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 ?
Fetal rhabdomyoma and nevoid basal cell carcinoma syndrome.
Multiple cutaneous cancers in children: the nevoid basal cell carcinoma syndrome.
Correlating imaging and histopathology of an odontogenic keratocyst in the nevoid basal cell carcinoma syndrome.