Salivary and Lymphoid Lesions
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MUCOCELE
A mucocele is a common benign mucus-containing cystic lesion that is caused by mucus accumulation and swelling of a minor salivary gland. The two subclassifications are 1) mucus extravasation cyst, and 2) mucus retention cyst. Extravasation mucoceles result from a damaged salivary gland duct and subsequent spillage into the surrounding soft tissues. Retention mucoceles result from a blockage of the salivary gland ducts. The common clinical presentation includes:
- Painless bluish swelling when mucin is near the surface.
- Commonly seen on the lower lip, but can affect buccal mucosa and the anterior ventral surface of the tongue.
- Often waxes and wanes in size.
Diagnosis is based on genetic testing, lesion history, clinical presentation, and microscopic findings, which may include:
- Mucin-containing cyst-like space surrounded by granulation tissue.
- Macrophage and neutrophil response to free mucin.
- Focal chronic sialadenitis.
Your differential diagnosis should include:
- Hemangioma.
- Varices.
- Pyogenic granuloma.
- Other salivary neoplasm.
- Connective tissue neoplasm.
Treatment options include:
- Surgical excision.
- Marsupialization (high recurrence rate).
- Laser ablation.
- Cryosurgery.
- Electrocautery.
NECROTIZING SIALOMETAPLASIA
Necrotizing Sialometaplasia is a benign, self-limiting inflammatory salivary gland lesion which may clinically and histologically mimic squamous cell carcinoma or mucoepidermoid carcinoma. Trauma to a minor salivary gland leads to local ischemic injury of the salivary gland lobules, resulting in the worrisome clinical appearance. The common clinical presentation includes:
- Most commonly affects major and minor salivary glands of the palate.
- Unilateral dysesthetic to painful submucosal swelling that turns into a necrotic crater/ulcer.
- May extend to and involve deep soft tissues and palatal bone.
Diagnosis is based on lesion history, clinical presentation, and microscopic findings, which may include:
- Pseudoepitheliomatous hyperplasia of the overlying epithelium.
- Salivary gland inflammation and lobular necrosis.
- Squamous metaplasia of ductal epithelium.
You differential diagnosis should include:
- Salivary gland tumor.
- Squamous cell carcinoma.
- Mucoepidermoid carcinoma.
- Granulomatous disease.
No treatment is required.
RANULAS
Ranulas are mucoceles that originate from major salivary glands, caused by the obstruction of the sublingual or submandibular gland ducts and subsequent extravasation of saliva into the soft tissues. When saliva accumulates above the mylohyoid muscles it is termed an oral ranula. When below the mylohyoid muscle in the fascial planes of the neck it is called a cervical or plunging ranula. The common clinical presentation includes:
- Unilateral, fluctuant swelling on the floor of the mouth.
- Usually has a bluish hue with slight translucent quality.
- Blockage is often caused by a salivary stone (sialolith).
- Occlusal radiographs may demonstrate a suspected sialolith.
High-resolution ultrasonography can be useful to detect calculi, abscesses, and cystic spaces. Diagnosis is based on lesion history, clinical presentation, and microscopic findings, which may include:
- Mucinous salivary fluid when aspirated.
- Granulation tissue lining around the mucin pool.
Your differential diagnosis should include:
- Varices.
- Hemangioma.
- Lymphangioma.
- Abscess.
- Dermoid cyst.
- Benign or malignant salivary gland tumor.
- Soft tissue tumor (e.g. Lipoma, Fibroma).
- Cystic hygroma.
Treatment options include:
- Surgical excision.
- Marsupialization.
- Excision of the involved gland (extravasation type).
- Laser ablation, cryosurgery, or electrocautery.
- Sialolithectomy (in obstructive type).
SJOGREN'S SYNDROME
Sjögren’s Syndrome is an autoimmune disease caused by autoantibody production against nuclear antigens SS-A and SS-B, which leads to exocrine gland dysfunction and mononuclear cell infiltration. It can present independent of other health conditions (primary Sjögren’s syndrome) or combined with other connective tissue disorders (secondary Sjögren’s syndrome) such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or systemic sclerosis. Sjögren’s syndrome is associated with an increased risk of developing lymphoma. The common clinical presentation includes:
- Sicca syndrome (xerostomia and xerophthalmia) with keratoconjunctivitis.
- Secondary dental caries, oral candidiasis, ocular discomfort.
- May include dry skin, respiratory problems, paresthesia in the arms and legs, fatigue, muscle and joint pains, and thyroid problems.
