Yoshioka Keiko, Nakano Yujiro, Horichi Moeka, Aono Daisuke, Takeshita Yumie, Takamura Toshinari
Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medical Sciences, Kanazawa University, Kanazawa, 920-8641, Ishikawa, Japan.
JCEM Case Rep. 2024 Dec 26;3(1):luae241. doi: 10.1210/jcemcr/luae241. eCollection 2025 Jan.
Pheochromocytoma and paraganglioma (PPGL) are rare chromaffin-cell tumors producing adrenaline and/or noradrenaline, or solely dopamine. A 52-year-old man presenting with hypertension (141/79 mm Hg) and weight loss (10 kg in 6 months) was admitted to our hospital. Computed tomography revealed a massive right adrenal mass (150 mm) with partial necrosis, accompanied by multiple liver nodules. These nodules showed a high signal intensity on T2-weighted magnetic resonance imaging. Subsequently, a diagnosis of PPGL was made based on elevated urinary excretion of adrenaline (355 µg/day [1937 nmol/day]; normal range: 3.4-26.9 µg/day; 18-146 nmol/day), noradrenaline (1690 µg/day [9989 nmol/day]; normal range: 48.6-168.4 µg/day; 287-995 nmol/day), and dopamine (53 000 µg/day [258 322 nmol/day]; normal range: 365-961.5 µg/day; 1779-4686 nmol/day). The I-metaiodobenzylguanidine scintigraphy and fluorodeoxyglucose positron emission tomography scan showed heterogenous uptake among the adrenal and the liver foci, respectively. Clustering analysis of previous PPGL cases highlighted the unique catecholamine profile of this case. These findings suggest a possibility that internodular heterogeneity between primary and metastatic foci on nuclear imaging may indicate varying differentiation grades and resultant catecholamine secretion. Further studies will be needed to verify these results and confirm this hypothesis.
嗜铬细胞瘤和副神经节瘤（PPGL）是罕见的嗜铬细胞瘤，可产生肾上腺素和/或去甲肾上腺素，或仅产生多巴胺。一名52岁男性因高血压（141/79 mmHg）和体重减轻（6个月内减轻10 kg）入院。计算机断层扫描显示右侧肾上腺有一个巨大肿块（150 mm），伴有部分坏死，同时伴有多个肝脏结节。这些结节在T2加权磁共振成像上显示高信号强度。随后，根据尿中肾上腺素（355 μg/天[1937 nmol/天]；正常范围：3.4 - 26.9 μg/天；18 - 146 nmol/天）、去甲肾上腺素（1690 μg/天[9989 nmol/天]；正常范围：48.6 - 168.4 μg/天；287 - 995 nmol/天）和多巴胺（53000 μg/天[258322 nmol/天]；正常范围：365 - 961.5 μg/天；1779 - 4686 nmol/天）排泄升高，诊断为PPGL。间碘苄胍闪烁扫描和氟脱氧葡萄糖正电子发射断层扫描分别显示肾上腺和肝脏病灶摄取不均匀。对既往PPGL病例的聚类分析突出了该病例独特的儿茶酚胺谱。这些发现提示，核成像上原发灶和转移灶之间的结节间异质性可能表明分化程度不同以及由此产生的儿茶酚胺分泌情况。需要进一步研究来验证这些结果并证实这一假设。
