Acromegaly, diabetes insipidus, and visual loss caused by metastatic growth hormone-releasing hormone-producing malignant pancreatic endocrine tumor in the pituitary gland. Case report.
The case of a 52-year-old woman with acromegaly, diabetes insipidus, and visual impairment caused by a metastatic growth hormone-releasing hormone (GRH)-produced pancreatic tumor is reported. Serum growth hormone (GH) and somatomedin C levels were elevated to 14 ng/ml (normal < 5 ng/ml), and 3.20 U/ml (normal < 1.88 U/ml), respectively. Paradoxical increases were observed in GH levels after glucose tolerance and thyrotropin-releasing hormone-stimulation tests. Biopsy of a pituitary tumor observed on computerized tomography scans and magnetic resonance studies revealed a metastatic cancer. When circulating GRH levels were measured, a marked increase in plasma GRH (1145 pg/ml; normal < 4-1 pg/ml) was observed. The patient died of cachexia due to metastases. Postmortem examination revealed that a primary tumor, a malignant endocrine lesion, was present in the pancreas, with metastatic tumors in the pituitary, lung, liver, and adrenal glands. Synthesis and production of GRH by the tumor was demonstrated by Northern blotting and immunohistochemical analysis. The pituitary gland showed hyperplastic, but not adenomatous changes. The authors stress the importance of both exploration for an ectopic source of GRH and the search for a GH-producing pituitary adenoma when unusual signs and symptoms are seen in patients with acromegaly.
Genka
S
S
Department of Neurosurgery, Tokyo Metropolitan Toshima Hospital, Japan.
Soeda
H
H
Takahashi
M
M
Katakami
H
H
Sanno
N
N
Osamura
Y
Y
Fuchinoue
T
T
Teramoto
A
A
D000172:Acromegaly / Q000209:etiology*; D000236:Adenoma / Q000378:metabolism*; D008949:Adenoma, Pleomorphic / Q000378:metabolism; D001766:Blindness / Q000209:etiology*; D003919:Diabetes Insipidus / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D005951:Glucose Tolerance Test; D013006:Growth Hormone / Q000097:blood; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000378:metabolism*; D010911:Pituitary Neoplasms / Q000378:metabolism* / Q000556:secondary*; D013973:Thyrotropin-Releasing Hormone
D013973:Thyrotropin-Releasing Hormone; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone