Kardalas Efstratios, Kyriakopoulos George, Antonopoulou Vasiliki, Kyriakou Aggeliki, Georgiadi Sofia, Tzanela Marinella, Ntali Georgia
Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece.
Department of Pathology, General Hospital Evangelismos, Αthens, Greece.
Hormones (Athens). 2025 May 28. doi: 10.1007/s42000-025-00676-w.
Herein, we present two interesting cases of adrenal ganglioneuromas (AGNs), namely, (a) a giant AGN which caused a severe episode of hypertension during a therapeutic dilation and curettage for early pregnancy loss, and (b) a case of a composite clinically silent adrenal ganglioneuroma-pheochromocytoma. Furthermore, we conducted a mini literature review on AGNs. CASE 1: A 31-year-old female patient presented with a history of early pregnancy loss at 9 ½ weeks. She underwent a therapeutic dilation and curettage during which she developed hypertension (~ 210/120 mm Hg). She reported no history of arterial hypertension, flushing, or tachycardia and her medical history was unremarkable. Further work-up, revealed a large heterogeneous tumor with microcalcifications in the left adrenal gland. Endocrine work-up was negative for hormonal excess. Magnetic resonance angiography (MRA) of the abdomen showed that the tumor was 'surrounding and strangling' the left renal vessels and the inferior vena cava. The patient successfully underwent an open left adrenalectomy and nephrectomy. Histology revealed an adrenal mature ganglioneuroma with Schwannian stroma being dominant. Her postoperative course was uneventful and she remains recurrence-free 6 years after surgery. CASE 2: A 37-year-old male patient was admitted to the hospital because of abdominal pain and hematuria. Computer tomography identified a 4-cm right adrenal lesion. Due to elevated urinary metanephrines, he underwent laparoscopic right adrenalectomy after appropriate alpha-blockade preparation preoperatively. Histology was consistent with pheochromocytoma with a component of ganglioneuroma, thus, a composite pheochromocytoma. His post-operative course was excellent and he remains asymptomatic and recurrence-free 60 months post-operatively.
These are two challenging cases of (a) a giant non-secreting AGN presenting with severe hypertension due to renal vessel compression and (b) a clinically silent composite pheochromocytoma-ganglioneuroma presenting with abdominal pain and hematuria. The size in the first case and the concurrence with a pheochromocytoma but a clinically silent phenotype in the second case highlight the fact that AGNs can be diagnostic chameleons.
在此，我们介绍两例有趣的肾上腺神经节细胞瘤（AGN）病例，即：（a）一例巨大AGN，在早期妊娠流产的治疗性刮宫过程中引发严重高血压发作；（b）一例复合型临床无症状肾上腺神经节细胞瘤 - 嗜铬细胞瘤。此外，我们对AGN进行了小型文献综述。病例1：一名31岁女性患者，有9.5周早期妊娠流产史。她接受了治疗性刮宫，在此期间出现高血压（约210/120 mmHg）。她报告无动脉高血压、潮红或心动过速病史，病史无异常。进一步检查发现左肾上腺有一个大的异质性肿瘤伴微钙化。内分泌检查未发现激素过多。腹部磁共振血管造影（MRA）显示肿瘤“环绕并压迫”左肾血管和下腔静脉。患者成功接受了开放性左肾上腺切除术和肾切除术。组织学检查显示为肾上腺成熟神经节细胞瘤，以施万细胞基质为主。她术后恢复顺利，术后6年无复发。病例2：一名37岁男性患者因腹痛和血尿入院。计算机断层扫描发现右肾上腺有一个4厘米的病变。由于尿甲氧基肾上腺素升高，他在术前进行适当的α受体阻滞剂准备后接受了腹腔镜右肾上腺切除术。组织学检查符合嗜铬细胞瘤合并神经节细胞瘤成分，即复合型嗜铬细胞瘤。他术后恢复良好，术后60个月无症状且无复发。
这是两例具有挑战性的病例，（a）一例巨大的无分泌功能AGN因压迫肾血管导致严重高血压，（b）一例临床无症状的复合型嗜铬细胞瘤 - 神经节细胞瘤表现为腹痛和血尿。第一例的肿瘤大小以及第二例与嗜铬细胞瘤并发但临床无症状的表型突出了AGN可表现多样、难以诊断的事实。
