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Rare disease: Diaphoresis and abdominal pain caused by extra-adrenal paragangliomas

机译:罕见疾病:肾上腺旁神经节瘤引起的出汗和腹痛

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摘要

A 63-year-old lady presented with suprapelvic pain, weight loss and night sweats. On examination, she was noted to be hypertensive with a distended abdomen. Imaging (CT) revealed a 9.5 cm retroperitoneal mass with a high degree of vascularity and necrotic centre. The patient’s urinary and plasma catecholamines were significantly raised and subsequent radio-isotope scan suggested the tumour was likely to be of a neuroendocrine nature. A diagnosis of a malignant paraganglioma was made. Malignant paragangliomas derive from sympathetic tissue and secrete catecholamines. Diagnostic uncertainty might lead to biopsy of tumour but this carries a high-risk of catecholamine-induced complications such as hypertensive crisis, cardiac arrhythmias and cardiac ischaemia and must be avoided.
机译:一位63岁的女士表现出腹上疼痛,体重减轻和盗汗。经检查,她患有腹部扩张的高血压。影像学检查(CT)显示9.5 cm的腹膜后肿块具有高度的血管和坏死中心。患者的尿和血浆儿茶酚胺明显升高,随后的放射性同位素扫描表明该肿瘤可能具有神经内分泌性质。诊断为恶性副神经节瘤。恶性副神经节瘤源于交感组织并分泌儿茶酚胺。诊断的不确定性可能会导致肿瘤的活检,但这会引起儿茶酚胺引起的并发症的高风险,例如高血压危象,心脏心律不齐和心脏缺血等,因此必须避免。

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