A. Wrong, this is one of the mutations that may happen in Noonan syndrome which is characterized by multiple central giant cell granulomas not odontogenic keratocyst.
B. Right, the patient was suffering from multiple odontogenic keratocysts, syndactyly, increased circumference of the head and frontal bossing; which are consistent with the diagnosis of Gorlin syndrome.
Gorlin syndrome requires the presence of two major or one major and two minor clinical criteria. Major criteria include: multiple (>2) basal cell carcinomas (BCC) or 1 BCC by ≤20 years of age, odontogenic keratocysts of the jaw proven by histology, palmar or plantar pitting, bilamellar calcification of the falx cerebri, bifid/fused/splayed ribs, first-degree relative with Gorlin syndrome. Minor criteria include: medulloblastoma, increased circumference of the head, congenital malformations (frontal bossing, coarse facies, cleft lip/palate, moderate or severe hypertelorism), other skeletal abnormalities (Sprengel deformity, marked pectus deformity, marked syndactyly of the digits),
C. Wrong, this is one of the mutations that may happen in LEOPARD syndrome which is characterized by distinct facial features with no specific mucosal or intrabony lesions in the oral cavity.
B. Right, the patient was suffering from multiple odontogenic keratocysts, syndactyly, increased circumference of the head and frontal bossing; which are consistent with the diagnosis of Gorlin syndrome.
Gorlin syndrome requires the presence of two major or one major and two minor clinical criteria. Major criteria include: multiple (>2) basal cell carcinomas (BCC) or 1 BCC by ≤20 years of age, odontogenic keratocysts of the jaw proven by histology, palmar or plantar pitting, bilamellar calcification of the falx cerebri, bifid/fused/splayed ribs, first-degree relative with Gorlin syndrome. Minor criteria include: medulloblastoma, increased circumference of the head, congenital malformations (frontal bossing, coarse facies, cleft lip/palate, moderate or severe hypertelorism), other skeletal abnormalities (Sprengel deformity, marked pectus deformity, marked syndactyly of the digits),
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