非黑色素瘤皮肤癌（NMSC）是全世界最常见的人类肿瘤。具体来说，基底细胞癌（BCC）是白皮肤人群中最常见的恶性肿瘤。由于登记实践不善，BCC 的发生率仍然难以评估；然而，在过去几年中，这一数字一直在增加。大约 85% 的散发性 BCC 携带 Hedgehog 通路基因突变，尤其是 PTCH、SUFU 和 SMO 基因，这会导致 GLI 转录因子异常激活，而这些转录因子在成年个体的细胞中通常是沉默的。晚期 BCC (aBCC) 的治疗，包括转移性 (mBCC) 和局部晚期 BCC (laBCC)，不适合手术切除或放疗，仍然具有挑战性。 Hh信号通路突变的发现为Hh通路抑制剂的开发铺平了道路，例如vismodegib和sonidegib，它们代表了aBCC治疗的突破。然而，这些药物的使用受到不良事件频繁发生或耐药性发展的限制。在这篇综述中，我们全面描述了有关 aBCC 药物管理可用选项的当前知识，并提供了有关新治疗策略的前瞻性更新，这些策略可以在不久的将来丰富 BCC 的治疗手段。

Non-melanoma skin cancers (NMSCs) are the most common human neoplasms world-wide. In detail, basal cell carcinoma (BCC) is the most frequent malignancy in the fair-skinned population. The incidence of BCC remains difficult to assess due to the poor registration practice; however, it has been increasing in the last few years. Approximately, 85% of sporadic BCCs carry mutations in Hedgehog pathway genes, especially in PTCH, SUFU and SMO genes, which lead to the aberrant activation of GLI transcriptional factors, typically silent in cells of adult individuals. The management of advanced BCC (aBCC), both metastatic (mBCC) and locally advanced BCC (laBCC), not candidates for surgical excision or radiotherapy, remains challenging. The discovery of mutations in the Hh signaling pathway has paved the way for the development of Hh pathway inhibiting agents, such as vismodegib and sonidegib, which have represented a breakthrough in the aBCC management. However, the use of these agents is limited by the frequent occurrence of adverse events or the development of drug resistance. In this review, we thoroughly describe the current knowledge regarding the available options for the pharmacological management of aBCCs and provide a forward-looking update on novel therapeutic strategies that could enrich the therapeutic armamentarium of BCC in the near future.