nevoid basal cell carcinoma syndrome

(redirected from Gorlin-goltz 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.
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References in periodicals archive ?
Among all the 30 cases, one case was Gorlin-Goltz syndrome.
It has been proposed that difficult operative access, leaving affected teeth in place after treatment, cystic satellite formation, and the association with Gorlin-Goltz syndrome are correlated with a higher recurrence of OKCs [6, 7].
Gorlin-Goltz syndrome is a rare genetic disease with autosomal dominant inheritance that leads to multi-organ disorder.
Multiple lesions are associated with Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome.
Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant syndrome characterized by multiple clinical features involving the skin, nervous system, eyes, endocrine system, and bones [1].
Multiple lesions are associated with the nevoid basal cell syndrome (Gorlin-Goltz syndrome).3
Furthermore, the KOTs that affect patients with Gorlin-Goltz syndrome commonly behave more aggressively, (7) most likely due to the higher rate of epithelial proliferation that occurs in these patients.
Nevoid basal cell carcinoma syndrome (NBCCS), also known as basal cell nevus syndrome, multiple basal cell carcinoma syndrome, Gorlin syndrome, and Gorlin-Goltz syndrome, is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones.
Gorlin-Goltz syndrome (also known as nevoid basal cell carcinoma syndrome) was first reported in 1894, but described by Gorlin and Goltz in 1960 as a distinct entity consisting of ectodermal and mesodermal abnormalities.(1) It is an hereditary autosomal dominant disease with a prevalence estimated in various studies to be between 1/57 000 and 1/256 000, and a male: female ratio of 1:1.(2) We describe in brief the important radiological features of an accidentally detected case of Gorlin syndrome in the form of a pictorial interlude.