Fountas Athanasios, Chai Shu Teng, Ayuk John, Gittoes Neil, Chavda Swarupsinh, Karavitaki Niki
Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.
Endocrinol Diabetes Metab Case Rep. 2018 Mar 28;2018. doi: 10.1530/EDM-18-0018. eCollection 2018.
Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed.
Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely.Meticulous review of unexpected biochemical findings is vital for correct diagnosis of dual pituitary pathology.The potential adverse impact of GH excess due to acromegaly in a patient with craniopharyngioma (and other neoplasm) mandates adequate biochemical control of the GH hypersecretion.
颅咽管瘤与肢端肥大症并存的情况鲜有报道。一名65岁男性出现视力减退、疲劳和前额头痛。磁共振成像显示鞍上有一个大小为1.9×2×1.9厘米的异质性肿块，主要为囊性，压迫视交叉并延伸至第三脑室；这些表现符合颅咽管瘤。垂体激素检查显示低促性腺激素性性腺功能减退、催乳素轻度升高、胰岛素样生长因子1（IGF-1）升高，甲状腺功能和皮质醇储备正常。患者接受了经蝶窦手术，标本病理诊断为造釉细胞瘤型颅咽管瘤。术后，他出现了尿崩症、低促性腺激素性性腺功能减退以及促肾上腺皮质激素和促甲状腺激素缺乏。尽管存在垂体功能减退，但其IGF-1水平仍升高，随后的口服葡萄糖耐量试验未显示生长激素（GH）完全被抑制。进一步复查术前影像发现一个靠近右侧颈动脉的12×4毫米垂体腺瘤，且无垂体增生迹象。当时，他最终还被诊断出左上肺叶鳞状细胞癌，并接受了根治性放疗。开始使用长效生长抑素类似物治疗，使肢端肥大症得到生化控制。最新影像显示没有颅咽管瘤复发的迹象，腺瘤稳定。这是一例颅咽管瘤、肢端肥大症和肺鳞状细胞癌并存的独特病例报告，突出了诊断和管理方面的挑战。还简要讨论了GH分泌过多对该患者并存肿瘤的潜在影响。
尽管是极为罕见的临床情况，但颅咽管瘤和肢端肥大症可并存；这两种情况之间不太可能存在病因学联系。对意外的生化检查结果进行细致复查对于正确诊断双重垂体病变至关重要。肢端肥大症导致的GH过多对患有颅咽管瘤（及其他肿瘤）的患者可能产生的不利影响，要求对GH分泌过多进行充分的生化控制。
