The benign lymphoepithelial cyst and a classification system for lymphocytic parotid gland enlargement in the pediatric HIV population.

OBJECTIVES/HYPOTHESIS: The objectives of this study are to present a series of parotid gland benign lymphoepithelial cysts (BLEC) in HIV-positive children and to propose a three-tiered classification system for HIV-associated lymphocytic parotid gland enlargement.

STUDY DESIGN: The authors conducted a retrospective case series and literature review.

METHODS: The authors conducted a retrospective chart review of four pediatric patients with HIV-associated parotid gland BLEC who presented to a tertiary care university medical center.

RESULTS: Four pediatric HIV-positive patients (four girls; age range, 7-17 years [mean age, 12.8 years]) were diagnosed with parotid gland BLEC. Two patients presented with acute parotitis and the others presented with asymptomatic enlargement of the parotid glands. Three patients had bilateral parotid gland BLEC. The other patient demonstrated persistent generalized lymphadenopathy (PGL) of the intraparotid and cervical lymph nodes and early BLEC limited to the left parotid gland. One patient also displayed parotid gland microcalcifications and cystic changes in the adenoids, neither of which have been described previously in the setting of HIV-associated BLEC. Computed tomography was performed on all patients, and one patient underwent fine needle aspiration to confirm the diagnosis. All patients opted for observation and antiretroviral medication therapy as long-term treatment. Based on these findings and a review of the literature, we propose a three-tiered classification system for lymphocytic parotid gland enlargement in the HIV population: 1) PGL, 2) benign lymphoepithelial lesions (BLEL), and 3) BLEC.

CONCLUSIONS: This series equals the largest pediatric series of HIV-associated parotid gland BLEC in the English literature. One patient in our series also demonstrated PGL; there were no cases of BLEL. A classification system based on morphology is proposed to help resolve the confusion in terminology used to describe this entity. Most pediatric HIV-infected patients with parotid gland BLEC can be treated with observation and antiretroviral medication therapy. For others, who are symptomatic or more concerned about their cosmetic appearance, sclerotherapy may offer a reasonable option. Radiation therapy and surgery should be reserved for select cases.