Sarcoidosis of the submandibular gland: A systematic review Zacharias Vourexakis, MD a , Pavel Dulguerov, MD a , Salim Bouayed, MD a , Karim Burkhardt, MD b , Basile N. Landis, MD a, ⁎ a Department of Otolaryngology, Head and Neck Surgery, University of Geneva Medical School, University Hospitals of Geneva, Switzerland b Department of Clinical Pathology, University of Geneva Medical School, University Hospitals of Geneva, Switzerland Received 9 June 2009 Abstract Introduction: Submandibular gland sarcoidosis is rare and little is known about its clinical presentation besides the usual neck swelling. The aim of the study was to extract clinical knowledge on submandibular sarcoidosis from the literature. Methods: A systematic review was performed using a search in Medline with the key-words “sarcoidosis,”“submandibular,”“submaxillary.” Results: Forty-six articles fitting the search criteria were found, whereas 31 had to be excluded because they did not report submandibular gland sarcoidosis. Twenty cases of submandibular gland sarcoidosis were considered suitable for analysis. Almost all reported cases concerned female patients. In some cases submandibular gland's swelling is the first and only manifestation of the disease. Conclusion: Sarcoidosis should be considered in the differential diagnosis of all progressive and painless swellings of the submandibular gland, especially in women. Rarely, it may be the first manifestation of the disease. © 2010 Elsevier Inc. All rights reserved. 1. Introduction Sarcoidosis is a systemic granulomatous disease of unknown etiology. Almost all systems of the human body may be affected and there is a great variety in the presenting symptoms. Consequently, the range of otolaryngological manifestations of the disease is wide. Submandibular gland swelling is commonly seen in daily ear, nose, and throat outpatient clinics and the differential diagnosis ranges from infectious, traumatic, congenital, and malignant to inflam- matory causes. However, sarcoidosis in this gland is rather uncommon and more often seen in parotid swelling. Thus, there are no clinical characteristics which could draw the physician's attention to suspect more particularly sarcoido- sis. The present systematic review aimed to extract the available knowledge on submandibular gland sarcoidosis to identify possible common clinical presentation characteris- tics. Besides the reported cases in the literature, we also added a recent case of submandibular gland sarcoidosis treated in our department. 2. Material and methods 2.1. Systematic review: materials and methods A search was performed in MEDLINE (from 1950 to February 2009) using the terms: “sarcoidosis,”“submandib- ular,” “submaxillary,” and “gland.” These terms were combined using Boolean operators. Searches were restricted to English-, French-, or German-language articles. Reference lists from identified articles were searched and cross- referenced to obtain further relevant articles. The authors searched for articles of submandibular gland sarcoidosis independently and blinded from each other. Patients' characteristics such as age, sex, affected side and organs, symptoms, investigations, as well as treatment and evolution were extracted and recorded when available. In case of Available online at www.sciencedirect.com American Journal of Otolaryngology–Head and Neck Medicine and Surgery 31 (2010) 424 – 428 www.elsevier.com/locate/amjoto ⁎ Corresponding author. Department of Otolaryngology, Head and Neck Surgery, University of Geneva Medical School and Hospitals, Rue Micheli-du-Crest 24, CH-1211 Geneva, Switzerland. E-mail address: bnlandis@yahoo.co.uk (B.N. Landis). 0196-0709/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2009.08.001