- Salivary and lacrimal gland enlargement.
Serologic analysis is used to demonstrate SS-A or SS-B antibodies, antinuclear antibodies, and rheumatoid factor. Diagnosis is based on genetic testing, lesion history and clinical presentation only. Your differential diagnosis should include:
- Sarcoidosis.
- Psychological disorders (Depression, Bulimia, Alcoholism etc.).
- Radiotherapy.
- Side effects associated with medications (e.g anticholinergic drugs).
- Lymphoma.
- Infection (mumps, human immunodeficiency virus).
- Graft-versus-host disease.
Treatment options include:
- Pilocarpine or cevimeline.
- Saliva substitutes and eye drops.
- Gustatory stimulation.
- Synthetic glycoprotein solutions.
- Carboxymethylcellulose sodium.
- Frequent preventative dental and ophthalmic examinations.
LYMPHOMAS
Lymphomas are a group of tumors derived from lymphocytes. Risk factors for the most common variant, non-Hodgkin lymphoma (90%), include autoimmune diseases, viral exposure (HIV, T-lymphotropic virus), immunosuppressants, radiation therapy and pesticides. Hodgkin lymphoma accounts for around 10% of lymphomas and is linked to Epstein-Barr virus exposure. The common clinical presentation includes:
- Painless enlargement of lymph nodes.
- Fever, unintended weight loss, itching, and fatigue.
- Mass of reddish-blue tissue that may exhibit pain if it becomes ulcerated.
- Paresthesia of the lip when lesion is found in the mandible.
BURKITT LYMPHOMA
Burkitt Lymphoma (BL) is a highly aggressive B cell non-Hodgkin lymphoma associated with various genetic mutations (C-MYC, P53, TCF transcription factor) and exposure to certain pathogens (Epstein-Barr virus, Malaria). The endemic variant (African variant) most commonly affects the facial bones of children in malaria-endemic regions. The sporadic variant (non-African variant) is rarely associated with the Eptein-Barr virus and is more strongly associated with the abdominal (retroperitoneal) region. The common clinical presentation includes:
- Rapidly progressive facial tumor causing asymmetry.
- Quick dissemination to extranodal sites including CNS and bone marrow.
- Mainly affects the mandible (especially endemic variant).
- Proptosis, pain and paresthesia.
- Abdominal pain, nausea, vomiting, gastrointestinal bleeding and distention (especially sporadic variant).
Diagnosis is based on the lesion history, clinical presentation and microscopic findings, which may include:
- Pyknotic cellular debris forming the “stars” in the so-called “starry sky” appearance.
- A diffuse proliferation of small B lymphocyte-derived lymphoid cells.
- Tumor cells have round nuclei and prominent nucleoli.
- Scattered macrophages and a high cell turnover (high apoptosis rate).
Your differential diagnosis should include:
- Other lymphoma (Diffuse large B cell lymphoma, Hodgkin’s lymphoma, Follicular lymphoma).
- Severe/acute infection.
- Sialolithiasis.
- Salivary gland tumors.
MULTIPLE MYELOMA
Multiple myeloma (MM) is a tumor caused by clonal proliferation of neoplastic plasma cells within the bone marrow. A solitary tumor is called a plasmacytoma. Solitary tumors invariably become multiple myeloma. Risk factors include obesity, radiation exposure, and family history. The common clinical presentation includes:
- Early lesion – absence of symptoms.
- Mature lesion – bone pain/ paresthesia, anemia, kidney dysfunction, and infections.
- Induration of the tongue (macroglossia) or gingiva may be the initial manifestation.
- Oral lesions may present as polypoid to lobular masses with purpura.
- Well-defined radiolucencies in many bones without opaque lining.
Protein electrophoresis of the blood and urine might show the presence of paraproteins (Bence Jones proteinuria), with IgG paraproteins the most common. A bone marrow biopsy is often requested. Diagnosis is based on the lesion history, clinical presentation and microscopic findings, which may include:
- Diffuse plasma cell proliferation.
- Mott cells containing multiple clustered cytoplasmic droplets (auer rods).
- Flame cells (fiery-red cytoplasm).
Your differential diagnosis should include:
- Lymphoma variant.
- Primary osseous tumor.
- Metastatic tumor.
- Traumatic bone cyst.
- Hemangioma.
Treatment options include:
- Chemotherapy.
- Stem cell transplant.
- Radiation therapy.
- Gene therapy.
