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Pre-aortic paraganglioma
  1. S Nag1,
  2. A McCulloch2
  1. 1James Cook University Hospital, Marton Road, Middlesbrough TS4 3BW, UK;
  2. 2Bishop Auckland General Hospital, Bishop Auckland, Co Durham, UK

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    A 49 year old man with chronic headaches, longstanding anxiety, and fatigue presented with poorly controlled hypertension. Type 2 diabetes had been diagnosed recently. There was no history of palpitations, flushing, or chest pain; blood pressure was 170/100 mm Hg. Physical examination was otherwise normal.

    His history prompted the measurement of urine catecholamines. Urine metadrenaline was raised at 85.6 μmol/24 hours (normal 0–5.5) and urine noradrenaline was markedly raised at 15388 μmol/24 hours (normal 90–600); urine dopamine and adrenaline were normal. I131MIBG scintigraphy showed increased isotope uptake in the epigastric area (arrow, fig 1). Abdominal computed tomography showed a lobulated mass in the region of the left coeliac axis (arrow, fig 2). A large vascular pre-aortic phaeochromocytoma was subsequently removed. Blood pressure and glucose tolerance normalised after surgery.

    Figure 1

     Scintigraphy showing increased isotope uptake in epigastric area.

    Figure 2

     Abdominal computed tomography showing lobulated mass in the region of the left coeliac axis.

    Phaeochromocytomas are rare tumours accounting for fewer than 1% of cases of hypertension. Extra-adrenal tumours termed paragangliomas occur in 10% of patients and arise from neural crest derivatives. They predominantly secrete noradrenaline. Phaeochromocytomas occur equally in both sexes and usually present in the third and fourth decades of life. Ninety percent of tumours are sporadic, while 10% occur as part of an inherited syndrome like multiple endocrine neoplasia type 2, Von Hippel-Lindau syndrome, or neurofibromatosis.

    In the absence of classical paroxysms, the symptoms of catecholamine excess may be protean and non-specific. A high index of suspicion remains the cornerstone of diagnosis.