Stewart L, Steinbok P, Daaboul J
Department of Paediatrics, British Columbia's Children's Hospital, University of British Columbia, Vancouver, Canada.
J Neurosurg. 1998 Feb;88(2):340-5. doi: 10.3171/jns.1998.88.2.0340.
Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (> 7 cm/year), bone age remained advanced (> +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery. Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.
下丘脑错构瘤（HHs）是一种常与中枢性性早熟相关的良性肿瘤。对于部分带蒂病变的特定病例，尤其是幼儿患者，推荐采用下丘脑错构瘤切除术作为一种治疗选择。鉴于促性腺激素释放激素类似物（GnRHas）有效药物治疗的可得性，必须对手术的作用进行评估。作者报告了两名中枢性性早熟儿童下丘脑错构瘤全切的长期结果，并将其与四名接受药物治疗儿童的情况进行比较。两名接受手术治疗的患者分别在1.75岁（病例1）和3.25岁（病例2）时接受了带蒂下丘脑错构瘤全切术，术后分别接受了9年和8年的随访。术后无并发症，计算机断层扫描证实两名患者的肿瘤均被完全切除。两名患者随后均出现了一些第二性征的消退。一名患者促黄体生成素对促性腺激素释放激素的反应变为青春期前水平，另一名患者的反应则减弱。然而，术后5年生长速度仍保持较高水平（>7厘米/年），骨龄仍超前（> +2标准差），降低了成人身高预测值。其中一名儿童在术后7年开始接受GnRHa治疗。四名儿童仅接受GnRHa激动剂治疗，分别接受了2.3年、6年、9年和9年的随访。这些患者的内分泌异常已完全消退，包括生长速度正常化和骨骼成熟速率降低。使用曲普瑞林治疗未观察到副作用，一名儿童在接受醋酸亮丙瑞林微球治疗时出现无菌性脓肿。GnRHa在抑制青春期和减缓骨龄进展方面已证实的疗效使作者建议，对于由下丘脑错构瘤引起的中枢性性早熟，不应将手术作为初始治疗方法。
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