Langerhans Cell Histiocytosis Presenting With Cardiac Tamponade: A Case Report

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.

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.

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. (Iranian Heart Journal 2022; 23(2): 142-149)