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Am J Med Sci. 2018 Nov 10. pii: S0002-9629(18)30414-2. doi: 10.1016/j.amjms.2018.11.001. [Epub ahead of print]

Can a High-grade Neuroendocrine Carcinoma be Functional?

Author information

1
Division of Internal Medicine, University of Kentucky, Lexington, Kentucky. Electronic address: Christopher.graham@uky.edu.
2
Division of Medical Oncology, Markey Cancer Center, Lexington, Kentucky.

Abstract

We present a rare high-grade, functional, neuroendocrine carcinoma of the pancreas secreting vasoactive intestinal peptide. This case is unique, given the tumor's aggressive features and high Ki-67 index while also secreting functional hormones, which is unusual in high-grade neuroendocrine tumors (NETs). Our patient initially presented with diarrhea and was found to have a 4.7 × 3.1 × 3.3 cm pancreatic mass with diffuse hepatic metastasis staining positive for vasoactive intestinal peptide, chromogranin A and synaptophysin. She was initially underscored as grade 2 NET due to low mitosis, but subsequently restaged based on Ki-67 index as a grade 3 neuroendocrine carcinoma. She unfortunately was not a candidate for chemotherapy at that time. We conclude that high-grade NETs have the potential to secrete active peptide hormones. We therefore recommend that NETs with aggressive features be biopsied and assessed for Ki-67 activity and mitosis regardless if they are functional tumors, with goal for early initiation of chemotherapy.

KEYWORDS:

Neuroendocrine carcinoma; Neuroendocrine tumor; Vasoactive intestinal peptide

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