Poster Presentation ESA-SRB Conference 2015

Flushed with excitement – a heartfelt case of carcinoid syndrome (#268)

Tara Naige 1 , Jessica Stranks 1 , Peak Mann Mah 1 , Anthony Zimmermann 1
  1. Department of Endocrinology, Northern Adelaide Local Health Network, South Australia

Neuroendocrine tumours (NETs) are a heterogeneous group of neoplasms that arise from neuroendocrine cells of gastrointestinal tract1. Carcinoid syndrome with the classic triad of flushing, palpitations and diarrhoea/abdominal pain, is more specifically attributed to well-differentiated serotonin-secreting midgut tumours and is present in approximately 20% of NETs in the duodenum and jejunum1,2.

Individuals with carcinoid syndrome can present with chest tightness or breathlessness3 leading to diagnoses of acute coronary syndrome or asthma. These symptoms are thought to be related to serotonergic activity of the NETs. Research suggests an association between the increased serotonergic activity4,5 with Takotsubo cardiomyopathy6, a transient cardiac syndrome with left ventricular hypokinesis or dyskinesis7.

We present the case of Ms LT, a 64-year old woman who presented with palpitations and chest pain in the setting of intense emotional stress, with dynamic ECG changes and troponin rise. This is on a background of multiple pulmonary embolisms, late-onset asthma and hypertension. Her family history is significant for Graves’ disease and the death of her father from a pancreatic tumour. Coronary angiogram showed pristine coronary arteries with apical ballooning and MRI showed basal inferior hypokinesis, normal contractility leading to a diagnosis of Takotsubo cardiomyopathy.

She represented one month later with PR bleeding, abdominal pain, diarrhoea, flushing and refractory palpitations. Investigations showed a neuroendocrine tumour of the ileal region with lymphatic and bony metastases. Chromogranin A and 24-hr urinary 5HIAA were elevated, however, after ceasing her proton-pump inhibitor her Chromogranin A normalised. She underwent surgical resection of her ileal tumour and was commenced on somatostatin analogue, which is keeping her carcinoid syndrome under control.

Increased plasma serotonin has been reported in Takotsubo cardiomyopathy. Clinical suspicion of carcinoid syndrome in a patient with Takotsubo cardiomyopathy should be raised with the presentation of the classic triad: flushing, palpitations and diarrhoea/abdominal pain.

  1. Ramage JK, et al. 2012. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs); Gut 2005;54: suppl 4 iv1-iv16. doi:10.1136/gut.2004.053314
  2. Modlin IM, et al. 2010. Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management. Med J Aust; 193(1): 46-52.
  3. Goldfinger SE, Strosberg JR. 2013. Clinical features of the carcinoid syndrome. UpToDate Topic 2614 Version 14.0
  4. Rouzard LC, et al. 2013. First evidence of increased plasma serotonin levels in Tako-Tsubo cardiomyopathy. Biomed Res Int: Sep 23. doi: 10.1155/2013/847069
  5. Mehta NK, Aurigemma G, Rafeq Z, Starobin O. 2011. Reverse Takotsubo Cardiomyopathy after an episode of serotonin syndrome. Tex Heart Inst J; 38(5): 568-572
  6. Reeder GS, Prasad A. 2014. Stress (Takotsubo) cardiomyopathy. UpToDate; Topic 3494 Version 11.0
  7. Mazzetti I, Maracccio MJ, Boutross-Tadross O, Salehian O, Demers C. 2013. The Ace of Spades: Reverse Takotsubo Cardiomyopathy in the context of angiographic embolization of recurrent metastatic serotonin-positive neuroendocrine tumour of the pancreas; Case Rep Med; Feb 7. doi: 10.1155/2013/793193