No abstract available.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell

No abstract available.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell

Introduction: Basal cell nevus syndrome (BCNS) (Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome) is a rare inherited multisystem and tumor-predisposing disorder caused by the patched tumor suppressor gene mutations and suppressor of fused gene. Its diagnosis follows a set of criteria based on specific cutaneous features and radiologic findings. Although an autosomal dominant disorder with a high degree of penetrance, BCNS has variable expression making its diagnosis difficult. The limited epidemiologic data among Asians especially in the Philippines hamper early detection or cause frequent misdiagnosis of the condition. Case report: A 56-year-old Filipino female with Fitzpatrick skin type V presented with early onset multiple basal cell carcinomas and bilateral palmoplantar pits. Radiologic investigation reveals odontogenic keratocyst, calcification of the falx cerebri, bridging of the sella turcica, bifid/splayed ribs and vertebral anomalies. The patient exhibits coarse facial features and bilateral cataracts. Cranial computed tomography scan shows cerebrocerebellar atrophy with ventricular dilatation. Management included wide excision of the nodular basal cell carcinomas (BCC), application of 5-flourouracil cream on the superficial BCC and electrodessication and curettage of the smaller lesions. Oral acitretin was also prescribed. Conclusion This is a case that highlights the approach to diagnosis, clinical features and management of BCNS in a Filipino patient. Since various phenotypic presentations may exist among dark-skinned individuals, early diagnosis poses a challenge among physicians. Epidemiologic and prevalence studies among Filipinos may be done to aid in the diagnosis and early management of this rare genodermatosis.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell

Basal cell nevus syndrome (BCNS) is principally characterized by cutaneous basal cell carcinomas, multiple odontogenic keratocysts and skeletal abnormalities. Our patient represented several characteristics of BCNS, such as, multiple odontogenic keratocysts, facial nevus, calcification of falx cerebri, parietal bossing and mental retardation. The cyst on posterior mandible showed recurrent and newly developing tendency.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Humans ; Intellectual Disability ; Mandible ; Nevus ; Odontogenic Cysts* ; Rabeprazole

Basal cell nevus syndrome(BCNS) is a rare autosomal dominant disorder characterized by variable developmental anomalies and predisposition to cancers. The main manifestations include multiple basal cell carcinomas of skins, odontogenic keratocysts, facial dysmorphism, skeletal abnormalities including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We experienced a case of 12-year-old girl with the clinical features of basal cell nevus syndrome.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell ; Child ; Humans ; Nevus ; Odontogenic Cysts ; Ribs ; Scoliosis ; Skin

Nevoid basal cell carcinoma syndrome is inherited as an autcsonal dominant trait with a high degree of penetrane and variable expressivity. The mair features are basal cell carcinoma. jaw cysts, skeletal anornalies, ectopic calcifications and palmoplantar pits. We report varous clinical ma.nifestations in brother and sister, wnich are jaw cyst,, bifid rib, shortened fourth metacarpals, ectopie calcification, palrnoplanar pits and basal cell carcinoma, Microscopically, histologic patterns of basal cell cacinomas are superficial, nodulocystic, keratotic, pigmented, adenoid and follicullar nevoid. Lange numbers of basal cell carcinomas were electrodessicated and curetted under general,nesthesia.
Adenoids ; Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Humans ; Jaw Cysts ; Metacarpal Bones ; Ribs ; Siblings

The nevoid basal cell carcinoma syndrome, or Gorlin-Goltz syndrome, is an autosomal dominant multiple system disorder with high penetrance and variable expressions, although it can also arise spontaneously. The diagnostic criteria for nevoid basal cell carcinoma syndrome include multiple basal cell carcinomas, palmoplantar pits, multiple odontogenic keratocysts, skeletal anomalies, positive family history, ectopic calcification and neurological anomalies. We report a brother and sister who were both diagnosed with nevoid basal cell carcinoma syndrome.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell ; Humans ; Odontogenic Cysts ; Penetrance ; Siblings

Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the clinical features of basal cell nevus syndrome, in which a PTCH gene mutation was confirmed by DNA testing.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Child ; DNA ; Humans ; Jaw ; Male ; Odontogenic Cysts ; Ribs ; Scoliosis

Basal cell nevus syndrome (BCNS) is a hereditary condition tansmitted as an autosomal dominant trait that exhibits high penetrance and variable expressivity. There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. The data upon which this review is based has been retrieved from published case reports in Korean dental and medical literatures between the years 1981 to 2002. This survey included unpublished 3 patients by reviewing patients files and charts from the Dental Hospital, Chosun University during same periods. We found 33 subjects who met the diagnostic criteria for BCNS reported by Evans et al. Relative frequencies of associated complications are presented and compared with those of the recent English survey. The frequencies of most manifestations are similar. Odontogenic keratocyst (OKC) and palmar and/or plantar pits, and hypertelorism were the most frequently observed anomalies. OKCs are often the first signs of BCNS and can be detected in patients younger than 9 years of age. However, the relatively low frequency of basal cell carcinomas and ectopic calcification were two major difference. This study more clearly defines the possible complications of the syndrome and gives earlier guidelines for counselling and screening affected and at risk persons.]]>
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell ; Humans ; Hypertelorism ; Mass Screening ; Odontogenic Cysts ; Penetrance

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome, shows autosomal dominant pattern of inheritance with variable expressivity. The most important features are the occurrence of basal cell carcinoma and odontogenic keratosis. Recently, we experienced a case of nevoid basal cell carcinoma syndrome with multiple odontogenic keratocysts, basal cell carcinoma on cheek, cerebral calcification and palmar pits. We report this case with literature review.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Cheek ; Keratosis ; Odontogenic Cysts ; Wills