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. 2020 Sep 9;24(2):315–321. doi: 10.4103/jomfp.JOMFP_358_19

Table 5.

Disorders reported in nevoid basal carcinoma syndrome[3]

Skin Central nervous system Stomatologic system Ocular system
Cutaneous dyskeratosis Ectopic calcification Odontogenic keratocysts Cataract, coloboma,
 Erythematous-squamous spots  Falx cerebri Dental ectopy, heterotopy microphthalmia
 degenerating in NBCC  Tentorium cerebelli Impacted teeth internal strabismus Chalazions
 Nodular or patch lesions  Spotted meningeal Dental agenesis malocclusion Rotatory nistagmus
 Palmo-plantar pits  calcification Maxillary fibrosarcoma Exophthalmus
 Multiple basal cell carcinomas  Complete or partial bony Ameloblastoma Hypertelorism
 Benign dermal cysts  bridging of the sella turcica Odontogenic myxoma Congenital blindness
 Multiple nevi  Meningioma Spindle cell carcinoma
 Multiform glioblastoma Cleft palate and lip
 Moderate mental retardation Mandibular prognathism
 MED High-arched palate
 Grand mal Squamous cell carcinoma
 Congenital hydrocephalus Skeletal open bite
 Huntington’s chorea Idiopathic pseudocyst
Hyperplasia of the mandibular coronoid processes

Musculo-skeletal system Cardio-vascular system Genito-urinary system Auditory system

Congenital skeletal anomalies Cardiac fibroma (interventricular septum) Males: Middle ear anomalies Middle ear anomalies Hearing angulated ears
 Bifid, fused, splayed, or missing ribs  hypogonadism  Otosclerosis
 bifid wedges fused vertebra Absent internal carotid artery  Cryptorchidism  Conductive Hearing
 Scoliosis  Females: Ovarian calcifications  loss
 Frontal, temporal and parietal bossing  Ovarian cysts  Posteriorly
 Polydactyly  Ovarian fibroma
 Sindactyly  Hypogonadism
 Short fourth metarcapal
 Sprengel shoulder
 Polyostotic bone cysts

Respiratory system Gastroenteric system Biochemical findings

Bronchogenic cysts Linfomesentery cysts High levels of CAMP
Hyaline membrane disease Gastric polyps High levels of AP

MED: Medulloblastoma, NBCC: Nevoid basal cell carcinoma