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首页> 外文期刊>Anticancer Research: International Journal of Cancer Research and Treatment >Treatment of Multiple Unresectable Basal Cell Carcinomas from Gorlin-Goltz Syndrome: A Case Report
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Treatment of Multiple Unresectable Basal Cell Carcinomas from Gorlin-Goltz Syndrome: A Case Report

机译：Gorlin-Goltz综合征引起的多种不可切除的基底细胞癌的治疗：一例报告

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摘要

Background: Nevoid basal cell carcinoma syndrome (NBCCS), which is also known by other names, including Gorlin-Goltz syndrome and multiple basal-cell carcinoma (BCC) syndrome, is a rare multi-systemic disease inherited in a dominant autosomal manner with complete penetrance and variable expressivity. The main clinical manifestations include multiple BCCs, odontogenic keratocysts of the jaw, hyperkeratosis of the palms and soles, skeletal abnormalities, intracranial calcifications and facial deformities. Patients and Methods: A 31-year-old male diagnosed with Gorlin-Goltz syndrome with multiple unresectable facial BCCs was treated with the Hedgehog inhibitor vismodegib. Results: After one month of therapy on vismodegib, there were significant reductions in the size of multiple BCCs on the patient's face. The patient remains on this therapy. Conclusion: Hedgehog pathway inhibition is an effective strategy to treat unresectable BCCs from Gorlin-Goltz syndrome. Although vismodegib shows some promising clinical results in the early phase of its use, there are concerns of possible resistance developing within months. Duration of therapy, role of maintenance treatment and drug modification to reduce resistance need to be explored in future case studies.
机译：背景：空洞性基底细胞癌综合征（NBCCS），也被称为其他名称，包括Gorlin-Goltz综合征和多发性基底细胞癌（BCC）综合征，是一种罕见的多系统疾病，以显性常染色体方式遗传并完全外表和可变表达。主要临床表现包括多个BCC，颌骨牙源性角化囊肿，手掌和脚底过度角化，骨骼异常，颅内钙化和面部畸形。患者和方法：使用Hedgehog抑制剂vismodegib治疗一名诊断为Gorlin-Goltz综合征且患有多个不可切除的面部BCC的31岁男性。结果：在使用vismodegib治疗一个月后，患者面部多个BCC的大小明显减少。病人继续接受这种疗法。结论：刺猬通路抑制是一种治疗无法切除的戈林-戈尔茨综合征BCC的有效策略。尽管vismodegib在其使用的早期阶段显示出一些有希望的临床结果，但仍存在可能在数月内产生耐药性的担忧。治疗的持续时间，维持治疗的作用以及减少耐药性的药物修饰等都需要在未来的案例研究中进行探讨。

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