Table 2 Disorders reported in patients with basal cell nevus syndrome.
From: Nevoid basal cell carcinoma syndrome (Gorlin syndrome)
| Clinical manifestations | |
|---|---|
| Skin | Central nervous system |
| Cutaneous dyskeratosis: | Ectopic calcification: |
| ∘ erythematous-squamous spots degenerating in NBCC | ∘ falx cerebri |
| ∘ tentorium cerebelli | |
| ∘ nodular or patch lesions | ∘ spotted meningeal calcification |
| ∘ palmo-plantar pits | ∘ complete or partial bony bridging of the sella turcica |
| Multiple basal cell carcinomas | |
| Benign dermal cysts | Meningioma |
| ∘ Multiple nevi | Medulloblastoma |
| Multiform glioblastoma | |
| Moderate mental retardation | |
| Grand mal | |
| Congenital hydrocephalus | |
| Huntington's chorea | |
| Stomatologic system | Ocular system |
| Odontogenic keratocysts | Cataract, coloboma, microphthalmia |
| Dental ectopy, heterotopy | Chalazions |
| Impacted teeth | Internal strabismus |
| Dental agenesis | Rotatory nistagmus |
| Malocclusion | Exophthalmus |
| Maxillary fibrosarcoma | Hypertelorism |
| Ameloblastoma | Congenital blindness |
| Odontogenic myxoma | |
| Spindle cell carcinoma | |
| Cleft palate and lip | |
| Mandibular prognathism | |
| High-arched palate | |
| Squamous cell carcinoma | |
| Skeletal open bite | |
| Idiopathic pseudocyst | |
| Hyperplasia of the mandibular coronoid processes | |
| Musculo-skeletal system | Cardio-vascular system |
| Congenital skeletal anomalies: | Cardiac fibroma (interventricular septum) |
| ∘ bifid, fused, splayed, or missing ribs | Absent internal carotid artery |
| ∘ bifid wedges fused vertebra | |
| ∘ scoliosis | |
| ∘ frontal, temporal and parietal bossing | |
| ∘ polydactyly | |
| ∘ sindactyly | |
| ∘ short fourth metarcapal | |
| ∘ sprengel shoulder | |
| Polyostotic bone cysts | |
| Genito-urinary system | Auditory system |
| Males: | Middle ear anomalies |
| ∘ hypogonadism | ∘ otosclerosis |
| ∘ cryptorchidism | ∘ conductive hearing loss |
| Females: | Posteriorly angulated ears |
| ∘ ovarian calcifications | |
| ∘ ovarian cysts | |
| ∘ ovarian fibroma | |
| hypogonadism | |
| Respiratory system | Biochemical findings |
| Bronchogenic cysts | High levels of CAMP |
| Hyaline membrane disease | High levels of AP |
| Gastro-enteric system | |
| Linfomesenteryc cysts | |
| Gastric polyps | |
