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