Drange M R, Melmed S
Division of Endocrinology and Metabolism, Cedars-Sinai Research Institute, University of California School of Medicine, Los Angeles 90048, USA.
J Clin Endocrinol Metab. 1998 Sep;83(9):3104-9. doi: 10.1210/jcem.83.9.5088.
Ectopic GHRH-secreting tumors, such as carcinoid, rarely cause acromegaly. As protracted exposure to high levels of GH is associated with considerable morbidity and mortality, these patients require early and effective medical therapy to control hormonal hypersecretion. We employed a prolonged release somatostatin analog, lanreotide, to treat a patient with disseminated GHRH-producing carcinoid. Before treatment, the patient had a biochemical profile characteristic of active acromegaly. Plasma GHRH levels were markedly elevated (200-fold), and urinary 5-hydroxyindolacetic acid (5-HIAA) levels were increased (4-fold). Magnetic resonance imaging revealed a large asymmetrical pituitary mass consistent with somatotroph hyperplasia. Somatostatin receptor scintigraphy revealed multiple bony and soft tissue lesions as well as striking pituitary uptake. Lanreotide (30 mg) was administered weekly by im injection for 12 weeks. Rapid and sustained symptomatic clinical improvement with diminished soft tissue swelling and hyperhidrosis was observed. GHRH levels decreased by 70%; glucose-suppressed GH and insulin-like growth factor I levels were reduced by 90% and 75%, respectively, to near normal values; urinary 5-HIAA levels normalized; and the pituitary mass remained unchanged. Unfortunately, the patient died due to complications of osteogenic sarcoma. In conclusion, prolonged release lanreotide induced clinical and biochemical remission in this patient with diffusely metastatic GHRH-producing carcinoid. This long-acting drug thus offers an effective, well tolerated, and convenient medical therapy for control of hormonal hypersecretion induced by excess GHRH.
异位分泌生长激素释放激素(GHRH)的肿瘤,如类癌,很少引起肢端肥大症。由于长期暴露于高水平的生长激素(GH)会导致相当高的发病率和死亡率,这些患者需要早期有效的药物治疗来控制激素分泌过多。我们使用长效生长抑素类似物兰瑞肽来治疗一名患有播散性分泌GHRH类癌的患者。治疗前,患者具有活跃肢端肥大症的生化特征。血浆GHRH水平显著升高(200倍),尿5-羟吲哚乙酸(5-HIAA)水平升高(4倍)。磁共振成像显示垂体有一个不对称的大肿块,符合生长激素细胞增生。生长抑素受体闪烁扫描显示多个骨和软组织病变以及明显的垂体摄取。兰瑞肽(30毫克)每周皮下注射一次,共12周。观察到软组织肿胀和多汗症迅速且持续的症状性临床改善。GHRH水平下降了70%;葡萄糖抑制的GH和胰岛素样生长因子I水平分别降低了90%和75%,降至接近正常水平;尿5-HIAA水平恢复正常;垂体肿块保持不变。不幸的是,患者因骨肉瘤并发症死亡。总之,长效兰瑞肽在这名患有弥漫性转移性分泌GHRH类癌的患者中诱导了临床和生化缓解。因此,这种长效药物为控制由过量GHRH引起的激素分泌过多提供了一种有效、耐受性良好且方便的药物治疗方法。