Endocrine Abstracts

We present the case of a 68-year-old man from Ireland with a history of neurofibromatosis type 1, diagnosed in 2000, who underwent surgery for a right pheochromocytoma in 2005. He was recently admitted to hospital for acute cholecystitis, which was treated conservatively with antibiotics. During his hospitalisation, an abdominal CT scan revealed a 1.8 cm lesion in the uncinate process of the pancreas compatible with a neuroendocrine tumor (NET). In addition, multiple lesions were detected in several liver segments, consistent with microabscesses. 18F-FDG PET/CT was performed and confirmed the presence of a hypermetabolic lesion in the uncinate process. Blood tests were performed to assess the functionality of the NET, which showed that it was a non-functioning tumour. A somatostatin receptor scintigraphy was negative for pathological findings. Based on these results, an endoscopy was performed to obtain a tissue sample from the pancreatic lesion. Histological examination confirmed the diagnosis of a well-differentiated NET (G1) with Ki67 <1% and 0 mitoses/10 HPF. The case was presented to a multidisciplinary committee and it was decided to request an abdominal MRI to assess the evolution of the microabscesses and to complement the study with 18F-DOPA PET/TC. The MRI showed multiple liver lesions compatible with metastasis in addition to the known pancreatic lesion. The 18F-DOPA PET/CT showed no pathological uptake. A Ga-68-DOTATOC PET/CT was also requested and showed no pathological uptake. The patient was referred to medical oncology and started on everolimus 10 mg daily. However, he developed a generalised skin reaction, which necessitated discontinuation of the drug. The case was reassessed by the committee and a two-stage surgical procedure was deemed the most appropriate course of action. Initially, a cephalic pancreaticoduodenectomy with pyloric preservation was performed. Histopathological analysis of the resected tissue revealed a well-differentiated NET, G1, measuring 2.2 cm, with extensive vascular-lymphatic invasion and lymph node involvement (18 lymph nodes involved). Liver lesions were subsequently resected: two in segment II, two in segment III, one in segment IVa, two in segment V, one in inferior segment VI and two in segment VIII. Histopathological evaluation confirmed that these lesions were metastases from a well-differentiated NET. This case highlights the importance of multidisciplinary management in the diagnosis, treatment and follow-up of NETs. A comprehensive approach involving oncologists, endocrinologists, surgeons and radiologists is essential for optimal patient care.