Abstract
The major phenotypes of multiple endocrine neoplasia type 1 (MEN 1) consist of three lesions characterized by hyperparathyroidism, pituitary tumors, and endocrine pancreatic tumors. The endocrine pancreatic tumors are a significant cause of disease-related mortality in MEN 1. Although symptomatic pancreatic tumors such as insulinoma and gastrinoma should be resected, the management of asymptomatic pancreatic tumors is not established. In asymptomatic pancreatic tumors, the most important factor is the propensity for malignant transformation of the tumors. Although there are no means to foresee it, the size of the pancreatic tumors might be predictive of malignant development in MEN 1. We report here a patient with MEN 1 who had a large asymptomatic pancreatic tumor. The patient (72-yr-old man) was diagnosed with primary hyperparathyroidism and underwent a total parathyroidectomy. Genetic examination showed a germline mutation of the MEN1 gene (E45G). Abdominal magnetic resonance imaging revealed a large (>6 cm) tumor with a heterogeneous pattern in the tail of the pancreas. No metastases of the tumor were evident. Serum levels of insulin, gastrin, and glucagon were normal, and the patient had no symptoms. Operative resection was performed, and microscopic examination revealed that the tumor was an islet cell tumor stained with multiple hormones. This is a case indicating that asymptomatic pancreatic tumors associated with MEN 1 might be indolent independent of their size.
Similar content being viewed by others
References
Trump, D., Farren, B., Wooding, C., et al. (1996) Quart. J. Med. 89, 653–659.
Vasen, H. F., Lamers, C. B., and Lips, C. J. (1989) Arch. Intern. Med. 149, 2717–2722.
Sheppherd, J. J. (1991) Arch. Surg. 126, 935–952.
Sato, M., Miyauchi, A., and Takahara, J.,(2000) Biomed. Pharmacother. 54, 86–89.
Chandrasekharappa, S. C., Guru, S. C., Manickam, P., et al. (1997) Science 276, 404–407.
Agarwal, S. K., Kester, M. B., Debelenko, L. V., et al. (1997). Hum. Mo. I. Genet. 6, 1169–1175.
Lemmens, I., Van de Ven, W. J., Kas, K. Hum. Mol. Genet. (1997), 6, 1177–1183.
Shimizu, S., Tsukada, T., Futami, H., et al. (1997) Cancer Res. 83, 1029–1032.
Bassett, J. H., Forbes, S. A., Pannett, A. A., et al. (1998) Am. J. Hum. Genet. 62, 232–244.
Sato, M., Matsubara, S., Miyauchi, A., Ohye, H., Imachi, H., Murao, K., and Takahara, J. (1998) J. Med. Genet. 35, 915–919.
Oberg, K. and Skogseid, B. (1998) J. Intern Med. 243, 471–476.
Skogseid, B., Eriksson, B., Lundqvist, G., Lorelius, L. E., Rastad, J., Wide, L., Akerstrom, G., and Oberg, K. (1991) J. Clin. Endocrinol. Metab. 73, 281–287.
Cadiot, G., Vuagnat, A., Doukhan, I., et al. (1999) Gastroenterology 116, 286–293.
Le Bodic M.-F., Heyman M.-F., Lecomte M., et al. (1996), Am. J. Surg. Pathol. 20, 1378–1384.
Lowney, J. K., Frisella, M. M., Lairmore, T. C., and Doherty, G. M. (1998) Surgery 124, 1043–1048.
Skogseid, B., Oberg, K., Benson, L., et al. (1987) J. Clin. Endocrinol. Metab. 64, 1233–1240.
Granberg, D., Stridsberg, M., Seensalu, R., Eriksson, B., Lundqvist, G., Oberg, K., and Skogseid, B (1999) J. Clin. Endocrinol. Metab. 84, 2712–2717.
Oberg, K., Skogseid, B., and Eriksson, B. (1989) Acta Oncol. 28, 383–387.
Mulligan, L. M., Kwok, J. B., Healey, C. S., et al. (1993), Nature 363, 458–460.
Guru S. C., Goldsmith, P. K., Burns, A. L., Marx, S. J., Spiegel, A. M., Collins, F. S., and Chandrasekharappa, S. C. (1998) Proc. Natl. Acad. Sci. USA 95, 1630–1634.
Agarwal, S. K., Guru, S. C., Heppner, C., et al. (1999), Cell 96, 143–152.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Sato, M., Kihara, M., Nishitani, A. et al. Large and asymptomatic pancreatic islet cell tumor in a patient with multiple endocrine neoplasia type 1. Endocr 13, 263–266 (2000). https://doi.org/10.1385/ENDO:13:3:263
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1385/ENDO:13:3:263