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与分泌生长激素和催乳素的垂体肿瘤相关并经经蝶窦垂体切除术治愈的巨人症。

Gigantism associated with a pituitary tumour secreting growth hormone and prolactin and cured by transsphenoidal hypophysectomy.

作者信息

Favre L, Rogers L M, Cobb C A, Rabin D

出版信息

Acta Endocrinol (Copenh). 1979 Jun;91(2):193-200. doi: 10.1530/acta.0.0910193.

Abstract

An 18-year old male is reported who presented with a history of a growtn spurt over the year preceding his admission. His height was above the 97th percentile, and he had incompletely developed secondary sexual characters. Pituitary evaluation demonstrated a moderately elevated level of growth hormone (hGH) not suppressible by a glucose load and not stimulable by TRH or by L-DOPA. Serum prolactin (PRL) concentration was also increased while gonadotrophin, thyroid and adrenal function were all subnormal. There was clear radiological evidence of a large pituitary tumour with suprasellar extension and transsphenoidal total hypophysectomy was performed. A mixed chromophobe and acidophilic adenoma was found and both growth hormone and prolactin were demonstrable in different cells of the tumour by the immunoperoxidase technique. Post-operatively the patient has hypopituitarism and levels of growth hormone and prolactin have remained low or undetectable after 6 months. Thus early diagnosis and surgical treatment of gigantism of this mixed hGH-PRL secreting pituitary tumour was associated with a cure, which contrasts with the unfavourable outcome of many of the patients previously reported.

摘要

据报道,一名18岁男性在入院前一年有生长加速史。他的身高高于第97百分位,第二性征发育不完全。垂体评估显示生长激素(hGH)水平中度升高,葡萄糖负荷试验不能抑制,促甲状腺激素释放激素(TRH)或左旋多巴(L-DOPA)刺激试验无反应。血清催乳素(PRL)浓度也升高,而促性腺激素、甲状腺和肾上腺功能均不正常。影像学检查明确显示有一个巨大的垂体肿瘤,向上延伸至鞍上,遂行经蝶窦全垂体切除术。术后发现是嫌色细胞和嗜酸细胞混合性腺瘤,通过免疫过氧化物酶技术在肿瘤的不同细胞中均检测到生长激素和催乳素。术后患者出现垂体功能减退,6个月后生长激素和催乳素水平仍低或检测不到。因此,对于这种分泌生长激素和催乳素的混合性垂体肿瘤导致的巨人症,早期诊断和手术治疗可实现治愈,这与之前报道的许多患者的不良预后形成对比。

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