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. 2006;15(3):85-91.
doi: 10.1297/cpe.15.85. Epub 2006 Aug 2.

Two children with xanthogranuloma of the sellar region

Toshihiro Tajima  1 , Yutaka Sawamura  2 , Katsura Ishizu  1 , Jyunko Tsubaki  1
Affiliations

Two children with xanthogranuloma of the sellar region

Toshihiro Tajima et al. Clin Pediatr Endocrinol. 2006.

Abstract

We report the cases of two Japanese children with cystic pituitary enlargement on magnetic resonance imaging (MRI) causing central diabetes insipidus (DI). In the first patient, endocrinological examination demonstrated slightly impaired growth hormone and thyroid stimulating hormone secretions, but normal responses of other anterior pituitary hormones. The second patient had normal basal levels of anterior pituitary hormones. Transsphenoidal resection of the tumors was performed in both patients. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, foamy macrophages, multinucleated giant cells and no epithelial component. Thus, these tumors were pathologically diagnosed as xanthogranuloma of the sellar region, different from adamantinomatous craniopharyngioma. Post-operatively, the two patients continue to have DI, however other hormone replacement therapy after one year of follow-up has not been required. Currently, it is not clear whether xanthogranuloma is a distinct entity from adamantinomatous craniopharyingioma. Although, to our knowledge, a clinical report of xanthogranuloma of the sellar region has not been reported at pediatric age, it would be included in the differential diagnosis of the sellar region.

Keywords: craniopharyngioma; pituitary; xanthogranuloma.

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Figures

Fig. 1
Fig. 1
Radiological appearance on magnetic resonance imaging (MRI). MRI demonstrated an intrasellar mass with well defined borders. The mass was hyperintense on the T1-weighted (A) and in the T2-weighted images (B). Gadolinium administration did not enhance the mass.
Fig. 2
Fig. 2
Histological findings of the tumor in Case 1. (A) Cholesterol clefts (arrows) were observed. No epithelial tissue was found. × 100. (B) Macrophages (white arrowheads) and multinucleated giant cells (black arrowheads) were observed. × 200.
Fig. 3
Fig. 3
Radiological appearance on MRI. The mass had a high appearance on the T1-weighted images (A). T2-weighted image showed a hypointense mass (B). Gadolinium administration caused no enhancement of the mass.
Fig. 4
Fig. 4
Histological findings of the tumor in Case 2. (A) Cholesterol clefts (arrows) were observed. (B) Macrophages (black arrowheads) were observed. × 200.
See this image and copyright information in PMC

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