Novel multidisciplinary approach for treatment of langerhans cell histiocytosis of the skull base

Abstract

Langerhans cell histiocytosis frequently manifests as lesions of the skull. The disease can present with a wide spectrum of forms, from an isolated eosinophilic granuloma to multiple lesions with diffuse systemic involvement. The authors report the case of a 12-year-old boy with a 1-month history of left temporal and periorbital pain and headaches. Noncontrast computed tomography of the head was done at the time of initial presentation and was interpreted as normal. Over the next month, the patient continued to have headaches and periorbital swelling and began having bloody discharge from his nose. Magnetic resonance imaging of the brain ordered by his pediatrician showed a lesion in the left infratemporal fossa, left orbit, and sphenoid bone. The lesion was biopsied and confirmed to be Langerhans cell histiocytosis. We describe a novel multidisciplinary approach for treatment of this tumor.

Keywords: Langerhans cell histiocytosis; eosinophilic granuloma; skull base tumor.

Figure 1

Initial noncontrast axial head computed tomography (CT) bone windows show only minimal destruction of the left lateral orbital wall. This CT was initially interpreted as negative for any lesions when the patient presented with left temporal and periorbital pain.

Figure 2

(A) Axial and (B) coronal computed tomography scans obtained 1 month later show an expansile lesion with destruction of the left lateral orbit and sphenoid sinuses.

Figure 3

Preoperative magnetic resonance imaging of the brain. (A) Axial, (B) sagittal, and (C) coronal T1 gadolinium-enhanced images show an enhancing lesion in the left infratemporal fossa with extension into the left orbit and sphenoid sinuses.

Figure 4

(A) Axial and (B) coronal postoperative enhanced magnetic resonance imaging of the brain shows gross total resection of the intracranial portion of the tumor and tumor within the sphenoid sinuses and lateral orbit.

Figure 5

Histological sections show sheets of histiocytoid mononuclear cells with moderate amounts of eosinophilic cytoplasm and pale staining nuclei. Occasional foamy histiocytes and scattered multinucleated giant cells and reniform nuclei are visible. (A) Hematoxylin and eosin (H & E) staining at × 20; (B) H & E staining at × 100; (C) CD1a staining at × 20 shows strong membranous staining of histiocytic cells.