A 36-year-old woman with HIV infection (CD4 cell count, 609/µL) developed acute right-sided neck pain, which progressed to swelling above the mandible within 2 weeks. She denied constitutional symptoms. Physical examination revealed bilateral neck swelling and tenderness, greater on the right side. Ultrasound study of her neck showed bilateral, anechoic, and primarily cystic lesions in the parotid glands. The cytopathology report after fine-needle aspiration was negative for malignancy. A subsequent CT scan detected a 3-cm, well-circumscribed, and homogeneously hypodense cystic lesion in the right parotid gland and a smaller, 1.2-cm multicystic lesion in the left (Figure). Multiple subcentimeter lymph nodes were present in the cervical and supraclavicular chains bilaterally. On the basis of these imaging findings and the associated lymphadenopathy, the lesions were characterized as benign lymphoepithelial cysts of the parotid gland, a condition found occasionally in HIV-positive individuals1 and that may herald HIV infection.
The patient underwent needle aspiration of the right cyst, with immediate and complete relief of symptoms. Within 3 weeks, however, the neck pain and swelling reappeared, prompting repeated aspiration. Needle aspirations were performed approximately 3 or 4 times a year for the next 3 years. The patient then began highly active antiretroviral therapy (HAART) with efavirenz/emtricitabine/tenofovir (CD4 cell count, 264/µL). One year later, her CD4 cell count had increased to 488/µL and her viral load had become undetectable (< 48 copies/mL). Swelling of the neck improved significantly during the first year of HAART, and the patient did not require aspiration procedures. This was the longest period of relief she had experienced since her symptoms began. To our knowledge, she has not undergone any aspiration procedures since beginning HAART.
In HIV-positive individuals, lymphoepithelial cysts of the parotid gland are typically multiple, bilateral, benign, and associated with lymphadenopathy.2 Approaches to management include aspiration, radiotherapy, surgery, corticosteroids, and antiretroviral therapy.2-4 Conservative management includes repeated aspirations. Surgical resection resolves cysts but associated risks include facial nerve injury.
Limited data suggest that HAART may be curative,4,5 although there are challenges in assessing the treatment’s efficacy, including limited duration of follow-up after initiation of therapy; studies that report “parotid enlargement” with concomitant use of corticosteroid therapy4; and no record of objective measurement of cyst size before and after therapy. To our knowledge, there have been no placebo-controlled randomized clinical trials to assess the impact of HAART on lymphoepithelial cysts.
Benign lymphoepithelial cysts of the parotid gland can be diagnostic indicators of underlying HIV infection, and HIV testing should be performed in patients who present with neck swelling and characteristic clinical or imaging findings.2,4
1. Holliday RA, Cohen WA, Schinella RA, et al. Benign lymphoepithelial parotid cysts and hyperplastic cervical adenopathy in AIDS-risk patients: a new CT appearance. Radiology. 1988;168:439-441.
2. Terry JH, Loree TR, Thomas MD, Marti JR. Major salivary gland lymphoepithelial lesions and the acquired immunodeficiency syndrome. Am J Surg. 1991;162:324-329.
3. Shaha AR, DiMaio T, Webber C, et al. Benign lymphoepithelial lesions of the parotid. Am J Surg. 1993;166:403-406.
4. Craven DE, Duncan RA, Stram JR, et al. Response of lymphoepithelial parotid cysts to antiretroviral treatment in HIV-infected adults. Ann Intern Med. 1998;128:455-459.
5. Holzapfel K, Burghartz M, Rummeny EJ, et al. Bilateral enlargement of the parotid glands in an HIV-positive patient [in German]. HNO. 2008;56:54-56.