Table 1.

Clinicopathologic Comparison of Pancreatic Neuroendocrine Tumors (PanNETs) and Pancreatic Neuroendocrine Carcinomas (PanNECs)

	PanNETs	PanNECs
Age, mean, y	50–56	59–65
Gender, M:F	1:1	7:5
Distribution in pancreas	Tail > body	Head
Positive imaging modalities	Somatostatin receptor scintigraphy	Positron emission tomography
Serum findings	Elevated CgA	Elevated NSE
Metastasis, %	34 (G1, G2)	95–100
	81% (G3)
Ki-67, %	0–10.2 (G1, G2)	50–70
	<55% (G3)
Clinical history
Course	Indolent	Aggressive
History of PanNET G1, G2	May be present	Not present
Histology
Differentiation	Relatively well	Poor
Necrosis	Rare	Common
Mitosis and apoptotic cells	Few	Abundant
Non-NE components	No	May be present
Immunohistochemistry stains	Loss of DAXX/ATRX	Abnormal expression of TP53, loss of RB
Molecular findings	MEN1, DAXX/ATRX, mTOR mutations	TP53, RB1, KRAS, SMAD4 mutations
Recommended therapy	Somatostatin analogues, sunitinib/everolimus	Alkylating agent, antimetabolite, platinum-based chemotherapy

Abbreviations: ATRX, a-thalassemia/mental retardation syndrome X-linked;CgA, chromogranin A;DAXX, death domain-associated protein;G1, grade 1;G2, grade 2; G3, grade 3;KRAS, Kirsten rat sarcoma;MEN1, multiple endocrine neoplasia type 1;mTOR, mammalian target of rapamycin; NE, neuroendocrine;NSE, neuron-specific enolase;RB, retinoblastoma protein;SMAD4, mothers against decapentaplegic homolog 4;TP53, tumor protein 53.