PANCREATIC TUMORS IN MULTIPLE ENDOCRINE NEOPLASIA TYPE-1 - CLINICAL PRESENTATION AND SURGICAL-TREATMENT

被引:88
作者
GRAMA, D
SKOGSEID, B
WILANDER, E
ERIKSSON, B
MARTENSSON, H
CEDERMARK, B
AHREN, B
KRISTOFFERSON, A
OBERG, K
RASTAD, J
AKERSTROM, G
机构
[1] UNIV HOSP UPPSALA, DEPT SURG, S-75185 UPPSALA, SWEDEN
[2] UNIV HOSP UPPSALA, DEPT MED, S-75185 UPPSALA, SWEDEN
[3] UNIV HOSP UPPSALA, DEPT PATHOL, S-75185 UPPSALA, SWEDEN
[4] KAROLINSKA HOSP, HELINGSBORG HOSP, DEPT SURG, S-10401 STOCKHOLM 60, SWEDEN
[5] UNIV LUND HOSP, S-22185 LUND, SWEDEN
[6] UMEA UNIV HOSP, S-90185 UMEA, SWEDEN
关键词
D O I
10.1007/BF02067335
中图分类号
R61 [外科手术学];
学科分类号
摘要
Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in all patients undergoing pancreatic surgery, including those with negative localization studies prior to operation. The patients also had additional macroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal pancreatic resection, was performed in 18 patients, eventually combined with caput tumor enucleation or duodenotomy, while a few patients underwent only tumor enucleation or a Whipple procedure. The long-term outcome of operation was most favorable in patients with hyperinsulinism; only 1 patient had clinical recurrence. Patients with hypergastrinemia experienced only transitory lowering of serum gastrin values after pancreatic surgery and 47% of them had or developed metastases. Such tumor spread was seen in 57% of the patients with non-functioning lesions. Nine patients died from progressive tumor disease during follow-up. Consistent with previous studies, we found that surgery is indicated in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by radiology. In addition, these indications should be extended to also include patients with only biochemical markers of disease, including elevations of gastrin, as these indicate the presence of gross tumors. This strategy should be applied especially in patients with aggressive family histories to possibly reduce the risk of malignant tumor progression.
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页码:611 / 619
页数:9
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