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 | |