Neuroendocrine tumour diagnosed in pregnancy, the challenges of complex disease


Dr Ali Naqvi, Dr Edouard Mills, Dr Chukwuma Uduku, Dr Pratibha  Machenahalli,  Dr Jeannie F  Todd, Imperial centre for Endocrinology, Hammersmith Hospital, Imperial Healthcare NHS Trust

The appropriate diagnosis and treatment of neuroendocrine tumours often involves collaboration between specialists in multiple disciplines, using specific biochemical, histological and surgical methods. The 28 years old lady presented with epigastric abdominal pain at 16 weeks of pregnancy. She was found to have pancreatic head lesion of 4.5 cm. She had EUS, showed 4 cm solid lesion at head of pancreas adjacent to portal vein and SMV without obvious invasion, subsequently FNA was positive for chromogranin and synaptophysin, consistnet with low grade Neuroendocrine tumour (NET). The obstetrician were very keen for her to get to 30 to 32 weeks with steroid cover before delivery, therefore it was discussed in NET MDT to monitor her over next few weeks of her pregnancy. She had elective caesarean at 30 weeks of gestation. After delivery, CT scan showed a large tumour, which appeared to involve the SMV and octreotide scan showed localised disease only. She was admitted 2 weeks after delivery for proposed elective Whipple’s procedure. The laparotomy revealed no evidence of metastases, but tumour extended posterior to the superior mesenteric artery. As artery was involved it was not possible to resect the tumour. The histology of lymph node showed an NET Ki-67 2 to 3 % with negative hormonal markers. She was referred for consideration of radiotherapy and chemotherapy. Her 4.5cm non functioning pancreatic neuroendocrine tumour reduced to 2.6 cm post pregnancy prior to chemotherapy but it was mainly due to reduction of cystic component. She did have a course of chemotherapy with 5-FU and Streptozocin but did not complete her cycles. She decided to have Nanoknife therapy to pancreatic neuroendocrine privately. Her pain improved after Nanoknife therapy. She was started on somatostatin analogue. It was decided against surgery in the MDT meeting because of possible complication associated with surgery and disease was stable on somatostatin analogue. The patient decided to have surgery at Heidelberg. She had Whipple’s resection and then partial gastrectomy, because of gastric ischaemia, it was further complicated by ascites and chyle fistula.  The histology showed complete resection of the tumour with clear margins, Ki 67 was 10 % but there was some lymphovascular invasion and 1/32 lymph node   positive, T3N1V1pN1R0. She developed diarrhoea and weight loss after extensive surgery. She was also diagnosed with bile salt malabsoption. The repeat gallium DOTATATE PET CT scan did show no focal tracer activity but showed moderate ascites of disease. She currently has significant diarrhoea and difficult gaining weight.

The primary treatment goal for patients with neuroendocrine tumours is curative with symptoms control and limitation of progression of disease. Surgery is a possible curative approach but can lead to significant morbidities, because of tumour location. Chemotherapy and Somatostatin analogue can also limit the progress of disease when tumour is inoperable and disease remains stable but it needs multi disciplinary team input and regular imaging to see progress of disease.