Attanasio Roberto, Montini Marcella, Valota Monia, Cortesi Liana, Barbò Regina, Biroli Francesco, Tonnarelli Giampiero, Albizzi Mascia, Testa Rosa Miranda, Pagani Giorgio
Endocrinology, Istituto Ortopedico Galeazzi, Dr. Roberto Attanasio, via Compagnoni 1, Milano 20129, Italy.
Pituitary. 2008;11(1):1-11. doi: 10.1007/s11102-007-0059-1.
Acromegaly is a chronic disease impacting on morbidity and mortality. Increased mortality is reverted after the achievement of hormonal targets. The relative role of treatment options is still matter of debate.
A retrospective chart review was performed on all the acromegalic patients attending our center along the last 20 years.
Data about 159 patients (83 F) were retrieved and analyzed: 18% had been lost to follow-up, while follow-up was >5 years in 79%. Growth hormone (GH) at diagnosis was 24 microg/L (median, range 3-239). Pituitary MRI showed a macro-, micro-adenoma or no lesion in 73.6, 22.9, and 3.5%, respectively. Hyperprolactinemia (hyperPRL) was present in 20.8%. Ninety-six and 29 patients had been treated by neurosurgery (NS) and irradiated (RT), respectively. Drugs had been employed in 149 patients (in 58 as the only treatment). At the last evaluation, 22% of patients were cured (hypopituitarism and GH deficiency in 6.3%), 37.1% were controlled by ongoing pharmacological treatment, 22.6% had discordant GH and Insulin-like growth factor I (IGF-I) values, and 18.2% had still active disease (median follow-up in this last group was 9 months). By evaluating the outcome with a multimodal approach, safe GH and normal IGF-I had been achieved in 78 and 63.5% of the whole series, 80.5 and 59.7% in patients submitted to NS (and adjuvantly treated with drugs), 95.8 and 91.7% in those submitted to NS + RT (and drugs as well), 70.2 and 55.2% in those treated only with drugs (increased to 82.2 and 60.9% if considering only patients treated with modern long-acting drugs). Hypopituitarism had occurred in 25, 66.6, and 13.8% in the three groups, respectively. At multivariate analysis, previous RT and NS were significant positive predictors of cure, whereas previous NS, follow-up, and year of diagnosis were significant positive predictors of control. Diabetes was a negative predictor both of cure and control. Sex, age, baseline GH levels, hyperPRL, tumor size, extrasellar extension, and invasiveness were not independent predictors of either cure or control.
This series seems to indicate that a multimodal approach can achieve control of disease in most patients.
肢端肥大症是一种影响发病率和死亡率的慢性疾病。在实现激素目标后,死亡率增加的情况会得到逆转。治疗方案的相对作用仍存在争议。
对过去20年在我们中心就诊的所有肢端肥大症患者进行回顾性病历审查。
检索并分析了159例患者(83例女性)的数据:18%失访,79%的患者随访时间>5年。诊断时生长激素(GH)水平为24μg/L(中位数,范围3 - 239)。垂体MRI显示大腺瘤、微腺瘤或无病变的比例分别为73.6%、22.9%和3.5%。高泌乳素血症(hyperPRL)发生率为20.8%。分别有96例和29例患者接受了神经外科手术(NS)和放射治疗(RT)。149例患者使用了药物(58例仅使用药物治疗)。在最后一次评估时,22%的患者治愈(6.3%存在垂体功能减退和GH缺乏),37.1%的患者通过持续药物治疗得到控制,22.6%的患者GH和胰岛素样生长因子I(IGF - I)值不一致,18.2%的患者仍有活动性疾病(最后一组患者的中位随访时间为9个月)。通过多模式方法评估结果,整个系列中分别有78%和63.5%的患者实现了安全的GH水平和正常的IGF - I水平;接受NS治疗(并辅助药物治疗)的患者中这一比例分别为80.5%和59.7%;接受NS + RT治疗(以及药物治疗)的患者中分别为95.8%和91.7%;仅接受药物治疗的患者中分别为70.2%和55.2%(如果仅考虑使用现代长效药物治疗的患者,这一比例分别提高到82.2%和60.9%)。三组患者垂体功能减退的发生率分别为25%、66.6%和13.8%。多因素分析显示,既往RT和NS是治愈的显著阳性预测因素,而既往NS、随访时间和诊断年份是控制的显著阳性预测因素。糖尿病是治愈和控制的阴性预测因素。性别、年龄、基线GH水平、hyperPRL、肿瘤大小、鞍外扩展和侵袭性均不是治愈或控制的独立预测因素。
本系列研究似乎表明,多模式方法可使大多数患者的疾病得到控制。