B4 Office, Department of Neurosurgery, University Hospital of Wales, Cardiff, CF 14 4XW, UK.
Department of Endocrinology, University Hospital of Wales, Cardiff, CF 14 4XW, UK.
Pituitary. 2018 Jun;21(3):256-265. doi: 10.1007/s11102-017-0859-x.
Xanthogranulomas 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.
A 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.
During 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.
The 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.
黄色肉芽肿是最常见于皮肤等外周部位的炎症性肿块。鞍上和鞍旁黄色肉芽肿罕见，术前难以与颅咽管瘤和 Rathke 裂囊肿等其他鞍内病变区分，诊断具有挑战性。其影像学表现尚未明确，术后临床结果也不确定。本研究回顾了一组垂体黄色肉芽肿患者的临床表现、影像学表现和临床结果。
2011 年 5 月至 2016 年 12 月，通过前瞻性维持的垂体手术数据库筛选出经组织学证实的垂体黄色肉芽肿患者。然后由三名独立审查员对病历进行回顾性评估。分析患者的人口统计学资料、临床表现、影像学表现和临床结果。
研究期间共进行了 295 例内镜经鼻垂体手术。6 例患者被证实患有垂体黄色肉芽肿（2%）。患者最常见的表现为视野缺损和/或内分泌功能障碍。常见的影像学特征包括：囊性一致性、T1 加权磁共振成像上的高信号强度，以及外周（n=3）或均匀（n=3）强化。最常见的术前内分泌缺陷是高泌乳素血症和肾上腺功能减退（至少有一项在 6/6 例患者中被识别；66%）。33%（2/6）的患者出现尿崩症。最常见的术后内分泌缺陷是肾上腺皮质功能障碍（66%）和促性腺激素缺乏（66%）。所有患者术后视力均恢复正常。所有患者均达到大体全切除，中位随访 33.5 个月后无肿瘤复发病例。
本研究中，垂体黄色肉芽肿的患病率高于文献报道。手术使所有病例的视力恢复正常，但 4 例患者（67%）术后需要长期激素替代治疗。外周边缘强化、鞍上肿瘤中心、无钙化或海绵窦侵犯等影像学特征被认为是潜在的指标，这些指标结合起来，在评估囊性鞍内和鞍旁肿瘤的患者时，应能提醒临床医生注意垂体黄色肉芽肿的可能性。
