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A shining heart at Valentine's Day
Annales d'Endocrinologie ( IF 2.9 ) Pub Date : 2021-05-24 , DOI: 10.1016/j.ando.2020.03.017
Younes Azzagnuni , Sacha Regenberg , Valerio Lucidi , Philippe Thoma , Philippe Van de Borne , Rodrigo Moreno-Reyes , Ioannis Karfis , Bernard Corvilain , Natacha Driessens

A 60-year-old men was admitted mid-January for acute left abdominal pain. He had no medical history and didn’t smoke or take any medication. Abdominal CT-scan showed a voluminous (61 × 57 × 47 mm), heterogeneous, hemorrhagic mass in left adrenal gland with a high spontaneous density (45UH). Pheochromocytoma diagnosis was first suspected although 24-hours urine fractionated metanephrines and catecholamines measurements were only slightly increased (NA:1.1xULN and NMN:1.2xULN). Hormonal evaluation did not demonstrate excessive cortisol or sexual steroids secretion. 18F-FDG PET-CT imaging revealed intense FDG-uptake in the left adrenal gland as well as in the right adrenal gland and in the heart. With this new information, the differential diagnosis includes carcinoid cardiac tumor, malignant cardiac myxoma, sarcoma and metastases. All others tumor markers were negative (5HIAA, NSE, CA 19-9, CA 125, CEA, PSA, β-hCG). For Valentine's Day, cardiac MRI confirmed a pathological thickening of right inter-atrioventricular groove with marked late enhancement at this level, corresponding to pathological areas in isotopic imaging. Next day, patient presented acute right flank pain similar to the one he had on left side one month earlier. New abdominal CT-scan showed a large hemorrhage in right adrenal gland that was not present on 18F-FDG PET-CT performed 3 days before. Latest Ga-DOTATATE-PET imaging didn’t show anywhere STT-receptors presence. Morning cortisol level (411 nmol/L) remained non-suggestive of adrenal insufficiency. This case was discussed by web-conference with the French experts of the national COMETE multidisciplinary committee: it was decided to obtain histology from left adrenal tumor by percutaneous biopsy rather than by unilateral adrenalectomy because the radicality of surgery might change depending on histological diagnosis. So far, diagnosis has not yet been established. This case underlines importance of discussing complex situations with experts as allowed by COMETE or ENDO-ERN networks.



中文翻译:

情人节的一颗璀璨的心

1月中旬,一名60岁的男性因急性左腹痛入院。他没有病史,也没有吸烟或服药。腹部CT扫描显示体积大(61  ×  57  ×  47 毫米),左肾上腺异质性出血性肿块,具有很高的自发密度(45UH)。最初怀疑是嗜铬细胞瘤的诊断,尽管24小时尿液中分离的肾上腺素和儿茶酚胺的测定值仅略有增加(NA:1.1xULN和NMN:1.2xULN)。激素评估未显示皮质醇或性类固醇分泌过多。18F-FDG PET-CT成像显示左肾上腺以及右肾上腺和心脏中强烈摄取FDG。有了这些新信息,鉴别诊断将包括类癌性心脏肿瘤,恶性心脏粘液瘤,肉瘤和转移灶。所有其他肿瘤标志物均为阴性(5HIAA,NSE,CA 19-9,CA 125,CEA,PSA,β-hCG)。在情人节那天 心脏MRI证实右房室沟的病理性增厚,并在此水平明显增强,这与同位素成像中的病理区域相对应。第二天,患者出现急性右胁痛,类似于一个月前他在左侧的疼痛。新的腹部CT扫描显示右肾上腺大出血,这是3天前进行的18F-FDG PET-CT所没有的。最新的Ga-DOTATATE-PET成像未显示STT受体存在的任何位置。早晨皮质醇水平(411 nmol / L)仍未提示肾上腺功能不全。通过网络会议与国家COMETE多学科委员会的法国专家讨论了此案:决定通过经皮穿刺活检而不是单侧肾上腺切除术从左肾上腺肿瘤获得组织学,因为手术的根本性可能会根据组织学诊断而改变。到目前为止,尚未确定诊断。该案例强调了COMETE或ENDO-ERN网络允许与专家讨论复杂情况的重要性。

更新日期:2021-05-25
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