SOMATOTROPH HYPERPLASIA - SUCCESSFUL TREATMENT OF ACROMEGALY BY REMOVAL OF A PANCREATIC-ISLET TUMOR SECRETING A GROWTH HORMONE-RELEASING FACTOR
SOMATOTROPH HYPERPLASIA - SUCCESSFUL TREATMENT OF ACROMEGALY BY REMOVAL OF A PANCREATIC-ISLET TUMOR SECRETING A GROWTH HORMONE-RELEASING FACTOR
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DOI：
10.1172/jci110708
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发表时间：
1982-01-01
影响因子：
15.9
通讯作者：
KOVACS, K
中科院分区：
文献类型：
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作者：
THORNER, MO;PERRYMAN, RL;KOVACS, K
一名21岁的女性特纳综合征表现为肢端肥大症的体征和症状。血清生长激素（GH）（95 ± 0.01）。9.4 ng/ml;平均值±。SEM [平均值的标准误差]）和生长调节素C（11 U/ml）水平升高，观察到葡萄糖后GH水平升高而不是正常抑制，TRH给药后GH水平升高而不是无变化，多巴胺给药后GH水平降低而不是刺激。垂体窝体积大于正常值（1440 mm 3），并假设存在垂体肿瘤。经蝶手术切除组织后，组织学研究显示生长激素增生，而不是一个离散的腺瘤。手术后，她仍处于临床肢端肥大症状态，并继续显示GH和生长调节素水平升高。对生长激素释放因子（GRF）的异位来源进行了研究。电脑断层扫描显示一个5厘米直径的肿瘤在尾部的胰腺。切除肿瘤后，血清GH在2小时内从70 ng/ml降至3 ng/ml，并在随后的5个月内保持较低水平。术后6周，血清生长调节素C水平从7.2降至正常。对葡萄糖、TRH和多巴胺不再有矛盾的GH反应。在手术中保持肿瘤细胞的培养基和肿瘤提取物都含有具有GRF活性的肽。肿瘤提取物中的GRF在Sephadex G-50柱层析上共洗脱，具有大鼠下丘脑GH释放活性。使用肿瘤提取物，在纳摩尔范围内实现了体外大鼠生长激素细胞的GH刺激。病人的病程证明了仔细解释垂体组织学的重要性。蝶鞍扩大患者血清GH和生长调节素C水平升高，肢端肥大症患者对TRH、多巴胺和葡萄糖的特征性反应并不只发生在离散性垂体瘤和肢端肥大症患者。这种情况也可能发生在生长激素细胞增生，然后在去除GRF源后恢复正常。因此，肢端肥大症患者应考虑异位GRF分泌，因此，仔细的垂体组织学检查是强制性的。在垂体手术前考虑胸部和腹部的计算机断层扫描，尽管它们的产率低，可能是合理的。
A 21-yr-old woman with Turner''s syndrome presented with signs and symptoms of acromegaly. The serum growth hormone (GH) (95 .+-. 9.4 ng/ml; mean .+-. SEM [standard error of the mean]) and somatomedin C (11 U/ml) levels were elevated, and an increase in GH levels after glucose instead of normal suppression, increase after TRH administration instead of no change, and decrease after dopamine administration instead of stimulation were observed. The pituitary fossa volume was greater than normal (1440 mm3) and the presence of a pituitary tumor was assumed. After tissue removal at transsphenoidal surgery, histological study revealed somatotroph hyperplasia rather than a discrete adenoma. Postoperatively, she remained clinically acromegalic and continued to show increased GH and somatomedin levels. A search was made for ectopic source of a growth hormone-releasing factor (GRF). Computer tomographic scan revealed a 5-cm diameter tumor in the tail of the pancreas. Following removal of this tumor, serum GH fell from 70 to 3 ng/ml over 2 h, and remained low for the subsequent 5 mo. Serum somatomedin C levels fell from 7.2 to normal by 6 wk postoperatively. There were no longer paradoxical GH responses to glucose, TRH, and dopamine. Both the medium that held the tumor cells at surgery and extracts of the tumor contained a peptide with GRF activity. The GRF contained in the tumor extract coeluted on Sephadex G-50 chromatography with rat hypothalamic GH-releasing activity. Stimulation of GH from rat somatotrophs in vitro was achieved at the nanomolar range, using the tumor extract. The patient''s course demonstrates the importance of careful interpretation of pituitary histology. Elevated serum GH and somatomedin C levels in a patient with an enlarged sella turcica and the characteristic responses seen in acromegaly to TRH, dopamine and glucose do not occur exclusively in patients with discrete pituitary tumors and acromegaly. This condition can also occur with somatotroph hyperplasia and then revert to normal after removal of the GRF source. Thus, in patients with acromegaly a consideration of ectopic GRF secretion should be made, and therefore, careful pituitary histology is mandatory. Consideration for chest and abdominal computer tomographic scans before pituitary surgery, in spite of their low yield, may be justified.