Kharroubi Hussein, Sawma Tedy, Sfeir Pierre, Khalife Mohammad
Faculty of Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Surgery, American University of Beirut Medical Center, Beirut, Lebanon.
BMJ Case Rep. 2023 Mar 3;16(3):e253847. doi: 10.1136/bcr-2022-253847.
A paraganglioma is a rare extra-adrenal neuroendocrine tumour with a variable clinical presentation. A paraganglioma can arise anywhere along the sympathetic and parasympathetic chains, but it can occasionally emerge from unusual locations such as the liver and the thoracic cavity. We report a rare case of a woman in her 30s who presented to our emergency department with symptoms of chest discomfort, episodic hypertension, tachycardia and diaphoresis. A diagnostic approach including a chest X-ray, an MRI and a positron emission tomography-CT scan showed a large exophytic liver mass protruding into the thoracic cavity. For further characterisation of the mass, a biopsy of the lesion was performed, demonstrating that the tumour is of neuroendocrine origin. This was supported by a urine metanephrine test showing high levels of catecholamine breakdown products. Treatment consisted of a unique multidisciplinary approach involving hepatobiliary and cardiothoracic surgery allowing a safe and complete extermination of the hepatic tumour and its cardiac extension.
副神经节瘤是一种罕见的肾上腺外神经内分泌肿瘤，临床表现多样。副神经节瘤可沿交感神经链和副交感神经链的任何部位发生，但偶尔也会出现在肝脏和胸腔等不寻常的部位。我们报告了一例罕见病例，一名30多岁的女性因胸部不适、发作性高血压、心动过速和多汗症状前来我院急诊科就诊。包括胸部X线、MRI和正电子发射断层扫描-CT扫描在内的诊断方法显示，有一个巨大的外生性肝脏肿块突入胸腔。为了进一步明确肿块的特征，对病变进行了活检，结果显示该肿瘤起源于神经内分泌。尿甲氧基肾上腺素检测显示儿茶酚胺分解产物水平升高，支持了这一诊断。治疗采用了独特的多学科方法，包括肝胆外科和心胸外科手术，从而安全、彻底地切除了肝脏肿瘤及其心脏延伸部分。
