Pituitary xanthogranulomas: clinical features, radiological appearances and post-operative outcomes

Abstract

BackgroundXanthogranulomas are inflammatory masses most commonly found at peripheral sites such as the skin. Sellar and parasellar xanthogranulomas are rare and present a diagnostic challenge as they are difficult to differentiate from other sellar lesions such as craniopharyngiomas and Rathke’s cleft cysts pre-operatively. Their radiological imaging features are yet to be clearly defined, and clinical outcomes after surgery are also uncertain. This study reviews clinical presentation, radiological appearances, and clinical outcomes in a cohort of patients with pituitary xanthogranulomas.MethodsA prospectively maintained pituitary surgery database was screened for histologically confirmed pituitary xanthogranulomas between May 2011–December 2016. Retrospective case note assessments were then performed by three independent reviewers. Patient demographics, clinical presentations, imaging, and clinical outcomes were analysed.ResultsDuring the study period 295 endoscopic endonasal pituitary surgeries were performed. Six patients had confirmed pituitary xanthogranulomas (2%). Patients most commonly presented with visual field deficits and/or endocrine dysfunction. Common imaging features included: a cystic consistency, hyperintensity on T1-weighted MR images, and contrast enhancement either peripherally (n = 3) or homogenously (n = 3). The most common pre-operative endocrine deficits were hyperprolactinaemia and hypoadrenalism (at least one of which was identified in 4/6 patients; 66%). Thirty-three percent (2/6) of patients presented with diabetes insipidus. The most common post-operative endocrinological deficits were adrenocortical dysfunction (66%) and gonadotropin deficiency (66%). Visual assessments normalised in all six patients post-operatively. Gross total resection was achieved in all patients, and at median follow up of 33.5 months there were no cases of tumour recurrence.ConclusionsThe prevalence of pituitary xanthogranulomas in our series is higher than that suggested in the literature. Surgery restored normal vision to all cases, however four patients (67%) required long-term hormonal replacement post-operatively. Imaging features such peripheral rim enhancement, a suprasellar tumour epicentre, and the absence of both calcification or cavernous sinus invasion were identified as potential indicators that together should alert clinicians to the possibility of pituitary xanthogranuloma when assessing patients with cystic sellar and parasellar tumours.

From Diabetes Insipidus to Sellar Xanthogranuloma – a “Yellow Brick Road” Demanding Team-Work

“…Sellar region xanthogranulomas (SXG) are exceptionally rare (3). SXG prevalence in large surgical cohorts was 0.6-2% in adults, reaching 10% in children (4)(5)(6)(7). Mean presenting age was 36.6 years, with a female predominance of up to 60% (3).…”

“…SXG can be complicated by CDI in up to 30%, but rarity of SXG makes it an overall exceptionally rare cause of CDI often not considered in differential diagnosis. A total of only 9 SXG adults associated with CDI at presentation have been reported in literature (2)(3)(4)(10)(11)(12).…”

“…In addition, MRI findings may evolve over the course of SXG progression. SXG are most often intra-suprasellar (67%), with suprasellar origin, less frequently only suprasellar (22%) and rarely exclusively intrasellar (11%) (3,4). Suprasellar extension is more prevalent in children (92%) including hypothalamic involvement in 46% (7).…”

“…In most cases a T1-weighted (T1w) hyperintensity is described (in 83%) -probably corresponding to cystic content and cholesterol clefts. T2-weighted (T2w) intensity is heterogeneous in 43% -particularly as necrosis and hemorrhage occur (3,4). A fluid-debris level may appear at the lesion`s depending portion, marking interface between high signal xanthogranulomatous component and hypointense necrotic debris (15).…”

Diabetes Insipidus: Little-Known Facts

“…За период 33,5 мес не отмечалось случаев рецидива опухоли. Таким образом, усиление контрастности периферической зоны или эпицентра супраселлярной опухоли, отсутствие кальцификации и инвазии кавернозного синуса были расценены как потенциальные индикаторы, которые должны ориентировать врача на поиск ксантогранулем гипофиза при выявлении кистозных изменений и параселлярной опухоли [33].…”