Resolution and Re-ossification of Orbital-Wall Langerhans Cell Histiocytosis Following Stereotactic Needle Biopsy

Abstract

Introduction Langerhans cell histiocytosis (LCH) is a rare disease that encompasses a spectrum of clinical syndromes. It is characterized by the proliferation and infiltration of white blood cells into organs or organ systems. Reports of management of these lesions have included biopsy, resection, curettage, radiation, and/or chemotherapy. Case Presentation A 40-year-old man presented with a history of right proptosis and retro-orbital pain and was found to have a lytic mass involving the greater wing of the sphenoid extending into the right orbit. A stereotactic needle biopsy using neuronavigation demonstrated this to be LCH. After no further treatment, the mass spontaneously resolved, with virtual normalization of the orbital magnetic resonance imaging at 10 months following the needle biopsy. The bony defect of the temporal bone caused by the mass also re-ossified following the needle biopsy. Discussion This report highlights the potential for an isolated LCH lesion to regress after simple needle biopsy, an outcome only rarely reported previously. Thus, expectant management of such lesions following biopsy or initial debridement should be considered prior to proceeding with additional treatment.

Keywords: Langerhans cell histiocytosis; biopsy; re-ossification; resolution; stereotactic.

Fig. 1

Preoperative MR axial fat saturation T1-weighted postcontrast ( A ) and CT ( B ) images demonstrating the mass in the right greater wing of the sphenoid. Postoperative axial T1-weighted MR image 3 months after the initial biopsy ( C ), and CT scan 17 months after biopsy showing complete re-ossification of the bony architecture ( D ). CT, computed tomography; MR, magnetic resonance.

Fig. 2

( A ) Diff-Quik stained aspirate smears at 60× magnification shows a cellular smear with a mixed inflammatory infiltrate, conspicuous eosinophils, occasional multinucleated giant cells, and abundant bland histiocytoid cells with moderate cytoplasm and oval to bean-shaped nuclei with nuclear grooving. ( B ) Similar features are seen on H&E-stained cell block (60 × ) and immunohistochemical stains performed on the cell block show positive staining of the histiocytoid cells with CD1a ( C ). H&E, hematoxylin and eosin.