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A Tale of Two Brothers with Neuroendocrine Tumours

S Wijetilleka, E Mills, R Agha-Jaffer, D Spalding, JF Todd, Hammersmith Hospital, Imperial Centre for Endocrinology, Imperial College Hospitals NHS Trust

We report the case of a 59-year-old Arabic businessman with a 6-month history of type 2 diabetes, hypertension, hypercholesterolaemia and gastro-oesophageal reflux disease.  Of note, his brother’s medical history was significant for a large bowel neuroendocrine tumour, which had recently been resected and was under the care of another Endocrine Unit.  Our patient was originally referred to the Respiratory Clinic with exertional dyspnoea, which was felt to be infective in aetiology and antibiotics were prescribed.  However, 6kg weight loss over 6-months was noted. Owing to a plain chest radiograph which demonstrated an elevated right hemi-diaphragm, further CT imaging was arranged.  This confirmed a raised right hemi-diaphragm, but also an incidental 2cm pancreatic mass lesion.  This prompted referral to Hepatobiliary and Pancreatic Surgery.

Cytology via endoscopic guided ultrasound was consistent for a neuroendocrine tumour.  Further MRI pancreas revealed a 2cm exophytic lesion without further metastatic spread.  Gallium-68 DOTATE PET CT revealed avid disease within the pancreatic lesion, but no evidence of metastatic nodal or distant disease.  Pre-operative gut hormones were normal and 24-hour urine 5-hydroxyindoleacetic acid (5-HIAA) levels were raised at 72.2umol/L (NR 10-40umol/L).  The advice of the Neuroendocrine Tumours MDT was surgical excision.

He underwent a laparoscopic resection with the histology confirming a completely excised, 30mm well-differentiated and glucagon-staining neuroendocrine tumour with Ki67 index <5%.  However, his post-operative CT imaging has since revealed a lesion within the surgical bed.  Repeat Gallium-68 DOTATE PET CT confirms that this lesion enhances.  Post-operative gut hormones remain normal.  The consensus from the Neuroendocrine Tumours MDT is that this lesion likely represents residual disease and repeat surgery is being considered.

Discussion: Non-functioning pancreatic neuroendocrine tumours are defined by their absence of hormone hypersecretion. They can present as an incidental finding, such was the case with our patient. Surgical resection of lesions > 2cm or where there is growth on interval imaging may offer curative treatment in the absence of liver metastases. Crucially, patients with neuroendocrine tumours are best managed within a Neuroendocrine Tumours MDT setting.