Table 5.

Subtypes of pancreatic endocrine tumors

Tumor type	Annual incidence (cases per million)	percentage of all PETs	Age (years)	Percent malignant§	Percent located in the pancreas	Percent associated with MEN-1
Insulinoma	0.7–4.0	30–45	30–60	5–10	> 95	4–8
Gastrinoma	0.5–4.0	16–30	20–70	40–90	25–70	12–22
VIPoma	0.1–0.6	< 10	20–80	> 50	75–90	6–11
Glucagonoma	≤ 0.1	< 10	40–60	> 50	> 95	5–13
Somatostatinoma	< 0.1	< 5	30–80	> 60	40–70	2–7
Other hormones*	rare	< 1	-	-	-	unknown
Nonfunctioning‡ (clinically silent)	≤ 1 0.1–10% in autopsy series	25–100†	50–60	> 50	100	0–21

Data compiled from original articles and reviews.(Creutzfeldt 1980; Demeure, et al. 1991; Eriksson et al. 1989; Grimelius et al. 1975; Halfdanarson et al. 2007; Harris et al. 1987; Jensen 1999; Kimura et al. 1991; Klöppel and Heitz 1988; Lam and Lo 1997; Lepage et al. 2004; Mansour and Chen 2004; Öberg and Eriksson 2005; Roy et al. 2000; Service et al. 1991; Smith et al. 1998; Soga and Yakuwa 1994, 1998b, 1999; Solcia et al. 1997; Tomassetti, et al. 2001; Warner 2005; Watson et al. 1989; Weil 1985; Wermers et al. 1996)

^*Data on these tumors is insufficient for further analysis

^†The higher percentage comes from autopsy studies

^‡Includes tumors that produce pancreatic polypeptide (PP)

^§Malignant behavior based on the presence of invasion and metastases