RESUMO
BACKGROUND: Although smoothened inhibitors (SMOi) have demonstrated efficacy in the management of basal cell carcinoma (BCC), no guidelines are available on how to utilize SMOi in the treatment of Gorlin syndrome (GS). OBJECTIVE: Assess the clinical response to SMOi in GS, provide practical guidance for clinicians, and identify areas for future research. METHODS: Alongside a comprehensive search of Medline and Google Scholar for systemic therapies related to Gorlin Syndrome (GS), expert opinion was specifically sought to provide detailed guidance on the use of SMOi in the management of GS. RESULTS: Intermittent dosing of SMOi and daily dosing had similar efficacy. Intermittent dosing resulted in better compliance with therapy. However, lack of tolerability limited long-term use of SMOi. LIMITATIONS: There are few studies solely on the use of SMOi in GS. Included studies often had a subgroup of GS patients. CONCLUSION: While the adverse events of SMOi, such as vismodegib and sonidegib, may result in their discontinuation during treatment of GS, intermittent dosing may improve compliance. Further research is needed to identify dosing strategies that minimize tumor burden while mitigating side effects to achieve maximum compliance.
RESUMO
Introduction: Cutaneous metastasis of urothelial carcinoma is a rare occurrence, accounting for a small percentage of skin metastases in cancer patients. This case presentation highlights the importance of considering cutaneous metastasis in patients with a history of urologic malignancy presenting with new dermal nodules. Case presentation: A 79-year-old male with a history of papillary urothelial carcinoma of the bladder and metastasis to the rectum presented with a painful and pruritic rash in the right inguinal region. Physical examination revealed firm papulonodules forming confluent, hyperpigmented to violaceous plaques. A punch biopsy confirmed the diagnosis of cutaneous metastasis of urothelial carcinoma based on histopathological and immunohistochemical findings. Conclusion: While cutaneous metastasis is uncommon in urothelial carcinoma, early recognition and diagnosis are crucial in guiding patient management and setting realistic expectations regarding prognosis. Timely identification of cutaneous lesions can help facilitate appropriate treatment decisions and discussions of goals of care.