gastrins and Neoplasm-Regression--Spontaneous

Of 44 patients with the Zollinger-Ellison syndrome treated at our institution, nine appeared to have undergone "regression" of their gastrinomas. Six of the nine patients had sporadic gastrinomas and became permanently eugastrinemic following excision of nodal metastases and total gastrectomy (n = 4), antrectomy (n = 1), or pancreatoduodenectomy (n = 1) (mean survival, 13 years). The other three patients had Zollinger-Ellison syndrome as part of the multiple endocrine adenopathy type 1 syndrome and became temporarily eugastrinemic after total gastrectomy (mean survival, 11 years). Occult submucosal duodenal-wall microgastrinomas (mean size, 3.0 mm) were found to have been serendipitously excised in four patients. Long-term follow-up of these nine patients, as well as of six other patients described in the literature, demonstrates that excision of occult duodenal-wall gastrinomas provides a plausible explanation for the phenomenon of apparent regression of primary gastrinomas and the eugastrinemia that may follow total gastrectomy.

Topics: Adolescent; Adult; Aged; Duodenal Neoplasms; Female; Follow-Up Studies; Gastrectomy; Gastrinoma; Gastrins; Humans; Male; Middle Aged; Neoplasm Regression, Spontaneous; Neoplasms, Multiple Primary; Neoplasms, Unknown Primary; Pancreatic Neoplasms; Stomach Neoplasms; Zollinger-Ellison Syndrome

The effect of total gastrectomy on the biologic behavior of malignant gastrinomas was studied from patient data collected in the ZE tumor registry. A total of 267 patients with documented metastatic tumor had definitive gastric operations. In the 137 patients who had total gastrectomy, survival was 75% at one year, 55% at five years and 42% at ten years. In the 130 patients who had lesser gastric operations, survival was 51% at one year, 27% at five years and 18% at ten years. Deaths from progressive tumor growth occurred in 17% of the patients at risk after total gastrectomy and 30% of the patients at risk after lesser gastric operations. A subgroup of 127 patients with documented liver metastasis had definitive gastric operations. Seventythree patients with liver metastasis had total gastrectomy with survival of 68% at one year, 42% at five years and 30% at ten years. Fifty-four patients with liver metastasis had lesser gastric operations with survival of 44% at one year, 7% at five years and none at ten years. Deaths from progressive tumor growth occurred in 25% of the patients at risk after total gastrectomy and 50% of the patients at risk after lesser gastric operations. Regression of metastatic ZE tumor was clearly documented in only four patients; all had total gastrectomy. Presumptive regression of primary tumor occurred in seven patients, five had total gastrectomy. The study clearly demonstrated that total gastrectomy was the procedure of choice for malignant ZE tumors, even in the presence of widespread metastasis. The results provided indirect evidence to support a gastric feedback effect which influences growth of gastrinomas; however, the results also show that total gastrectomy furnished neither predictable nor permanent protection from subsequent tumor growth and metastasis.

Topics: Adolescent; Adult; Female; Gastrectomy; Gastrins; Humans; Liver Neoplasms; Male; Neoplasm Metastasis; Neoplasm Regression, Spontaneous; Pancreatic Neoplasms; Prognosis; Zollinger-Ellison Syndrome