ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2017) 52 P38 | DOI: 10.1530/endoabs.52.P38

Paraneoplastic syndrome or De Novo diagnosis?: Metastatic small bowel neuroendocrine tumour presenting with Giant cell arteritis

Ruth Nanda1,2, Ellie Smith3 & Alia Munir4


1Sheffield Teaching Hospitals NHS FT Trust, Sheffield, UK; 2University of Sheffield, Sheffield, UK; 3Weston Park Hospital, Sheffield, UK; 4Sheffield Teaching Hospitals NHS FT, Sheffield, UK.


Neuroendocrine tumours are rare, with an incidence of 8/100,000 in the UK. They result from excessive proliferation of neuroendocrine cells and are classified based on their site of origin, differentiation and clinical syndrome. Giant cell arteritis (GCA), a systemic vasculitis of unknown aetiology, rarely appears as a paraneoplastic syndrome. It is histologically characterised by granulomatous infiltrates with multinucleated giant cells at the intima media junction. The relationship between association or causation of malignancy remains unclear. With an incidence of 1-2/10,000 in the UK, diagnostic criteria include at least three of the following:

i) Sudden onset headache

ii) Age over 50 years

iii) Elevated ESR

iv) Temporal artery abnormality

V) Biopsy abnormality

We present a 70 year old lady who was referred to the NET clinic with intractable pain, and metastatic NET from a small bowel primary with extensive liver and bone lesions and carcinoid syndrome. On careful review of her pain history, she gave a history of proximal muscle weakness and pain at presentation and a 2 week history of severe headache, causing nocturnal wakening. She was reviewed having commenced opiate analgesia (including tramadol, oromorph and buprenorphine then fentanyl patch) and neuropathic agents (amitriptyline) for management of presumed bone pain, with little relief. However clinically there was temporal artery/scalp tenderness and jaw claudication. Multimodal imaging had already been performed. CRP and ESR were measured at 73 mg/l and 106 mm/Hr respectively. She was commenced on high dose prednisolone, with stomach protection, with rapid improvement of symptoms leading to subsequent discontinuation of opiates. Subsequent ESRs were measured at 17 mm/Hr and prednisolone tapered accordingly. Literature search on CGA/NET was quite minimal. In one study the temporal relationship of CGA diagnosis 1 year prior to a malignancy, was 3.4% compared to 2.7% in controls, possibly leaning to a paraneoplastic aetiology. In another study 7.4% of patients had concurrent malignancy, albeit 45% of these were haematological. CGA has been reported in atypical pulmonary NET, but to our knowledge never previously in metastatic small bowel neuroendocrine tumour. This case highlights the need for stringent clinical assessment in this group as successful therapeutic changes maybe indicated.

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