gms | German Medical Science

Objective: Xanthogranuloma of the sellar region is classified as a distinct entity only since 1999. Thus far, few cases have been documented in the literature. Therefore, knowledge about the pathogenesis and key-features of this unusual entity is limited. Here we report about our experience in the surgical management of 3 cases.

Methods: All patients were operated from December 2008 until September 2009 using microsurgical techniques, in one case transcranial and in two cases transphenoidal. The mean age of the patients was 49 years (28–65 years). The preoperative radiological studies included cMRI and cCT scan in all cases. The clinical follow-up time was 3–6 months for all patients (mean follow-up time 4 months). Histopathologicaly, in all 3 cases cholesterin-clefts with hemosiderin deposits and lymphoplasmacellular infiltration, foreign-body giant cells, focal hemorrhages and in two cases calcification, foam cells and epithelium with partly ciliated cells were observed.

Results: The patients presented with progressive visual deficit, polyuria and polydipsia, chronic headache and finally signs of anterior pituitary dysfunction. Regarding the radiological features all tumors appeared hyperintense on T1-weighted images (100%), hypointense or heterogeneous on T2-weighted images (78%). Postoperatively one patient developed transient diabetes insipidus. Hormonal substitution could be withdrawn by one patient or reduced by two patients after 3 months. Visual deficits improved.

Conclusions: Xanthogranulomas differ from other lesions of the sellar region considering specific clinical and pathological features. Typical radiological findings are usually found on MR imaging. A suprasellar location with chiasmal involvement or affection of the pituitary stalk has not been reported so far. Xanthogranulomas are rare lesions which may present in the sellar or suprasellar region and need to be considered as differential diagnosis. The pathogenesis is still unclear.