Cystic lymphoepithelial lesion of the parotid as an early indicator of HIV infection
KS Kothari1, CV Madiwale1, AA Deshpande2,
1 Department of Pathology, Seth GS Medical College, Mumbai, India
2 Department of Surgery, Seth GS Medical College, Mumbai, India
K S Kothari
Department of Pathology, Seth GS Medical College, Mumbai
|How to cite this article:|
Kothari K S, Madiwale C V, Deshpande A A. Cystic lymphoepithelial lesion of the parotid as an early indicator of HIV infection.J Postgrad Med 2009;55:135-136
|How to cite this URL:|
Kothari K S, Madiwale C V, Deshpande A A. Cystic lymphoepithelial lesion of the parotid as an early indicator of HIV infection. J Postgrad Med [serial online] 2009 [cited 2020 May 28 ];55:135-136
Available from: http://www.jpgmonline.com/text.asp?2009/55/2/135/52847
A 52-year-old male presented with a painless, cystic, left preauricular swelling of seven months duration. Hemogram, urine examination, and chest radiograph were normal. Aspiration yielded 0.5 cc of brown fluid. The cytologic diagnosis was a benign cyst showing few lymphocytes, benign squamous epithelial cells and cyst macrophages. During surgery, the lesion located in the superficial parotid was excised along with two upper cervical lymph nodes measuring 1 cm each. Gross examination revealed a 5 cm multiloculated cystic lesion with thick fibrous septa and few solid white areas [Figure 1]. Histology showed multiple, variable sized cysts lined by benign squamous cells and solid epithelial islands amidst lymphoid tissue showing severe follicular hyperplasia [Figure 2]. Solid epithelial islands were also composed of squamous cells and lymphocytes. There were only a few atrophic parotid acini. Occasional giant cells in the cyst walls, intra-luminal macrophages, eosinophilic material, and few plasma cells were present. Lymph nodes had a solid white cut surface and showed similar lesions on histology. The immunohistochemistry pattern was as follows: expression for epithelial membrane antigen (EMA) in epithelial components, CD20 in follicular centers, CD3 in mantle zones, and a lack of bcl2 staining in lymphoid centers. There was polytypic B cell proliferation as evidenced by staining for both kappa and lambda light chains. The diagnosis was cystic lymphoepithelial lesion (CLEL) of the parotid and cervical lymph nodes. HIV testing was recommended by the pathologist and was found to be positive. Anti-retroviral therapy was prescribed. However, the patient came back for a follow-up visit only once after 8 months with swelling in the opposite parotid region and cervical lymph nodes. He did not comply with further follow-up visits.
Parotid CLEL is an important head and neck manifestation of HIV infection seen in 3-6% of HIV-positive patients.  Parotid CLEL is secondary to HIV associated hyperplasia of the intra-parotid lymph node as well as hyperplasia of HIV-induced diffuse interstitial lymphoid infiltrates.  Such HIV-induced lymphoid tissue is well known in the parotid and lungs. Patients usually lack features of AIDS and seek medical help only for cosmetic reasons. Thus, parotid CLEL may occasionally be the presenting manifestation of HIV infection.  Since HIV-related lymphoid hyperplasia is generalized, the lesions are usually bilateral. Occurrence in other salivary glands is rare as these lack lymphoid tissue. Terry, et al. found cervical lymphadenopathy due to hyperplasia in 35 of 38 cases with HIV-associated parotid CLEL, however, lymph node CLEL was noted in only two cases.  Extraparotid CLEL that occurs in cervical lymph nodes is thought to originate from intranodal salivary inclusions.
The hyperplastic lymphoid tissue largely replaces salivary tissue. Salivary acini undergo pressure atrophy while duct obstruction by exuberant lymphoid proliferation causes cyst formation.  Some atrophic acini are replaced by solid cellular proliferations composed of epithelial and myoepithelial cells. The cyst lining and epithelial islands typically show lymphocytic infiltration. Cysts of CLEL can show squamous, cuboidal, coloumnar, ciliated columnar, muco-epidermoid, or sebaceous cells.  Foreign body giant cells and granulomas in response to keratin may be seen.  Immunohistochemistry highlights the reactive nature of this process.  The intraepithelial lymphocytes in HIV-unassociated cases are strongly bcl positive, while in the HIV setting there is absence of bcl-2 expression as HIV is postulated to cause down regulation of bcl-2 protein.  The HIV p24 antigen has been demonstrated within the follicular center cells in HIV-positive cases.  Fine needle aspiration demonstrates a proteinaceous background with epithelial cells, lymphocytes, and macrophages.  These cytologic findings along with radiologic detection of bilateral multi-centric parotid cysts should arouse suspicion of CLEL and warrants HIV testing. 
Parotid CLEL can occur unassociated with HIV infection, it is most commonly associated with Sjogren's syndrome (SS).  Conversely, some HIV-positive patients may develop Sjogren's-like syndrome (SLS), which differs from SS by its lack of female predilection and the rheumatoid factor.  CLEL lesions have also been noted in patients with severe allergies, unexplained lymphocytosis, and in normal individuals. Lesions not associated with HIV are usually solid, solitary lesions and are not associated with lymphadenopathy.  Parotid MALT lymphoma may be multicystic and mimic CLEL. However, monocytoid B cells and monotypic immunoglobulins that characterize MALT are lacking in CLEL. Branchial cleft cyst, Warthin's tumor, mucoepidermoid carcinoma, or other malignancies with cystic change may resemble CLEL but each has sufficiently distinctive histological features. 
CLELs are treated by local excision.  Since cysts are multicentric, conservative management by repeated aspirations is difficult. Zidovudine therapy may be useful.  Follow-up studies are few and some cases where CLEL was the initial indicator of the underlying immunocompromised state have been described to develop into full-blown AIDS.  Malignant transformation has not been described.
This case reinforces important aspects of the relationship between CLEL and HIV infection and thereby merits documentation.
|1||Shaha AR, DiMaio T, Webber C, Thelmo W, Jaffe BM. Benign lymphoepithelial lesions of the parotid. Am J Surg 1993;166:403-6.|
|2||Maiorano E, Favia G, Viale G. Lymphoepithelial cysts of salivary glands: An immunohistochemical study of HIV-related and HIV-unrelated lesions. Hum Pathol 1998;29:260-5.|
|3||Weidner N, Geisenger KR, Sterling RT, Miller TR, Yen TSB. Benign lymphoepithelial cysts of the parotid gland: A histologic, cytologic and ultrastructural study. Am J Clin Pathol 1986;85:395-401.|
|4||Chetty R. HIV associated lymphoepithelial cysts and lesions: Morphological and immunohistochemical study of the lymphoid cells. Histopathology 1998;33:222-9.|
|5||Terry JH, Loree TR, Thomas MD, Marti JR. Major salivary gland lymphoepithelial lesions and the acquired immunodeficiency syndrome. Am J Surg 1991;162:324-9.|
|6||Kordossis T, Paikos S, Aroni K, Kitsanta P, Dimitrakopoulos A, Kavouklis E, et al . Prevalence of Sjogren's like syndrome in a cohort of HIV-1 positive patients: Descriptive pathology and immunopathology. Br J Rheumatol 1998;37:691-5.|