Singh Rajiv, Mohandas Cynthia
Department of Internal MedicineDarent Valley Hospital, Dartford, UK.
Department of Diabetes and EndocrinologyDarent Valley Hospital, Dartford, UK.
Endocrinol Diabetes Metab Case Rep. 2021 Apr 1;2021. doi: 10.1530/EDM-20-0198.
A phaeochromocytoma is a rare neuroendocrine tumour derived from the chromaffin cells of the adrenal medulla. Tumours can produce excessive amounts of catecholamines. The presenting symptoms can vary but often include the classic triad of episodic headaches, sweating and palpitations. Due to catecholamine excess, patients can develop cardiomyopathy. Bradycardia and collapse could be the result of sinus node dysfunction or transient dysregulation of the autonomic nervous system. Patients with co-existing diabetes can have improvement or resolution of their diabetes after successful adrenalectomy. We report a case of an 87-year-old lady who initially presented with sweating, palpitations and collapse, resulting in a permanent pacemaker insertion. She was later found to have a large adrenal incidentaloma with subsequent markedly elevated plasma metanephrine levels. She later presented with chest pain and in acute pulmonary oedema with normal coronary arteries visualised on coronary angiogram. After surgical excision of her phaeochromocytoma, her diabetes resolved with her HbA1c improving from 68 to 46 mmol/mol, with no further requirement for diabetic medications. Her pulmonary oedema improved with no ongoing need for diuretic therapy. This case highlights that phaeochromocytomas can affect multiple systems and there should be a very high index of suspicion in patients presenting with sweating, palpitations, hypertension and a history of diabetes and even in those with collapse.
There should be a high index of suspicion for phaeochromocytomas in patients with palpitations, diaphoresis, anxiety, hypertension and diabetes. Rarely phaeochromocytomas can present as bradycardia and collapse due to sinus node dysfunction or transient autonomic dysregulation and that should be considered in older patients. Catecholamine cardiomyopathy can occur in phaeochromocytoma with potential resolution after successful surgical excision. Diabetes can resolve after successful surgical treatment of a phaeochromocytoma.
嗜铬细胞瘤是一种罕见的神经内分泌肿瘤,起源于肾上腺髓质的嗜铬细胞。肿瘤可分泌过量的儿茶酚胺。其临床表现多样,但常包括发作性头痛、出汗和心悸这一经典三联征。由于儿茶酚胺过量,患者可发展为心肌病。心动过缓和虚脱可能是窦房结功能障碍或自主神经系统短暂失调的结果。合并糖尿病的患者在成功进行肾上腺切除术后,糖尿病病情可能改善或缓解。我们报告一例87岁女性患者,最初表现为出汗、心悸和虚脱,因此植入了永久性起搏器。后来发现她有一个较大的肾上腺意外瘤,随后血浆间甲肾上腺素水平显著升高。她后来出现胸痛,并伴有急性肺水肿,冠状动脉造影显示冠状动脉正常。切除嗜铬细胞瘤后,她的糖尿病病情缓解,糖化血红蛋白从68 mmol/mol降至46 mmol/mol,不再需要糖尿病药物治疗。她的肺水肿也有所改善,不再需要利尿剂治疗。该病例强调嗜铬细胞瘤可影响多个系统,对于出现出汗、心悸、高血压以及有糖尿病史甚至有虚脱症状的患者,应高度怀疑该病。
对于有心悸、多汗、焦虑、高血压和糖尿病的患者,应高度怀疑嗜铬细胞瘤。罕见情况下,嗜铬细胞瘤可因窦房结功能障碍或短暂的自主神经失调而表现为心动过缓和虚脱,老年患者应考虑这种情况。嗜铬细胞瘤可发生儿茶酚胺性心肌病,成功手术切除后有可能缓解。成功切除嗜铬细胞瘤后糖尿病可能缓解。