Paraganglioma Crisis: the challenges of a silent neuroendocrine neoplasm


M Abdel-Malek, M Whitlock, E Wilkes, R Sharma, F Wernig, N Chapman, D Spalding, T Tan, Imperial College Healthcare NHS Trust



Case Presentation: A 47-year-old female presented with sudden visual loss and blurring associated with severe hypertension. She had a six-week prodrome of feeling generally tired with low energy and palpitations as well as significant weight loss (>25kg) over two years. She was otherwise normally fit and well with no past medical or drug history of note, aside from anaemia. On clinical examination, she was alert, orientated and apyrexial with a blood pressure of 224/147 and normal heart rate. Her chest was clear, abdomen soft and non-tender with a right-sided palpable mass, and her neurology intact. There was no meningism, stigmata of endocrinopathy or diaphoresis elicited. On ophthalmologic evaluation, she had bilateral retinopathic changes with macular oedema. She was promptly commenced on calcium channel and angiotensin II receptor blockers for blood pressure control but remained severely hypertensive with no response.


She was hyperglycaemic on admission and mildly hypokalaemic with a raised urea level and deranged liver function. She was biochemically euthyroid, had a satisfactory cortisol level and raised renin activity with normal aldosterone levels. She had severe concentric left ventricular hypertrophy on echocardiography. Abdominal ultrasound showed a 21 x 18 cm mass that filled the entire upper abdomen extending from the vertebral column to the anterior abdominal wall and MRI confirmed a large retroperitoneal mass which did not appear to involve the adrenal glands. Moreover, her plasma and urinary catecholamine status was significantly abnormal; the plasma Normetadrenaline level was 69,106 (normal range < 1180) and Metadrenaline 99,134 (normal range < 510). Her neuroendocrine secretory proteins, Chromogranin A and B levels were also raised. Further differentiation with Ga68 DOTATATE PET CT revealed that the mass likely represented an extra-adrenal paraganglioma.


Conclusion: This case represents the diagnostic challenges from such a rare neuroendocrine tumour. Abdominal paraganglioma tend to be functional and slow-growing, and in this case presented as an inoperable mass, making them complex to medically manage. It is a vital diagnosis not to miss that may initially masquerade with few paroxysmal symptoms or undiagnosed hypertension, before acutely presenting as a crisis.