Juvenile Xanthogranuloma of the Clivus

Objectives: Juvenile xanthogranuloma (JXG) is a form of non-Langerhans histiocytosis that typically presents within the first several years of life as solitary, painless cutaneous lesions. While lesions not involving the skin and subcutaneous tissues account for only 4–5% of cases, they are associated with substantially greater morbidity. Although spontaneous regression is commonly seen, diagnosis and treatment of these lesions allows for alleviating symptoms related to mass effect and disseminated disease. Here, we describe an unusual case of JXG of the clivus.

Study Design: Case report.

Methods: The clinical and radiographic presentation, operative technique, and histopathological analysis are presented of a patient referred to a tertiary medical center with a soft tissue mass centered around the clivus.

Results: A 10-year-old female was referred to the head and neck surgery clinic with a 1-year history of headaches and ear pain. Computed tomography and magnetic resonance imaging revealed an erosive 2.2 cm clival mass with extension into bilateral sphenoid sinuses (Fig. 1), suggesting a clival chordoma. An endonasal approach was employed to perform sphenoidectomy and partial clivectomy with resection of the mass. Pathologic examination revealed extensive histiocytic infiltration, positive for CD68 and CD163 and negative for S-100 and CD1a. Also noted were Touton giant cells, seen in lesions with high lipid content. Together, this indicated a mature non-Langerhans histiocytic proliferation consistent with JXG. No further therapy was recommended.

Conclusions: JXG affecting the axial skeleton is remarkably infrequent and, to our knowledge, this is the first report of JXG involving the clivus. The natural history usually involves spontaneous regression, but symptomatic lesions should be actively managed. Treatment ranges from surgical resection to external beam radiation and chemotherapy. In this case, an endonasal approach provided a minimally invasive means for surgical resection, and is favored for surgical management of lesions in this area.

Fig. 1 Axial T1-weighted magnetic resonance imaging (MRI), following gadolinium contrast administration, reveals an enhancing, destructive lesion centered at the clivus.