Introduction:
The benign lymphoepithelial lesion is a disease in which the body's immune system starts attacking normal cells. It is associated with clinical manifestations of Sjogren's syndrome. It is also called Mikulicz's disease. There is swelling of one or more salivary and lacrimal glands. In addition, there is decreased secretion from the glands, causing xerostomia (decreased salivation causing drying of mouth) and keratoconjunctivitis (dryness of conjunctiva causing dry eyes). It destroys the acinar (small sac-like cavity in the gland) tissue and causes proliferation and damage to the epithelium of ducts. Smears (microscopic examination) show marked clusters of ductal epithelial cells, lymphocytes ( a type of blood cell), and lymphoid cells (a type of immune cell seen in blood and lymph). The smear pattern is similar to autoimmunethyroiditis (a condition where antibodies start attacking the thyroid gland). It is associated with systemic conditions, including tuberculosis, leukemia, syphilis, Hodgkin's disease (a type of cancer that affects lymphatic system), lymphosarcoma (malignant tumor of lymphoid tissue), Sjogren’s syndrome (a disorder of lymphatic system that causes dry eyes and dry mouth), and systemic lupus erythematosus (a autoimmune disorder that affects skin, joints, kidneys, and blood vessels). Godwin published the first description of salivary gland lymphoepithelial tumors in 1952.
What Is the Pathophysiology of Benign Lymphoepithelial Lesion?
The benign lymphoepithelial lesion is an autoimmune disease of salivary glands. Clinical features of Sjogren syndrome are present in 80 % of the population. There is an increased risk of developing malignant lymphoma (cancer of the body's lymphatic system). Rarely associated with minor salivary glands, palatal swellings are usually neoplastic (cancer).
What About the Epidemiology of Benign Lymphoepithelial Lesion?
Benign lymphoepithelial lesions are common in HIV (human immunodeficiency virus) patients. It is mainly seen in HIV-positive patients without AIDS (acquired immunodeficiency syndrome). The parotid gland is the most commonly affected. Lacrimal glands are also affected. Benign lymphoepithelial lesions are rarely seen in the submandibular glands or sublingual glands. The tonsils and neck can also be involved.
What About the Clinical Presentation of Benign lymphoepithelial Lesions?
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A benign lymphoepithelial lesion is seen around 50 years of age in adults.
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It mainly occurs in females.
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There are diffuse swellings of the salivary gland, which can be asymptomatic and associated with mild pain.
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HIV-infected patients are the most affected ones.
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There is swelling of either one or both lacrimal glands and salivary glands. Along with these symptoms, systemic diseases should be considered benign lymphoepithelial lesions.
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Patients may have a recurring fever.
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Inflammation of the eyes is called uveitis.
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There is difficulty in swallowing food.
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Painless swelling of salivary glands.
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Glands near the submaxillary jaw may become swollen.
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The benign lymphoepithelial lesion is a rare condition challenging to diagnose.
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These lesions, as discussed, are early manifestations of HIV infection.
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These lesions occur in adults and children and are equally seen in males and females.
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The biopsy (diagnostic procedure for cancer in which a small part of abnormal growth is taken out for microscopic examination) is required to distinguish benign lymphoepithelial lesions from sialadenitis (non-inflammatory swelling of salivary glands).
What Is the Etiology Associated With Benign Lymphoepithelial Lesion?
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The exact cause of Mikulicz syndrome is unknown.
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It is an autoimmune disorder when the antibodies begin to attack healthy tissue for unknown reasons.
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It may occur because of excessive accumulation of specific white blood cells (lymphocytes) into many glands of the face, mouth, or neck.
What About Pathology Associated With Benign Lymphoepithelial Lesion?
A benign lymphoepithelial lesion arises because of the dilatation (enlargement) of intraglandular duct obstruction. This obstruction is mainly due to lymphoid hypertrophy (excess growth of lymphoid tissue). The lesion is usually bilateral.
What Are the Radiographic Features of Benign Lymphoepithelial lesions?
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There are well-circumscribed cystic spaces.
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There can be thin rim enhancement on magnetic resonance imaging.
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Ultrasound depicts multiple small septations (division) in glands and small mural nodules (cancer-like but exactly cancer).
What About Differential Diagnosis of Benign Lymphoepithelial Lesion?
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A congenital (present from birth) condition in which tissue develops incorrectly on one side of the neck or both sides is called a branchial cleft cyst.
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Benign neoplasm (non-spreading cancer) of major salivary glands is called warthin tumor.
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The disorder in which there are dry eyes and mouth is called Sjogren syndrome.
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The subcutaneous (under a layer of skin) cavity containing saliva is called sialocele.
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The growth of inflammatory cells and granulomas (area of inflammation) in the body are called sarcoidosis. Sarcoidosis is an immune disorder that affects the heart, eyes, skin, and other organs.
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Parotid lymph nodes are the most common metastases (cancer spreads to different parts from origin) of the head and neck region.
What About the Treatment of Benign Lymphoepithelial Lesions?
Treatment generally consists of careful observation unless the patient has concerns such as pain, drainage associated with glands, or other symptoms. Then, a detailed history, serological tests (a test that checks the presence of antibodies), and clinical examination are required to diagnose the condition. In such cases, surgical removal of the affected gland is recommended. Treatment options for benign lymphoepithelial cysts include drainage, surgery, radiotherapy, sclerotherapy (injecting a solution directly into the veins), and conservative therapy using highly active antiretroviral medication. Aspiration (drainage of content) can also be performed multiple times. Aspiration is commonly performed in weak patients whose surgery benefits would be outweighed by the risks. The prognosis is usually good. This condition may rarely develop into lymphoma (cancer of the lymphatic system).
Conclusion:
A benign lymphoepithelial lesion is associated with HIV infection. This lesion is rare and difficult to diagnose. It is an early manifestation of HIV infection and is rarely seen in active antiretroviral therapy patients. The benign lymphoepithelial lesions of major salivary glands are mainly seen in parotid glands and are associated with Sjögren syndrome. It occurs in salivary glands and their lymph nodes in the oral cavity, tonsil, thyroid gland, and pancreas. These lesions enlarge the parotid glands and cervical lymph node enlargement and cause nasopharyngeal lymphoid hyperplasia (excessive growth of pharynx and nasopharynx lymphoid tissue). This pathologic state is sometimes associated with Sjogren syndrome.
