Langerhans Cell Histiocytosis: a Differential Diagnosis of Anterior Mediastinal Tumors in Children

Abstract

Introduction: Langerhans cell histiocytosis (LCH) is a heterogeneous group of disorders characterized by monoclonal proliferation of dendritic cells. With an incidence rate of 5 to 10 cases / million / year, it presents unknown aetiology and a broad clinical spectrum, manifesting itself mainly through skin and bone lesions. It predominates in male children under 15 years old. Case report: We reported the case of a female child with 4 years old who had a large mass in the anterior mediastinum, attached to the great vessels of the heart. Biopsy of the lesion suggested LCH, which was confirmed by immunohistochemistry, positive for the markers CD1a and S100. On further investigation, it was also observed cutaneous involvement for the disease. Conclusion: The lymph node involvement by LC H occurs only in 5-10% of the cases. By affecting the mediastinal lymph nodes, it can result in tumors of varying sizes. In such cases, the diagnosis is a challenge considering the large number of diseases that are manifested by mediastinal masses. Thus, we emphasize the importance of including the LCH in the differential diagnosis of mediastinal masses in children.