Extra-adrenal pheochromocytoma with initial symptom of haemoptysis: a case report and review of literature.

Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan. dragonpegasus@keio.jp.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Surgery, Tokyo Saiseikai Central Hospital, Tokyo, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.Department of Pathology, Keio University School of Medicine, Tokyo, Japan.Department of Surgery, Keio University School of Medicine, Tokyo, 1608582, Japan.

BMC surgery. 2021;(1):13
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Abstract

BACKGROUND Pheochromocytoma is a catecholamine-secreting tumour that leads to various symptoms. Haemoptysis is rarely caused by a pheochromocytoma occurring outside the bronchus or thoracic cavity. Here, we report the case of an extra-adrenal abdominal pheochromocytoma initially manifesting as haemoptysis/dyspnoea during exercise without classic symptoms. CASE PRESENTATION A 22-year-old man with a history of severe dyspnoea experienced difficulties in breathing following a marathon owing to haemoptysis that required ventilator management 1 year before presentation. His father had undergone surgery for ectopic pheochromocytoma. Computed tomography (CT) revealed a 30-mm tumour between the inferior vena cava and pancreatic head while urinalysis revealed abnormally high noradrenaline levels. He was clinically diagnosed with an extra-adrenal abdominal ectopic pheochromocytoma. After controlling blood pressure, surgery was performed, and the tumour was successfully removed. Histopathology revealed chromogranin A (+), synaptophysin (+), S100 protein (+), and MIB-1 index of 1%. Therefore, the patient was finally diagnosed with extra-adrenal abdominal ectopic pheochromocytoma. CONCLUSIONS Haemoptysis is a rare manifestation of abdominal ectopic paraganglioma. Prompt consideration of pheochromocytoma/paraganglioma when patients experience haemoptysis without any other possible aetiology may prevent inappropriate diagnosis and treatment and ultimately fatalities.

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Publication Type : Case Reports ; Review

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