Paraganglioma arising from the liver and abutting the heart

Abstract

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. © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.