title: Vismodegib induces significant clinical response in locally advanced trichoblastic carcinoma.
abstract: 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.
authors.affiliation: Department of Dermatology, CHRU, Lille, France.
authors.forename: P
authors.initials: P
authors.lastname: Lepesant
authors.affiliation: Department of Pathology, CHRU, Lille, France.
authors.forename: M
authors.initials: M
authors.lastname: Crinquette
authors.affiliation: Department of Dermatology, CHRU, Lille, France.
authors.forename: S
authors.initials: S
authors.lastname: Alkeraye
authors.affiliation: Department of Radiation Oncology, Oscar Lambret Comprehensive Cancer Center, Lille, France.
authors.forename: X
authors.initials: X
authors.lastname: Mirabel
authors.affiliation: Department of Modern Languages, Ecole Centrale de Lille, Lille, France.
authors.forename: V
authors.initials: V
authors.lastname: Dziwniel
authors.affiliation: Department of Pathology, CHRU, Strasbourg, France.
authors.forename: B
authors.initials: B
authors.lastname: Cribier
authors.affiliation: Department of Dermatology, CHRU, Lille, France.
authors.forename: L
authors.initials: L
authors.lastname: Mortier
mesh_terms: D000328:Adult; D000813:Anilides / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002277:Carcinoma / Q000188:drug therapy* / Q000473:pathology; D002280:Basal Cell Carcinoma / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D064847:Multimodal Imaging; D009361:Neoplasm Invasiveness; D049268:Positron-Emission Tomography; D011725:Pyridines / Q000627:therapeutic use*; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000813:Anilides; D000970:Antineoplastic Agents; C538724:HhAntag691; D011725:Pyridines