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

Author:

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

Abstract

AbstractBackgroundPheochromocytoma 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 presentationA 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.ConclusionsHaemoptysis 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.

Publisher

Springer Science and Business Media LLC

Subject

General Medicine,Surgery

Reference14 articles.

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2. Frymoyer PA, Anderson GH Jr, Blair DC. Hemoptysis as a presenting symptom of pheochromocytoma. J Clin Hypertens. 1986;2:65–7.

3. Naruse M, Tsuiki M, Nanba K, Nakao K, Tagami T, Tanabe A. Etiology and clinical guidelines for the diagnosis and treatment of pheochromocytoma in Japan. Nihon Geka Gakkai Zasshi. 2012;113:378–83.

4. Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001;86:5210–6.

5. Fishbein L, Merrill S, Fraker DL, Cohen DL, Nathanson KL. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 2013;20:1444–50.

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