Gastrin Cell Neoplasm / Gastrinoma of the Duodenum
Definition
- Duodenal neoplasm of gastrin producing cells, termed gastrinoma if there is clinical evidence of gastrin secretion
Alternate/Historical Names
-
G cell neoplasm
Diagnostic Criteria
- Histologic appearance is indistinguishable from other GI well differentiated endocrine neoplasms
- Uniform cells with bland round regular nuclei
- Trabecular and pseudo glandular growth patterns
- Perivascular pseudo rosettes
- Gastrin can be immunohistologically identified in both functioning and non-functioning neoplasms
- Other markers variably demonstrable include pancreatic polypeptide, insulin and somatostatin)
- Non-functioning gastrin cell (G cell) neoplasms nearly all solitary and located in bulb of duodenum
- Usually benign
- 15% of duodenal G cell neoplasms are gastrinomas (functioning) with associated Zollinger-Ellison (ZE) syndrome
- G cell neoplasms are termed gastrinomas if there is clinical evidence of gastrin secretion(ZE syndrome)
- Increasing numbers of ZE patients are reported to be associated with duodenal gastrinomas (up to 70%)
- Remainder appear to be due to pancreatic neoplasms
- Earlier reports of primary nodal gastrinomas and of diffuse gastrin overproduction in the stomach appear to be due instead to very small (<1 mm) duodenal neoplasms
- Functioning G cell neoplasms (gastrinomas) may be solitary or multiple and may be located throughout the duodenum or into proximal jejunum
- Local lymph nodes frequently involved (50-80%)
- May occur even if primary neoplasm is <1 mm
- Liver involved at presentation in about 5%
- Local lymph nodes frequently involved (50-80%)
- Most cases of ZE syndrome due to sporadic gastrinomas
- Approximately 20% associated with MEN1
- Duodenal G cell neoplasms associated with MEN1 are more often multiple
- Same behavior as sporadic neoplasms if matched for function and stage
- Duodenal G cell neoplasms associated with MEN1 are more often multiple
- Sporadic G cell neoplasms more often solitary and <1 cm
- 10 year survival >90%
for duodenal gastrinomas without liver metastases
(Weber 1995, Stabile 1985)
- 10 year survival if liver metastases found at presentation 30%
- Local lymph node involvement not predictive of poor behavior
- 10 year survival if regional nodes involved also >90%
- Same behavior even if incompletely resected
- Note that WHO 2008 incorrectly gives 10 year survival of 59% for duodenal gastrinomas without liver metastases, referencing Weber 1995 who actually reported 94% survival
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates : 7/27/10, 11/22/11, 12/28/11
