Endocrine Unit, Department of Medicine, Hospital Kuala Lumpur, Malaysia.
Histopathology Unit, Department of Pathology, Hospital Kuala Lumpur, Malaysia.
J ASEAN Fed Endocr Soc. 2022;37(1):87-90. doi: 10.15605/jafes.037.01.10. Epub 2022 Feb 4.
Ganglioneuromas (GNs) are benign tumors that originate from neural crest cells, composed mainly of mature ganglion cells. These tumors, which are usually hormonally silent, tend to be discovered incidentally on imaging tests and occur along the paravertebral sympathetic chain, from the neck to the pelvis and occasionally in the adrenal medulla. Rarely, GNs secrete catecholamines. Adrenal GNs occur most frequently in the fourth and fifth decades of life, whereas GNs of the retroperitoneum and posterior mediastinum are usually encountered in younger adults. Adrenal GNs are commonly hormonally silent and asymptomatic; even when the lesion is of substantial size. We report an incidentally detected asymptomatic case of an adrenal ganglioneuroma with mildly elevated urinary catecholamine levels in an elderly male. After preoperative alpha blockade, the patient underwent open right adrenalectomy. Upon microscopic examination, the right adrenal mass proved to be a ganglioneuroma, maturing type and the immunohistochemistry examination showed immunoreactivity to synaptophysin, chromogranin, and CD 56, while S100 was strongly positive at the Schwannian stroma. Following resection, catecholamine levels normalized, confirming the resected right adrenal ganglioneuroma as the source of the catecholamine excess. This case represents a rare presentation of catecholamine-secreting adrenal ganglioneuroma in the elderly.
神经节细胞瘤(GNs)是起源于神经嵴细胞的良性肿瘤,主要由成熟的神经节细胞组成。这些肿瘤通常无激素活性,往往在影像学检查中偶然发现,沿椎旁交感神经链分布,从颈部到骨盆,偶尔也在肾上腺髓质。很少见的情况下,GNs 会分泌儿茶酚胺。发生于肾上腺的 GN 最常发生于第四和第五个十年,而发生于腹膜后和后纵隔的 GN 通常见于年轻成人。肾上腺 GN 通常无激素活性且无症状;即使病变很大。我们报告了一例偶然发现的无症状老年男性的肾上腺神经节细胞瘤病例,其尿儿茶酚胺水平轻度升高。在术前进行α受体阻断后,患者接受了开放的右肾上腺切除术。显微镜下检查,右肾上腺肿块证实为神经节细胞瘤,成熟型,免疫组化检查显示突触素、嗜铬粒蛋白和 CD56 免疫反应性,而 Schwannian 基质中的 S100 呈强阳性。切除后,儿茶酚胺水平恢复正常,证实切除的右侧肾上腺神经节细胞瘤是儿茶酚胺过多的来源。该病例代表了老年分泌儿茶酚胺的肾上腺神经节细胞瘤的罕见表现。