Unit of Endocrinology, and Diabetes Center, 'G. Gennimatas' General Hospital of Athens, Athens, Greece.
Department of Endocrinology, University Hospital of Ioannina, Ioannina, Greece.
Endocrine. 2022 Dec;78(3):570-579. doi: 10.1007/s12020-022-03182-w. Epub 2022 Sep 8.
Pheochromocytomas are rare tumors and biochemically silent ones with normal catecholamine levels are even rarer. Up to date, biochemically inactive pheochromocytomas are poorly investigated. We aimed to systematically assess the pre- and peri-operative characteristics and the outcomes of patients with these tumors who had been treated and followed-up in 2 tertiary centers.
Clinical, laboratory and imaging data, treatment outcomes and follow-up of biochemically silent pheochromocytoma patients were recorded.
Ten patients (5 men) [median age at diagnosis 52.5 years (24-72)] were included. Adrenal masses were incidentally discovered in all patients except from one who presented with pheochromocytoma-related manifestations. Twenty-four-hour urine metanephrine and normetanephrine levels were in the low-normal, normal and high-normal range in 4, 4 and 2 patients and in 1, 6 and 3 patients, respectively. Tumors were unilateral [median size 46 mm (17-125)] and high density on pre-contrast CT imaging or high signal intensity on T2-weighted MRI scans were found in all cases. Pre-operatively, 5 patients were treated with phenoxybenzamine [median total daily dose 70 mg (20-100)]. Intra-operatively, 4 patients developed hypertension requiring vasodilator administration and 8 developed hypotension; vasoconstrictors were required in 5 cases. One patient, not pre-operatively treated with phenoxybenzamine, developed Takotsubo cardiomyopathy. During a median 24-month (12-88) follow-up period, one patient had disease progression.
The majority (90%) of patients with biochemically silent pheochromocytomas developed hemodynamic instability during adrenal surgery. In patients with biochemically silent adrenal lesions and a high suspicion index for pheochromocytoma based on tumor imaging characteristics, pre-operative alpha-blockade treatment may be advisable.
嗜铬细胞瘤是一种罕见的肿瘤，而正常儿茶酚胺水平的生化无活性型嗜铬细胞瘤则更为罕见。迄今为止，生化无活性型嗜铬细胞瘤的研究还不够充分。我们旨在系统评估在 2 家三级中心接受治疗和随访的此类肿瘤患者的术前、术中和围手术期特征以及治疗结果。
记录了生化无活性型嗜铬细胞瘤患者的临床、实验室和影像学数据、治疗结果和随访情况。
共纳入 10 名患者（5 名男性）[诊断时的中位年龄为 52.5 岁（24-72 岁）]。除 1 例因嗜铬细胞瘤相关表现就诊的患者外，其余所有患者均为偶然发现肾上腺肿块。24 小时尿甲氧基肾上腺素和去甲氧基肾上腺素水平在 4 例患者中处于低正常值、4 例患者中处于正常值、2 例患者中处于高正常值范围，而在 1 例患者中、6 例患者中、3 例患者中分别处于正常值、高正常值和高正常值范围。所有病例的肿瘤均为单侧[中位大小为 46mm（17-125mm）]，且在 CT 平扫时为高密度或在 T2 加权 MRI 扫描时为高信号强度。术前，5 名患者接受了酚苄明治疗[中位总日剂量为 70mg（20-100mg）]。术中，4 名患者发生高血压需要血管扩张剂治疗，8 名患者发生低血压；5 例患者需要使用血管收缩剂。1 名未接受术前酚苄明治疗的患者发生 Takotsubo 心肌病。在中位 24 个月（12-88 个月）的随访期间，1 名患者出现疾病进展。
大多数（90%）生化无活性型嗜铬细胞瘤患者在肾上腺手术期间出现血流动力学不稳定。对于基于肿瘤影像学特征高度怀疑嗜铬细胞瘤的生化无活性型肾上腺病变患者，术前接受α受体阻滞剂治疗可能是明智的。
