Roca Elena, Mattogno Pier Paolo, Porcelli Teresa, Poliani Luigi, Belotti Francesco, Schreiber Alberto, Maffezzoni Filippo, Fontanella Marco Maria, Doglietto Francesco
Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Brescia, Italy.
Department of Endocrinology, Montichiari Hospital, Brescia, Italy.
World Neurosurg. 2018 Jun;114:e158-e164. doi: 10.1016/j.wneu.2018.02.120. Epub 2018 Mar 6.
Plurihormonal adenomas (PHAs) represent 10%-15% of all functioning pituitary adenomas. The most frequent hormonal associations are with prolactin and growth hormone (GH). Here we describe a rare case of functional adrenocorticotropic hormone (ACTH) and GH microadenoma and report our findings from a systematic literature review of PHA.
We searched PubMed using the terms "plurihormonal pituitary adenoma," "ACTH GH pituitary adenoma," and "acromegaly AND Cushing's disease". In the 17 articles that were selected for literature review, only 20% (4/20) of patients presented with clinical signs of both diseases. Histologically, 19 were pituitary adenomas composed of two distinct cell populations, while only in 1 case was there evidence of a single cell producing both ACTH and GH. In the case reported here, a 60-year-old woman was incidentally diagnosed with a pituitary microadenoma. Endocrine assessment documented increased levels of insulin-like growth factor 1 and GH; ACTH and cortisol values were within normal ranges. Echocardiography documented ventricular hypertrophy. Because of clinical and biochemical evidence of acromegaly, surgery was recommended. Postoperatively, hormonal replacement therapy was started because of adrenal insufficiency. Her antihypertensive therapy was discontinued due to evidence of normal blood pressure values. Histological examination revealed an ACTH-GH PHA with 2 distinct populations of secreting cells. At 3-year follow-up, the patient showed stable clinical remission and was no longer receiving hormonal replacement therapy.
This is an additional case to the 20 previously reported cases of ACTH-GH PHA. Awareness of this relatively rare entity is clinically relevant. The cytogenesis of ACTH-GH PHA remains a matter of debate, and several hypotheses have been postulated.
多激素腺瘤（PHA）占所有功能性垂体腺瘤的10% - 15%。最常见的激素组合是与催乳素和生长激素（GH）相关。在此，我们描述一例罕见的功能性促肾上腺皮质激素（ACTH）和GH微腺瘤病例，并报告我们对PHA进行系统文献综述的结果。
我们在PubMed上使用“多激素垂体腺瘤”、“ACTH - GH垂体腺瘤”以及“肢端肥大症和库欣病”等检索词进行搜索。在为文献综述所选的17篇文章中，仅有20%（4/20）的患者同时出现两种疾病的临床症状。组织学上，19例为垂体腺瘤，由两个不同的细胞群体组成，而仅有1例有证据表明单个细胞同时产生ACTH和GH。在本文报道的病例中，一名60岁女性偶然被诊断为垂体微腺瘤。内分泌评估显示胰岛素样生长因子1和GH水平升高；ACTH和皮质醇值在正常范围内。超声心动图显示心室肥厚。由于有肢端肥大症的临床和生化证据，建议进行手术。术后，因肾上腺功能不全开始激素替代治疗。由于血压值正常，停用了她的抗高血压治疗。组织学检查显示为ACTH - GH PHA，有两个不同的分泌细胞群体。在3年的随访中，患者临床缓解稳定，不再接受激素替代治疗。
这是此前报道的20例ACTH - GH PHA病例之外的又一例。认识到这种相对罕见的疾病实体具有临床意义。ACTH - GH PHA的细胞发生仍然是一个有争议的问题，并且已经提出了几种假说。
