Iranian Heart Association Iranian Heart Journal 23 2 2022 04 01 Langerhans Cell Histiocytosis Presenting With Cardiac Tamponade: A Case Report 142 149 147119 EN Kumaresh Jeyaraman Hariram Kasturba Medical College, Manipal University of Higher Education, Manipal, India. 0000-0002-4038-7268 Ashwal Adamane Jayaram Kasturba Medical College, Manipal University of Higher Education, Manipal, India. 0000-0002-6975-974X M Sudhakar Rao Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Karnataka, India. 0000-0001-9439-3703 Abdul Razak Uddina Kumeri Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Karnataka, India. 0000-0002-1853-7725 Krishnananda Nayak Department of Cardiovascular Technology, School of Allied Health Science (SOAHS), Manipal Academy of Higher Education, Manipal, India. 0000-0001-7376-0834 Umesh M Pai Department of Cardiovascular Technology, School of Allied Health Science (SOAHS), Manipal Academy of Higher Education, Manipal, India. Harsha M Sagar Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Karnataka, India. Journal Article 2021 11 15 <strong><em>Background:</em></strong> Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with an unknown etiology. We herein present an LCH case with cardiac tamponade and the oral cavity involvement to raise awareness of the distinguishing features of this diagnostically challenging entity. <strong><em>Case Report:</em></strong> This study describes a 52-year-old man who presented with dyspnea (NYHA functional class II) and bilateral pedal edema of 4 days’ duration. A clinical examination revealed a large pericardial effusion with cardiac tamponade. A positron emission tomography-computed tomography scan showed a heterogeneously enhancing soft tissue lesion, 2.6×1.7×1.6 cm in size, in the right mid-submandibular region. An intraoral examination confirmed the right submandibular mass. The biopsy of the submandibular gland mass confirmed the diagnosis of LCH. <strong><em>Conclusions:</em></strong> LCH is often diagnosed in childhood but may manifest in any age group, from infancy to adulthood. Pericardial effusion is a rare finding in this case of LCH. The rarity and the variable system involvement of LCH necessitate a multidisciplinary approach for accurate diagnosis and effective treatment. <strong><em>(Iranian Heart Journal 2022; 23(2): 142-149)</em></strong> https://journal.iha.org.ir/article_147119_c147cc04c5ac79a251a3f999dcb910ae.pdf