa, mucobuccal fold.
- “Blunderbuss” expansion of inferior alveolar foramen.
- Uniform expansion of alveolar canal in the body of the mandible.
Diagnosis is based on the lesion history, clinical and radiographic presentation, and microscopic findings, which may include:
- Unencapsulated mass of spindle cells.
- Slender fibroblast-like cells with a storiform pattern and low amount of stroma.
- Wavy, twisted nuclei.
- Scattered mast cells in lesions.
Your differential diagnosis should include:
- Localized neurofibroma.
- Neuroma.
- Fibroma.
- Syndrome (neurofibromatosis).
Solitary lesions are usually removed via excisional biopsy. When a patient presents with multiple lesions (neurofibromatosis) treatment is directed towards improving function and/or esthetics.
PAGET'S DISEASE
Paget’s Disease (osteitis deformans) is a condition characterized by aberrant cellular remodeling and deformity of one or more bones. Pathological structural changes cause the bone to weaken, which may result in deformity, pain, fracture or arthritis of associated joints. The exact cause is unknown, but possible etiological factors include viral exposure or autoimmune-mediated vascular disorders. The common clinical presentation includes:
- Pain, often presenting as deep aching pain in affected bones.
- Bones are usually affected bilaterally and symmetrically.
- Bony enlargement is common.
- The mandible and maxilla are commonly affected.
- Associated neurologic complications including vertigo, headache, auditory and/or visual disturbances, and facial paresthesia.
- Dental patients often complain of ill-fitting prostheses or slow separation of teeth.
- Possible radiographic changes include multiple lytic lesions, patchy “cotton wool” like radiopaque pattern, “drumstick” hypercementosis, and obliterated periodontal ligament spaces.
Patients usually present with increased serum alkaline phosphatase, urinary calcium, and hydroxyproline levels. Serum calcium and phosphate levels are normal. Diagnosis is based on the lesion history, clinical and radiographic presentation, and microscopic findings, which may include:
- Early phase – elevated osteoclastic resorption, fibrous tissue replacement of bone, and prominent blood vessels.
- Late phase – elevated osteoblastic function, which results in dense bony deposition with numerous reversal lines.
Your differential diagnosis should include:
- Osteosarcoma.
- Fibrous dysplasia.
- Acromegaly.
CEMENTO-OSSEOUS DYSPLASIA
Florid Cemento-osseous Dysplasia is a asymptomatic non-neoplastic fibro-osseous lesion that originates from the periodontal membrane and presents as multi-quadrant radiopaque cementum-like masses. The cause is unknown. The common clinical presentation includes:
- Radiopaque masses within peripheral radiolucent rim.
- Becomes more radiopaque with age.
- Lamina dura is usually absent.
- Most commonly located in the mandibular anterior region.
- May involve apices of one or more vital teeth.
- Noted usually in fourth and fifth decades.
Diagnosis is based on the lesion history and clinical presentation only. A biopsy is usually not recommended due to the risk of severe infection, bone sequestration and osteomyelitis. Your differential diagnosis should include:
- Multiple periapical abscesses (vitality testing essential).
- Florid osseous dysplasia.
No treatment is required.
