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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Basal cell nevus syndrome (BCNS)
Synonyms
- Gorlin syndrome, Gorlin-Goltz syndrome, nevoid basal cell syndrome, nevoid basal cell carcinoma (BCCa) syndrome
Definitions
- Autosomal dominant disorder with multiple odontogenic keratocysts (OKC), BCCa, medulloblastoma, intracranial dural calcifications, bifid ribs
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Lin MJ et al: Basal cell nevus syndrome: from DNA to therapeutics. Clin Dermatol. 38(4):467-76, 2020
- Borghesi A et al: Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviour. Insights Imaging. 9(5):883-97, 2018
- John AM et al: Basal cell naevus syndrome: an update on genetics and treatment. Br J Dermatol. 174(1):68-76, 2016
- Ally MS et al: The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome. JAMA Dermatol. 150(5):542-5, 2014
- Athar M et al: Sonic hedgehog signaling in basal cell nevus syndrome. Cancer Res. 74(18):4967-75, 2014
- Sartip K et al: Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children. Pediatr Radiol. 43(5):620-7, 2013
- Lam EW et al: The occurrence of keratocystic odontogenic tumours in nevoid basal cell carcinoma syndrome. Dentomaxillofac Radiol. 38(7):475-9, 2009
- Efron PA et al: Pediatric basal cell carcinoma: case reports and literature review. J Pediatr Surg. 43(12):2277-80, 2008
- Mitropoulos P et al: Nevoid basal cell carcinoma syndrome (Gorlin syndrome): updated review of minimally invasive treatments. Cutis. 81(1):53-60, 2008
- Wallin JL et al: Sinonasal carcinoma after irradiation for medulloblastoma in nevoid basal cell carcinoma syndrome. Am J Otolaryngol. 28(5):360-2, 2007
- Wang XX et al: Familial multiple odontogenic keratocysts. J Dent Child (Chic). 74(2):140-2, 2007
- Campbell RM et al: Meningiomas after medulloblastoma irradiation treatment in a patient with basal cell nevus syndrome. J Am Acad Dermatol. 53(5 Suppl 1):S256-9, 2005
- Kimonis VE et al: Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med. 6(6):495-502, 2004
- Palacios E et al: Odontogenic keratocysts in nevoid basal cell carcinoma (Gorlin's) syndrome: CT and MRI evaluation. Ear Nose Throat J. 83(1):40-2, 2004
- Oda D et al: Odontogenic keratocyst: the northwestern USA experience. J Contemp Dent Pract. 1(2): 60-74, 2000
- Jose Tincani A et al: Nevoid basal-cell syndrome: literature review and case report in a family. Rev Paul Med. 113(3): 917-21, 1995
- Kakarantza-Angelopoulou E et al: Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg. 48(6): 593-9; discussion 599-600, 1990
- Blinder G et al: Widespread osteolytic lesions of the long bones in basal cell nevus syndrome. Skeletal Radiol. 12(3):196-8, 1984
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Basal cell nevus syndrome (BCNS)
Synonyms
- Gorlin syndrome, Gorlin-Goltz syndrome, nevoid basal cell syndrome, nevoid basal cell carcinoma (BCCa) syndrome
Definitions
- Autosomal dominant disorder with multiple odontogenic keratocysts (OKC), BCCa, medulloblastoma, intracranial dural calcifications, bifid ribs
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Lin MJ et al: Basal cell nevus syndrome: from DNA to therapeutics. Clin Dermatol. 38(4):467-76, 2020
- Borghesi A et al: Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviour. Insights Imaging. 9(5):883-97, 2018
- John AM et al: Basal cell naevus syndrome: an update on genetics and treatment. Br J Dermatol. 174(1):68-76, 2016
- Ally MS et al: The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome. JAMA Dermatol. 150(5):542-5, 2014
- Athar M et al: Sonic hedgehog signaling in basal cell nevus syndrome. Cancer Res. 74(18):4967-75, 2014
- Sartip K et al: Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children. Pediatr Radiol. 43(5):620-7, 2013
- Lam EW et al: The occurrence of keratocystic odontogenic tumours in nevoid basal cell carcinoma syndrome. Dentomaxillofac Radiol. 38(7):475-9, 2009
- Efron PA et al: Pediatric basal cell carcinoma: case reports and literature review. J Pediatr Surg. 43(12):2277-80, 2008
- Mitropoulos P et al: Nevoid basal cell carcinoma syndrome (Gorlin syndrome): updated review of minimally invasive treatments. Cutis. 81(1):53-60, 2008
- Wallin JL et al: Sinonasal carcinoma after irradiation for medulloblastoma in nevoid basal cell carcinoma syndrome. Am J Otolaryngol. 28(5):360-2, 2007
- Wang XX et al: Familial multiple odontogenic keratocysts. J Dent Child (Chic). 74(2):140-2, 2007
- Campbell RM et al: Meningiomas after medulloblastoma irradiation treatment in a patient with basal cell nevus syndrome. J Am Acad Dermatol. 53(5 Suppl 1):S256-9, 2005
- Kimonis VE et al: Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med. 6(6):495-502, 2004
- Palacios E et al: Odontogenic keratocysts in nevoid basal cell carcinoma (Gorlin's) syndrome: CT and MRI evaluation. Ear Nose Throat J. 83(1):40-2, 2004
- Oda D et al: Odontogenic keratocyst: the northwestern USA experience. J Contemp Dent Pract. 1(2): 60-74, 2000
- Jose Tincani A et al: Nevoid basal-cell syndrome: literature review and case report in a family. Rev Paul Med. 113(3): 917-21, 1995
- Kakarantza-Angelopoulou E et al: Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg. 48(6): 593-9; discussion 599-600, 1990
- Blinder G et al: Widespread osteolytic lesions of the long bones in basal cell nevus syndrome. Skeletal Radiol. 12(3):196-8, 1984
