General information about pancreatic endocrine neoplasms:

1) 3-5% of pancreatic neoplasms

2) “Islet cell tumors” is inaccurate, as they are believed to arise from pluripotential ductal cells that have the capacity to differentiate along neuroendocrine lines

3) All are considered malignant except microadenomas (less than 5 mm, usually incidental at autopsy), since are no definite histologic criteria to differentiate benign and malignant (except for metastases);

4) no TNM staging exists for these tumors

5) One proposal: classify as low grade if no necrosis and 0-1 MF/50 HPF; as intermediate grade if necrosis OR 2+ MF/50 HPF

5) 80% occur in MEN1 patients, usually age 50-60

6) Most tumors are functional and secrete multiple hormones, hCG

7) Usually occurs in adults in body/tail

8) Tumors are either syndromic or not; non-syndromic may produce hormones so don’t call “non-functional”

9) Tumors are hypervascular and circumscribed with octreotide scan (highlights somatostatin receptors),

10) Usually slow growing, metastases to nodes, liver, bone; recommend resection of metastases for palliative relief