nevoid basal cell carcinoma syndrome


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Related to nevoid basal cell carcinoma syndrome: Gorlin Syndrome, ameloblastoma, Basal Cell Nevus Syndrome, Calcifying odontogenic cyst

nevoid basal cell carcinoma syndrome

[nē′void]
an inherited form of premalignant skin lesion. It is an autosomal-dominant trait, but the cause is unknown. It is associated with other abnormalities of the skin or bone, the nervous system, the eyes, and the reproductive system. It affects persons under the age of 20 and is accompanied by palmar pits, mandibular cysts, bifid ribs, and other birth defects.
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Nevoid basal cell carcinoma syndrome

basal cell nevus syndrome

An uncommon autosomal dominant condition (OMIM:109400) characterised by childhood onset of multiple nevoid basal cell carcinomas accompanied by “pits” in the hands and feet (in the form of 2–3-mm-diameter “dells”, occasionally filled with carcinoma), milia, sebaceous cysts, lipomas, fibromas, lympho-mesenteric cysts, CNS disease (mental retardation), EEG abnormalities, calcification of dura, medulloblastoma, schizophrenia, ovarian cysts or fibroma, male hypogonadism, female escutcheon, scanty facial hair, canthal dystopia, hypertelorism, coloboma, congenital blindness, hypertelorism, lateral displacement of medial canthi, frontoparietal bossing, mandibular prognathism, accentuated supraorbital ridges, jaw cysts and a broad nasal root, spina bifida occulta, fused, absent or cervical ribs, kyphosis, scoliosis, cervical and thoracic vertebral fusion, bridging of sella turcica, shortened 4th–5th metacarpals, epithelial jaw cysts.
References in periodicals archive ?
Treatment of basal cell carcinomas in patients with nevoid basal cell carcinoma syndrome.
Mutations in the human homologue of the Drosophila patched gene in Caucasian and African-American nevoid basal cell carcinoma syndrome patients.
2-4) However, no one has reported an FR of the tongue in a patient with nevoid basal cell carcinoma syndrome (NBCCS).
Treatment of diffuse basal cell carcinomas and basaloid follicular hamartomas in nevoid basal cell carcinoma syndrome by wide-area 5-aminolevulinic acid photodynamic therapy.
We describe the imaging findings in a 13-year-old boy with nevoid basal cell carcinoma syndrome (NBCCS).