Langerhans Cell Histiocytosis of the Temporal Bone in Children: Two Cases Report

Y Labani,O Oulghoul,O Benhoummad,A Raji,Y Rochdi

doi:10.24018/ejmed.2021.3.3.839

Abstract

Langerhans cell histiocytosis is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. We report 2 cases confirmed by Histological and immunohistochemical evaluation of temporal bone biopsies. The first case is a 2-year-old child with chronic bilateral otorrhea and polyuria-polydipsia syndrome. Physical examination showed bilateral retroauricular redness and induration with filling all of the right and left external ear canal. Computed tomography showed osseous lysis of the temporal bone bilaterally. Magnetic resonance imaging of the brain showed the presence of pituitary infiltration. The second case is 2-year-old girl with chronic left otorrhea. Clinical examination objectified the presence of redness and induration in the left mastoid with the presence of an inflammatory polyp filling the entire left external auditory canal. Computed tomography revealed a left temporal bone lysis. Both patients were treated with chemotherapy.

Highlights

Langerhans cell histiocytosis (LCH) is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells [1]
Histological study objectified the presence of a fibrous tissue seat of a cellular infiltrate made up of inflammatory cells and histiocytic cells (Fig. 3), Immuno-markers were positive for PS100, CD1a and CD68 confirming the diagnostic of LCH
Clinical examination objectified the presence of redness and induration in the left mastoid with the presence of an inflammatory polyp filling the entire left external auditory canal

Summary

INTRODUCTION

Langerhans cell histiocytosis (LCH) is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells [1]. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. We report two cases: a 2-year-old child with bilateral temporal and pituitary involvement and a 2year-old girl with unilateral left temporal involvement. Showed the presence of pituitary infiltration with enlargement of the pituitary stem enhancing to T1 sequences after injection of contrast agent (Fig. 1). Computed tomography (CT) revealed bone lysis involving the temporal bone bilaterally (Fig. 2). Histological study objectified the presence of a fibrous tissue seat of a cellular infiltrate made up of inflammatory cells and histiocytic cells (Fig. 3), Immuno-markers were positive for PS100, CD1a and CD68 confirming the diagnostic of LCH

CLINICAL CASE 1

DISCUSSION

Findings

CONCLUSION