Lefebvre S, De Paepe L, Abs R, Rahier J, Selvais P, Maiter D
Division of Rheumatology, Clinique du Refuge, Mouscron, Belgium.
Eur J Endocrinol. 1995 Sep;133(3):320-4. doi: 10.1530/eje.0.1330320.
Diagnosis of ectopic acromegaly was made in a 21-year-old female patient who 3 years before had undergone a right pneumectomy for a disseminated bronchial carcinoid. Plasma growth hormone-releasing hormone (GHRH) concentrations were markedly elevated (6440 ng/l; normal value < 100 ng/l), as were serum GH (187 micrograms/l; normal < 5 micrograms/l) and plasma insulin-like growth factor I (IGF-I) levels (6.7 U/ml; normal < 2 U/ml). Retrospective immunohistochemical examination of the carcinoid tumor was positive for GHRH and the tumoral content of GHRH was 2130 ng/g wet weight. Subcutaneous treatment with octreotide was begun and first resulted in a profound inhibition of GH hypersecretion, normalization of plasma IGF-I and only partial reduction of GHRH concentrations. However, the initial dose of 3 x 100 micrograms had to be increased gradually to 4 x 750 micrograms because of a progressive deterioration of the hormonal control. After 15 months of intermittent therapy, octreotide was administered by continuous sc infusion. This treatment improved compliance, allowed the daily dose of octreotide to be reduced to 1500 micrograms and normalized serum GH levels. A near-normalization of the plasma IGF-I concentrations was also obtained, whereas the suppression of plasma GHRH concentrations remained incomplete. Despite favorable evolution of the endocrine parameters, intramedullar metastases were diagnosed and required radiation therapy. This observation emphasizes the superiority of continuous over intermittent administration of octreotide in the treatment of ectopic acromegaly. It also shows that the somatostatin analog acts more at the pituitary level to inhibit GH secretion than at the site of the neuroendocrine tumor.
一名21岁女性患者被诊断为异位性肢端肥大症,该患者3年前因弥漫性支气管类癌接受了右肺切除术。血浆生长激素释放激素(GHRH)浓度显著升高(6440 ng/l;正常值<100 ng/l),血清GH(187微克/升;正常<5微克/升)和血浆胰岛素样生长因子I(IGF-I)水平(6.7 U/ml;正常<2 U/ml)也升高。对类癌肿瘤进行回顾性免疫组化检查,结果显示GHRH呈阳性,肿瘤组织中GHRH含量为2130 ng/g湿重。开始皮下注射奥曲肽治疗,最初导致GH分泌过多受到显著抑制,血浆IGF-I恢复正常,而GHRH浓度仅部分降低。然而,由于激素控制逐渐恶化,初始剂量3×100微克不得不逐渐增加至4×750微克。经过15个月的间歇治疗后,改为奥曲肽持续皮下输注。这种治疗提高了依从性,使奥曲肽的每日剂量降至15,00微克,并使血清GH水平恢复正常。血浆IGF-I浓度也接近正常,而血浆GHRH浓度的抑制仍不完全。尽管内分泌参数有所改善,但仍诊断出髓内转移,需要进行放射治疗。该观察结果强调了奥曲肽持续给药优于间歇给药治疗异位性肢端肥大症。这也表明,生长抑素类似物在垂体水平抑制GH分泌的作用比在神经内分泌肿瘤部位更明显。