Chortis Vasileios, May Christine J H, Skordilis Kassiani, Ayuk John, Arlt Wiebke, Crowley Rachel K
Institute of Metabolism and Systems Research, University of Birmingham.
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.
Endocrinol Diabetes Metab Case Rep. 2019 Mar 23;2019. doi: 10.1530/EDM-18-0151.
Context Adrenal incidentalomas (AI) represent an increasingly common problem in modern endocrine practice. The diagnostic approach to AIs can be challenging and occasionally reveals surprising features. Here we describe two rare cases of complex adrenal lesions consisting of phaeochromocytomas with synchronous metastases from extra-adrenal primaries. Case descriptions Patient 1 - a 65-year-old gentleman with a newly diagnosed malignant melanoma was found to harbour an adrenal lesion with suspicious radiographic characteristics. Percutaneous adrenal biopsy was consistent with adrenocortical adenoma. After excision of the skin melanoma and regional lymphatic metastases, he was followed up without imaging. Three years later, he presented with abdominal discomfort and enlargement of his adrenal lesion, associated with high plasma metanephrines. Adrenalectomy revealed a mixed tumour consisting of a large phaeochromocytoma with an embedded melanoma metastasis in its core. Patient 2 - a 63-year-old lady with a history of NF-1-related phaeochromocytoma 20 years ago and previous breast cancer presented with a new adrenal lesion on the contralateral side. Plasma normetanephrine was markedly elevated. Elective adrenalectomy revealed an adrenal tumour consisting of chromaffin cells intermixed with breast carcinoma cells. Conclusions Adrenal incidentalomas require careful evaluation to exclude metastatic disease, especially in the context of a history of previous malignancy. Adrenal biopsy provides limited and potentially misleading information. Phaeochromocytomas are highly vascularised tumours that may function as a sieve, extracting and retaining irregularly shaped cancer cells, thereby yielding adrenal masses with intriguing dual pathology. Learning points: Adrenal incidentalomas require careful evaluation focused on exclusion of underlying hormone excess and malignant pathology. Adrenal biopsy can be misleading and should only be considered in select cases. Phaeochromocytomas harbouring intratumoural metastases from other, extra-adrenal primary malignancies represent rare pathological entities that highlight the complexities that can be presented by adrenal tumours.
背景
肾上腺偶发瘤（AI）在现代内分泌实践中是一个日益常见的问题。对肾上腺偶发瘤的诊断方法可能具有挑战性，偶尔还会发现令人惊讶的特征。在此，我们描述两例罕见的复杂肾上腺病变病例，这些病变由嗜铬细胞瘤与来自肾上腺外原发灶的同步转移瘤组成。
病例描述
患者1：一名65岁男性，新诊断为恶性黑色素瘤，发现肾上腺有一个具有可疑影像学特征的病变。经皮肾上腺活检结果符合肾上腺皮质腺瘤。切除皮肤黑色素瘤及区域淋巴结转移灶后，对其进行随访，未进行影像学检查。三年后，他出现腹部不适，肾上腺病变增大，并伴有血浆间甲肾上腺素水平升高。肾上腺切除术显示为一个混合性肿瘤，由一个大的嗜铬细胞瘤组成，其核心部位嵌入了一个黑色素瘤转移灶。
患者2：一名63岁女性，20年前有与神经纤维瘤病1型（NF-1）相关的嗜铬细胞瘤病史，既往有乳腺癌病史，现对侧出现一个新的肾上腺病变。血浆去甲间肾上腺素水平显著升高。择期肾上腺切除术显示肾上腺肿瘤由嗜铬细胞与乳腺癌细胞混合组成。
结论
肾上腺偶发瘤需要仔细评估以排除转移性疾病，尤其是在既往有恶性肿瘤病史的情况下。肾上腺活检提供的信息有限且可能产生误导。嗜铬细胞瘤是血管高度丰富的肿瘤，可能起到筛子的作用，提取并保留形状不规则的癌细胞，从而产生具有有趣双重病理特征的肾上腺肿块。
肾上腺偶发瘤需要仔细评估，重点是排除潜在的激素过量和恶性病理情况。肾上腺活检可能会产生误导，仅在特定病例中才应考虑。含有来自其他肾上腺外原发恶性肿瘤的瘤内转移灶的嗜铬细胞瘤是罕见的病理实体，突出了肾上腺肿瘤可能呈现的复杂性。
