O Uso de Vismodegibe no Carcinoma Basocelular em pacientes com Síndrome de Gorlin-Goltz: Uma Revisão Sistemática.

Abstract

Basal cell nevus syndrome, basal cell carcinoma syndrome or Gorlin-Goltz syndrome is a hereditary, autosomal dominant disease. It is mainly characterized by numerous and recurrent basal cell carcinomas (BCCs). The pathological basis of this type of neoplasia is the Hedgehog signaling pathway (HH), one of those responsible for controlling cell proliferation - which, when out of control, is known to be the cornerstone of the definition of neoplasia. In 2012, the Food and Drug Administration approved the first antineoplastic drug that inhibits the HH pathway for the treatment of locally advanced or metastatic basal cell carcinomas, and since then, several studies have shown its efficacy and safety, including in the GGS population. Objective: to investigate the impact of targeted therapy with the HH pathway inhibitor antineoplastic Vismodegib (VMGB) on basal cell carcinoma in patients with Gorlin-Goltz syndrome, the benefits of its use in this population and the main adverse effects associated with Vismodegib therapy in these patients. Methodology: this is a qualitative systematic review of scientific literature in which the PubMed, Embase, Scopus and Cochrane Library databases were used, using a pre-specified search strategy. Results: Seventeen articles were included in this review. It was found that the use of VMGB can result in complete or partial remission of BCC lesions in patients with SGG. Furthermore, the therapy has brought other benefits such as reducing the emergence of new basal cell carcinomas. Despite this, the development of adverse effects during medication use has been repeatedly cited as a key factor in poor adherence and long-term continuation of VMGB therapy in this population. Conclusion: The use of Vismodegib has positive impacts on the remission and control of basal cell carcinomas in patients with Gorlin-Goltz Syndrome. However, the diversity and intensity of adverse effects produced by the use of the medication constitute an important factor in resistance to the use of the therapy, as well as in the continuation of treatment with the drug, due to its toxicity.