Intermittent Vismodegib Therapy in Basal Cell Nevus Syndrome
نویسندگان
چکیده
منابع مشابه
The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome.
IMPORTANCE Keratocystic odontogenic tumors (KCOTs) of the jaw affect more than 65% of patients with basal cell nevus syndrome (BCNS). Surgery frequently causes facial disfigurement and is not always curative. Most BCNS-related and some sporadic KCOTs have malignant activation of the Hedgehog signaling pathway. OBSERVATIONS We examined the effect of vismodegib (an oral Hedgehog pathway inhibit...
متن کاملMeningeal calcification in basal cell nevus syndrome.
To cite: Nair PP, Khilari M, Peddawad D, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013200545 DESCRIPTION A 20-year-old woman presented with history of headache for 1 year. The headaches were mild to moderate in intensity, bilateral and non-throbbing, with no symptoms suggestive of raised intracranial pressure. The patient had hyperpigmented skin lesi...
متن کامل[A case of basal cell nevus syndrome].
A case of basal cell nevus syndrome is reported. The patient, a 25-year-old man, had no family history of this condition. The patient showed multiple mandibular cysts, calcification of the falx cerebri, bridging of the sella turcica, ocular hypertelorimsm, a broad nasal root, and pitting of the palms. These findings correlate with reports in the literature and permitted the diagnosis of basal c...
متن کاملBasal cell nevus syndrome. A case report.
An 11-year-old boy with multiple dentigerous cysts in the maxilla and mandible is described. Other findings seen in the face, plantar skin, skeletal system and oral cavity indicated the lesions to be due to the basal cell nevus syndrome. This was further confirmed by the presence of similar abnormalities in his father and brother.
متن کاملTargeted Therapy for Advanced Basal-Cell Carcinoma: Vismodegib and Beyond
Basal-cell carcinoma is a commonly occurring skin malignancy that has the potential to progress into locally invasive or resistant disease, as well as spread distantly. Due to advances in the molecular understanding of the disease over the last two decades, it has been discovered that the Hedgehog pathway plays an important role in the pathogenesis of this disease and can be exploited as a trea...
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ژورنال
عنوان ژورنال: JAMA Dermatology
سال: 2016
ISSN: 2168-6068
DOI: 10.1001/jamadermatol.2015.3210