Konofaos et al. Journal of Medical Case Reports 2011, 5:380 http://www.jmedicalcasereports.com/content/5/1/380 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Primary parotid gland lymphoma: a case report Petros Konofaos*, Eleftherios Spartalis, Paraskevas Katsaronis and Grigorios Kouraklis Abstract Introduction: Mucosa associated lymphoid tissue lymphomas are the most common lymphomas of the salivary glands. The benign lymphoepithelial lesion is also a lymphoproliferative disease that develops in the parotid gland. In the present case report, we describe one case of benign lymphoepithelial lesion with a subsequent low transformation to grade mucosa associated lymphoid tissue lymphoma appearing as a cystic mass in the parotid gland. Case presentation: A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision. The pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (mucosa associated lymphoid tissue lymphoma) following benign lymphoepithelial lesion of the gland. Conclusions: Salivary gland mucosa associated lymphoid tissue lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. Parotidectomy is recommended in order to treat the tumor and to ensure histological diagnosis for further follow-up planning. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal. Introduction Mucosa associated lymphoid tissue (MALT) lymphomas are non-encapsulated clusters of lymphocytes found throughout the mucosal tissues of the aero-digestive tract. The non-Hodgkin type lymphomas that arise from these lymphocyte aggregates (MALT lymphoma) are of B-cell lineage, the commonest involving the salivary glands [1]. A MALT lymphoma has been presumed to be associated with autoimmune or inflammatory dis- eases [2]. The benign lymphoepithelial lesion (BLL) is also a lymphoproliferative disease that develops in the parotid gland. Although, BLL is a benign disease, subse- quent malignancies have also been reported [3,4]. The lymphoma arising from MALT was first described by Isaacson and Wright in 1983 [5]. MALT lymphoma arising from salivary glands is a rare entity; available data in the literature are scarce, confined to small series and isolated case reports. The characteristics and clinical outcome of this unusual presentation are largely unknown [6]. Early diagnosis relies on a high index of suspicion. * Correspondence: petros_konofaos@yahoo.com 2nd Department of Propedeutic Surgery, ‘LAIKO’ General Hospital 17, Ag Thomas Street, Athens 11527, Greece In the present case report, we describe one case of BLL with a subsequent low transformation to grade MALT lymphoma appearing as a cystic mass in the par- otid gland. Case Presentation A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient was otherwise asymptomatic. She had no history of malignancy or autoimmune diseases. A firm mobile mass was present in the left parotid gland. Examination revealed a 5 cm firm mobile mass in the superficial lobe of the left parotid. The left facial nerve was intact (House-Brackmann scale Grade I). No other abnormalities were found in the nasopharynx, oral cav- ity, larynx or ears. There was no pathological enlarge- ment of the cervical lymph nodes. Laboratory tests were within normal limits. Hepatitis B virus (HBV) and hepa- titis C virus (HCV) serologies were negative. The patient had undergone ultrasonography-guided fine needle aspiration of the left parotid gland several months before. The cytological examination revealed mononuclear and inflammatory cells and a diagnosis of © 2011 Konofaos et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.