Association between lymphoepithelial cysts of the pancreas and HIV infection

Abstract

Background & aims

To report the association of lymphoepithelial cysts (LEC) of the pancreas with Human Immunodeficiency Virus (HIV) infection. An association between LEC and HIV infection is already established in the parotid gland (PG).

Methods

Report of the first two cases of LEC of the pancreas associated with HIV infection and comparison of the clinical and histopathological aspects of LECs of the pancreas and of the PG.

Results

LECs of the pancreas were discovered by CT imaging in 2 patients with a history of HIV infection. Notably, LEC completely resolved in one patient after initiation of antiretroviral therapy.

Conclusion

This is the first report of an association of LEC of the pancreas and HIV infection. In the presence of LEC of the pancreas, we propose a systematic screening for HIV infection and associated lesions in the PG. Antiretroviral therapy should be initiated in untreated patients. Surgery should be reserved for symptomatic patients in whom medical therapy has failed.

Introduction

A variety of cysts can develop in the pancreatic gland. Pseudocysts account for approximately 30%, while 30–50% are of neoplastic nature.[1] Squamous-lined cysts of the pancreas are rarely encountered or diagnosed. They include dermoid cysts, accessory-splenic epidermoid cysts and lymphoepithelial cysts (LECs) [2].

LECs have been reported in various organs, such as the parotid gland, lung, thymus, thyroid, parathyroid and pancreas [3], [4], [5]. LEC of the pancreas is an uncommon type of pancreatic cyst first described by Luchtrath and Schriefers in 1985.[6] Around 70 cases have been published so far [7]. These lesions are usually benign and develop in middle-aged patients (mean age, 55 y; range 35–82 y), with a male predominance (M/F: 4/1) [3]. Forty percent of patients are asymptomatic. Symptoms are generally aspecific and may include abdominal pain, nausea, vomiting or diarrhea. The reported sizes range from 1.2 to 17 cm. They can be multi- (60%) or unilocular (40%) and do not have preferential locations within the pancreatic gland. Both CEA and CA19-9 can be elevated [8]. Radiologically, they appear as cystic lesions with internal heterogeneous hyperechogenicity on ultrasound, hyperdensity on computed tomography (CT) and granular hypointensity on T2-weighted magnetic resonance (MR) imaging [9], [10]. Their sharp delineation from the surrounding pancreatic tissue may suggest the diagnosis. LECs have no malignant potential, but can be misdiagnosed as mucinous cystic lesions [11], [12], [13], [14]. Fine needle aspiration (FNA) under endoscopic ultrasonography (EUS) guidance is the preferred diagnostic method [15], [16], [17]. It typically retrieves characteristic “caseous” material. Cytologically, it appears as abundant anucleated squamous debris admixed with rare small lymphocytes. However, the definitive distinction between LECs and other squamous-lined cysts in the pancreas may be difficult. Furthermore, gastro-intestinal contamination on the FNA material can mimic a cystic mucinous lesion [12], [14]. The histological aspect of LEC is quite typical with a stratified squamous epithelium lining a dense subepithelial lymphoid tissue that contains lymphoid follicles [4], [18], [19]. The treatment of LECs is usually conservative, surgical resection being reserved for symptomatic cases. In such cases, enucleation or partial pancreatectomy is favoured [20], [21].

Although LEC is better defined and much more frequent in the parotid gland (PG), its pathogenesis remains incompletely understood. However, the well-known association between LEC of the PG and Human Immunodeficiency Virus (HIV) has led some authors to recommend investigating HIV serology in the presence of LEC of the PG [22], [23]. Such an association has not yet been reported in the pancreas.

We report herein on the first two cases of LEC of the pancreas associated with HIV infection, an association that emphasizes the similarity between LECs of the pancreas and of the PG.