Endocrine Abstracts

Background: Pancreatic neuroendocrine neoplasms (NENs) are rare tumors which can sometimes be diagnosed based on symptoms of hormone excess, but, more often, they are asymptomatic, and patients frequently present with metastatic disease. While they can occur in hereditary cancer syndromes such as multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau (VHL), or neurofibromatosis (NF1), the majority of pancreatic NENs are sporadic

Case report: We report the case of a 37-year-old male referred to a gastroenterology clinic for nonspecific gastrointestinal symptoms. Abdominal ultrasound reported a large hepatic mass, which was confirmed with an abdominal CT scan. This prompted an ultrasound-guided hepatic biopsy, which raised the suspicion of metastatic liver disease arising from a primary pancreatic NEN. The diagnosis was confirmed with immunohistochemistry, which graded the tumor as a NET G2 based on a ki-67 of 5%, and thus the patient was referred to our department. Considering the patient’s young age, biochemical screening for MEN1 was performed, but serum calcium, PTH, gastrin, insulin, IGF-1, and prolactin were normal, as were NEN-specific tumor markers. The patient also had no symptoms of hormone hypersecretion, no clinical features of VHL or NF1, and no remarkable family history. Somatostatin receptor SPECT/CT was performed, reporting a primary pancreatic NEN, with multiple metastases to the liver, abdominal lymph nodes and left adrenal gland, and portal vein invasion. The patient received monthly intramuscular lanreotide injections, in combination with a systemic chemotherapy regimen. However, owing to the unsatisfactory response of the treatment and the significant adverse reactions, the patient was recommended for Peptide Receptor Radionuclide Therapy (PRRT). Following five Lu-177-DOTATATE PRRT cycles, partial response was observed, and debulking surgery is being considered as the next therapeutic step.

Conclusions: Widespread metastatic disease is often reported in pancreatic NENs, frequently requiring multiple lines of treatment. As such, multidisciplinary teams play a pivotal role in the management of such complex cases, carefully balancing risks, benefits, the biology of the tumor, and patients’ wishes.