Two Different Pituitary Adenomas in a Patient with Multiple Endocrine Neoplasia Type 1 Associated with Growth Hormone-Releasing Hormone-Producing Pancreatic Tumor Clinical and Genetic Features

YASUMI SHINTANI; KATSUHIKO YOSHIMOTO; HIDEAKI HORIE; TOSHIAKI SANO; YOSHIKO KANESAKI; EMIKO HOSOI; YUTAKA YOKOGOSHI; HIROSHI BANDO; HIROYUKI IWAHANA; SEIJI KANNUKI; KEIZO MATSUMOTO; MITSUO ITAKURA; SHIRO SAITO

doi:10.1507/endocrj.42.331

Two Different Pituitary Adenomas in a Patient with Multiple Endocrine Neoplasia Type 1 Associated with Growth Hormone-Releasing Hormone-Producing Pancreatic Tumor

Clinical and Genetic Features

YASUMI SHINTANI, KATSUHIKO YOSHIMOTO, HIDEAKI HORIE, TOSHIAKI SANO, YOSHIKO KANESAKI, EMIKO HOSOI, YUTAKA YOKOGOSHI, HIROSHI BANDO, HIROYUKI IWAHANA, SEIJI KANNUKI, KEIZO MATSUMOTO, MITSUO ITAKURA, SHIRO SAITO

Author information

YASUMI SHINTANI
First Department of Internal Medicine, School of Medicine, The University of Tokushima
KATSUHIKO YOSHIMOTO
Otsuka Department of Clinical and Molecular Nutrition, School of Medicine, The University of Tokushima
HIDEAKI HORIE
First Department of Internal Medicine, School of Medicine, The University of Tokushima
TOSHIAKI SANO
Departments of Pathology, School of Medicine, The University of Tokushima
YOSHIKO KANESAKI
First Department of Internal Medicine, School of Medicine, The University of Tokushima
EMIKO HOSOI
First Department of Internal Medicine, School of Medicine, The University of Tokushima
YUTAKA YOKOGOSHI
First Department of Internal Medicine, School of Medicine, The University of Tokushima
HIROSHI BANDO
First Department of Internal Medicine, School of Medicine, The University of Tokushima
HIROYUKI IWAHANA
Otsuka Department of Clinical and Molecular Nutrition, School of Medicine, The University of Tokushima
SEIJI KANNUKI
Departments of Neurosurgery, School of Medicine, The University of Tokushima
KEIZO MATSUMOTO
Departments of Neurosurgery, School of Medicine, The University of Tokushima
MITSUO ITAKURA
Otsuka Department of Clinical and Molecular Nutrition, School of Medicine, The University of Tokushima
SHIRO SAITO
First Department of Internal Medicine, School of Medicine, The University of Tokushima

Keywords: MEN 1, Pituitary adenoma, Ectopic GHRH-producing tumor, Loss of heterozygosity, Chromosome 11

JOURNAL FREE ACCESS

1995 Volume 42 Issue 3 Pages 331-340

DOI https://doi.org/10.1507/endocrj.42.331

Details

Abstract

The clinical and genetic features of a 43-year-old male patient with multiple endocrine neoplasia type 1 were reported. He developed hyperparathyroidism, a GHRH-producing pancreatic tumor, and acromegaly between 1980 and 1983. Because his pituitary gland increased in size even after resecting the GHRH-producing pancreatic tumor, transsphenoidal hypophysectomy was performed six years later. The pituitary contained two histologically-different adenomas composed of somatotroph cells and null cells. Genetic analyses revealed loss of heterozygosity on chromosome 11 in common in the pituitary adenomas, the pancreatic endocrine tumors, and a parathyroid hyperplasia. On the other hand, mutations of ras, p53, Gsα, and Gi2α genes were not found in these tumors. The loss of the tumor suppressor gene on chromosome 11q12-13 was involved in the formation of two pituitary adenomas, two pancreatic endocrine functioning tumors, and a parathyroid hyperplasia in this patient, but the tumorigenic factors in the specific endocrine organs remain to be studied.

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