Genka S, Soeda H, Takahashi M, Katakami H, Sanno N, Osamura Y, Fuchinoue T, Teramoto A
Department of Neurosurgery, Tokyo Metropolitan Toshima Hospital, Japan.
J Neurosurg. 1995 Oct;83(4):719-23. doi: 10.3171/jns.1995.83.4.0719.
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.
报告了一例52岁女性患者，患有肢端肥大症、尿崩症，因转移性生长激素释放激素（GRH）分泌性胰腺肿瘤导致视力受损。血清生长激素（GH）和生长介素C水平分别升高至14 ng/ml（正常<5 ng/ml）和3.20 U/ml（正常<1.88 U/ml）。葡萄糖耐量试验和促甲状腺激素释放激素刺激试验后，GH水平出现反常升高。计算机断层扫描和磁共振研究中观察到的垂体肿瘤活检显示为转移性癌。测量循环GRH水平时，血浆GRH显著升高（1145 pg/ml；正常<4 - 1 pg/ml）。患者因转移导致恶病质死亡。尸检显示胰腺存在原发性肿瘤，为恶性内分泌病变，垂体、肺、肝和肾上腺有转移性肿瘤。通过Northern印迹法和免疫组化分析证实肿瘤合成并分泌GRH。垂体显示增生性改变，但无腺瘤样改变。作者强调，对于肢端肥大症患者出现不寻常体征和症状时，探寻GRH的异位来源以及寻找分泌GH的垂体腺瘤均具有重要意义。
