Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis

Abstract

Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.

Keywords: Langerhans cell histiocytosis; biopsy; orbital tumors; spontaneous regression.

Figure 1.

Cerebral MRI in coronal, sagittal and axial section showing a superolateral extraconal tissular mass extending through the superior orbital fissure and along the dura mater measuring 36.6 mm x 32.7 mm.

Figure 2.

(a) Langerhans cell histiocytosis showing intermediate-sized cells with grooved, indented and lobed nuclei and pale eosinophilic cytoplasm surrounded by large numbers of eosinophils (HEx400). Langerhans cell histiocytosis with S100 (b) and CD1a (c) immunoreactivity on the majority of cells (IHCx400).

Figure 3.

Orbital MRI revealing no abnormalities 9 months after diagnosis.