Vismodegib induces significant clinical response in locally advanced trichoblastic carcinoma. (27th August 2015)

• Record Type: Journal Article
• Authors: Lepesant, P.; Crinquette, M.; Alkeraye, S.; Mirabel, X.; Dziwniel, V.; Cribier, B.; Mortier, L.
• Is Part Of: British journal of dermatology. Volume 173:Number 4(2015:Oct.)
• Page Start: 1059
• Page End: 1062
• Publication Date: 2015-08-27
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Periodicals; 616.5
• DOI: 10.1111/bjd.13919
• Languages: English
• ISSNs: 0007-0963

Abstract

Abstract : What's already known about this topic? Vismodegib is a new therapeutic strategy for patients with advanced or metastatic basal cell carcinoma. Up until now, there has been no effective treatment for metastatic or unresectable trichoblastic carcinoma. What does this study add? This observation is the first case of trichoblastic carcinoma with significant response to vismodegib. This observation tends to suggest that the patched sonic hedgehog pathway may have a relevant role in the pathogenesis of trichoblastic carcinoma. Summary: Patients with advanced basal cell carcinoma due to local extension or metastatic disease were previously at a therapeutic impasse. Targeted inhibition of the sonic hedgehog pathway by vismodegib represents a new therapeutic strategy. Adnexal carcinomas are rare malignant skin tumours derived from epithelial annexes. Conventional treatment of adnexal tumours is based on surgical excision. Although the radiosensitivity of adnexal carcinomas has not been established, radiotherapy could be offered alone or in combination in locally advanced or inoperable disease. Chemotherapy represents a therapeutic option in the treatment of metastatic adnexal tumours. Currently there is no effective treatment for these tumours when they become metastatic or unresectable, and treatment is palliative. Sunitinib represents a new therapeutic strategy, with efficiency described in the literature for a small number of patients. However, its efficacy is partial, and its tolerance is not always good. We report a patient with trichoblastic carcinoma, initially diagnosed as basal cell carcinoma, treated effectively with vismodegib. The remarkable response we have observed in this patient suggests an encouraging therapeutic role of vismodegib in trichoblastic carcinoma that should be evaluated in a carefully designed trial.