Langerhans cell histiocytosis of the sella in a pediatric patient: case report with review of the literature

Childs Nerv Syst. 2024 Sep;40(9):2947-2952. doi: 10.1007/s00381-024-06456-y. Epub 2024 May 22.

Abstract

Purpose: Langerhans cell histiocytosis (LCH) is a rare condition arising from the monoclonal expansion of myeloid precursor cells, which results in granulomatous lesions that characteristically express CD1a/CD207. We report a case of LCH in a 3-year-old male involving the sphenoid bone with extension into the sellar/suprasellar region.

Case report: A 3-year-old male presented with progressively worsening headaches and associated night sweats, neck stiffness, and fatigue over the previous 4 weeks. Magnetic resonance imaging (MRI) revealed a 2.4-cm lytic lesion within the basisphenoid, exerting mass effect upon the pituitary gland. A biopsy was performed to determine the etiology of the lesion. Postoperatively, the patient developed an intralesional hematoma with visual complications requiring emergent surgical resection via endoscopic endonasal approach. Final pathology confirmed LCH. The patient had improvement in his vision long term.

Conclusions: LCH extending into the sella is a rare but important diagnosis to consider in pediatric patients presenting with lesions in this region. We presented a case of a pediatric patient presenting with LCH of the sphenoid bone extending into the sella, with subsequent apoplexy and vision loss. Review of the literature showed varying treatment options for these patients, including purely surgical and non-surgical treatments. Early intervention may be necessary to avoid potentially devastating neurologic sequelae.

Keywords: Endoscopic endonasal; Langerhans cell histiocytosis; Sella; Skull base.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child, Preschool
  • Histiocytosis, Langerhans-Cell* / complications
  • Histiocytosis, Langerhans-Cell* / diagnostic imaging
  • Histiocytosis, Langerhans-Cell* / pathology
  • Histiocytosis, Langerhans-Cell* / surgery
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Sella Turcica / diagnostic imaging
  • Sella Turcica / pathology
  • Sella Turcica / surgery
  • Sphenoid Bone / diagnostic imaging
  • Sphenoid Bone / pathology
  • Sphenoid Bone / surgery