BMC Surgery (Jan 2021)

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

  • Yutaka Endo,
  • Minoru Kitago,
  • Masahiro Shinoda,
  • Hiroshi Yagi,
  • Yuta Abe,
  • Shutaro Hori,
  • Masanori Odaira,
  • Takahiro Yokose,
  • Kaori Kameyama,
  • Yuko Kitagawa

DOI
https://doi.org/10.1186/s12893-020-01038-6
Journal volume & issue
Vol. 21, no. 1
pp. 1 – 5

Abstract

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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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