Abstract：
BACKGROUND: Pasireotide is a multireceptor somatostatin analogue approved for the treatment of patients with Cushing\'s disease (CD) who are ineligible or poor candidates for pituitary surgery. Here we present a patient with severe recurrent CD who was treated with pasiretide and showed opposite results between hormonal levels and pituitary tumour size.
METHODS: A 54-year-old woman was diagnosed with CD, a first surgical transsphenoidal procedure was performed at the time of diagnosis, and the disease recurred seven years later. She underwent a second transsphenoidal surgery, but despite apparent complete removal of the adenoma, the hypercortisolism worsened. Magnetic resonance imaging showed a tiny remnant of the adenoma adjacent to the cavernous sinus, and ketoconazole was started at a dose of 800 mg/day. Due to the persistence of pathological urinary free cortisol levels, 600 μg bid pasireotide was added. The combination therapy induced first normalisation of urinary free cortisol and later hypoadrenalism, so that ketoconazole was discontinued and pasireotide was maintained. A marked clinical improvement was achieved with pasireotide. Adrenal insufficiency persisted despite progressive tapering of the pasireotide dose to 150 mg once daily. Pituitary magnetic resonance imaging performed at 12 and 24 months during low-dose pasireotide treatment showed a few millimetres increase of the remnant.
CONCLUSIONS: This report suggests that CD Pas induces an opposite effect between hormonal profile and increase of pituitary tumor size. This peculiar phenomenon may be a consequence of the unusually low doses of pasireotide needed to control hormonal hypersecretion.
METHODS: A 54-year-old woman was diagnosed with CD, a first surgical transsphenoidal procedure was performed at the time of diagnosis, and the disease recurred seven years later. She underwent a second transsphenoidal surgery, but despite apparent complete removal of the adenoma, the hypercortisolism worsened. Magnetic resonance imaging showed a tiny remnant of the adenoma adjacent to the cavernous sinus, and ketoconazole was started at a dose of 800 mg/day. Due to the persistence of pathological urinary free cortisol levels, 600 μg bid pasireotide was added. The combination therapy induced first normalisation of urinary free cortisol and later hypoadrenalism, so that ketoconazole was discontinued and pasireotide was maintained. A marked clinical improvement was achieved with pasireotide. Adrenal insufficiency persisted despite progressive tapering of the pasireotide dose to 150 mg once daily. Pituitary magnetic resonance imaging performed at 12 and 24 months during low-dose pasireotide treatment showed a few millimetres increase of the remnant.
CONCLUSIONS: This report suggests that CD Pas induces an opposite effect between hormonal profile and increase of pituitary tumor size. This peculiar phenomenon may be a consequence of the unusually low doses of pasireotide needed to control hormonal hypersecretion.
摘要：
背景：帕瑞肽是一种多受体生长抑素类似物，被批准用于治疗不适合或不适合垂体手术的库欣病（CD）患者。在这里，我们介绍了一名患有严重复发性CD的患者，该患者接受了帕瑞肽治疗，并且在激素水平和垂体肿瘤大小之间显示出相反的结果。
方法：一名54岁女性被诊断患有CD，在诊断时进行了第一次手术经蝶窦手术，这种疾病七年后复发。她做了第二次经蝶窦手术,但是尽管腺瘤明显完全切除，皮质醇增多症恶化。磁共振成像显示海绵窦附近有微小的腺瘤残留，酮康唑以800mg/天的剂量开始。由于病理性尿游离皮质醇水平的持续存在，加入600μgbidpasireotide。联合疗法首先引起尿游离皮质醇正常化，然后引起肾上腺功能减退，因此停用酮康唑并维持帕瑞肽。pasireotide取得了明显的临床改善。尽管帕瑞肽的剂量逐渐减少至每天150mg，但肾上腺功能不全仍然存在。在低剂量pasireotide治疗期间，在12个月和24个月进行的垂体磁共振成像显示残留物增加了几毫米。
结论：本报告表明，CDPas在激素分布和垂体肿瘤大小增加之间产生相反的作用。这种特殊现象可能是控制激素分泌过多所需的帕瑞肽异常低剂量的结果。
方法：一名54岁女性被诊断患有CD，在诊断时进行了第一次手术经蝶窦手术，这种疾病七年后复发。她做了第二次经蝶窦手术,但是尽管腺瘤明显完全切除，皮质醇增多症恶化。磁共振成像显示海绵窦附近有微小的腺瘤残留，酮康唑以800mg/天的剂量开始。由于病理性尿游离皮质醇水平的持续存在，加入600μgbidpasireotide。联合疗法首先引起尿游离皮质醇正常化，然后引起肾上腺功能减退，因此停用酮康唑并维持帕瑞肽。pasireotide取得了明显的临床改善。尽管帕瑞肽的剂量逐渐减少至每天150mg，但肾上腺功能不全仍然存在。在低剂量pasireotide治疗期间，在12个月和24个月进行的垂体磁共振成像显示残留物增加了几毫米。
结论：本报告表明，CDPas在激素分布和垂体肿瘤大小增加之间产生相反的作用。这种特殊现象可能是控制激素分泌过多所需的帕瑞肽异常低剂量的结果。
