目的:研究神经内分泌肿瘤(NENs)患者原发性肾上腺肿瘤和肾上腺转移的患病率,并对其进行详细描述。NENs可进一步分为神经内分泌肿瘤(NET)和神经内分泌癌(NEC)。
方法:对2010-2023年在卡罗林斯卡大学医院接受68镓-DOTATOC-PET/CT或1999-2023年进行肾上腺切除术的所有患者进行了医学档案审查。
结果:总计,对1750例NEN患者进行了68镓-DOTATOC-PET/CT,其中12例(0.69%)有肾上腺肿瘤。其中,9例(0.51%)为NEN转移。在1072例肾上腺切除术中,4(0.37%)显示NEN转移的证据。因此,16例NEN患者表现为肾上腺肿瘤。NEN诊断后平均5年发现肾上腺肿瘤,同时发生NEN的肾上腺肿瘤中有19%是良性的。很少有人测量所有肾上腺激素。没有肾上腺功能不全或肾上腺活检。在发现肾上腺肿瘤时,发现了69%的同步转移。在平均2年随访期间,38%的受试者死亡(排除了据称表现为良性肾上腺肿瘤的个体31%),全部是由于肿瘤并发症。通过68镓-DOTATOC-PET/CT鉴定的个体与接受肾上腺切除术的个体之间的比较显示,前者的NETs患病率较高,后者的NECs患病率较高。
结论:NEN患者的肾上腺原发肿瘤和肾上腺转移很少发生。大多数病例在最初发现肾上腺肿瘤时涉及NEN转移的存在。发现总体预后良好。
OBJECTIVE: To study the prevalence of primary adrenal tumors and adrenal metastases in patients with neuroendocrine neoplasms (NENs) and describe these in detail. NENs can be further divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC).
METHODS: A review of medical files was conducted for all patients who underwent a 68Gallium-DOTATOC-PET/CT during 2010-2023 or adrenalectomy during 1999-2023 at the Karolinska University Hospital.
RESULTS: In total, 68Gallium-DOTATOC-PET/CT was performed on 1750 individuals with NEN, among whom 12 (0.69%) had adrenal tumors. Of these, 9 (0.51%) were NEN metastases. Out of 1072 adrenalectomies, 4 (0.37%) showed evidence of NEN metastases. Thus, 16 patients with NEN exhibited adrenal tumors. The adrenal tumors were found on average 5 years after the NEN diagnosis and 19% of the adrenal tumors with simultaneous NEN were benign. Few had all adrenal hormones measured. None had an adrenal insufficiency nor an adrenal biopsy. Another synchronous metastasis was found in 69% at the time of the adrenal tumor discovery. During the median 2-year follow-up, 38% of the subjects had deceased (with the exclusion of individuals presenting supposedly benign adrenal tumors 31%) all due to tumor complications. A comparison between individuals identified through 68Gallium-DOTATOC-PET/CT and those who underwent adrenalectomy revealed a higher prevalence of NETs in the former group and NECs in the latter group.
CONCLUSIONS: Adrenal primary tumors and adrenal metastases are infrequent occurrences in patients with NEN. Most cases involved the presence of NEN metastasis upon the initial discovery of adrenal tumors. The overall prognosis was found to be favorable.