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Basal Cell Nevus Syndrome
Bernadette L. Koch, MD
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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 syndrome
            • Definitions

              • Autosomal dominant disorder with multiple keratocystic odontogenic tumors (KOT), basal cell carcinoma (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

                                          1. John AM et al: Basal cell naevus syndrome: an update on genetics and treatment. Br J Dermatol. 174(1):68-76, 2016
                                          2. 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
                                          3. Athar M et al: Sonic hedgehog signaling in basal cell nevus syndrome. Cancer Res. 74(18):4967-75, 2014
                                          4. Sartip K et al: Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children. Pediatr Radiol. 43(5):620-7, 2013
                                          5. Lam EW et al: The occurrence of keratocystic odontogenic tumours in nevoid basal cell carcinoma syndrome. Dentomaxillofac Radiol. 38(7):475-9, 2009
                                          6. Efron PA et al: Pediatric basal cell carcinoma: case reports and literature review. J Pediatr Surg. 43(12):2277-80, 2008
                                          7. Mitropoulos P et al: Nevoid basal cell carcinoma syndrome (Gorlin syndrome): updated review of minimally invasive treatments. Cutis. 81(1):53-60, 2008
                                          8. Wallin JL et al: Sinonasal carcinoma after irradiation for medulloblastoma in nevoid basal cell carcinoma syndrome. Am J Otolaryngol. 28(5):360-2, 2007
                                          9. Wang XX et al: Familial multiple odontogenic keratocysts. J Dent Child (Chic). 74(2):140-2, 2007
                                          10. 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
                                          11. 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
                                          12. 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
                                          13. Oda D et al: Odontogenic keratocyst: the northwestern USA experience. J Contemp Dent Pract. 1(2): 60-74, 2000
                                          14. 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
                                          15. Kakarantza-Angelopoulou E et al: Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg. 48(6): 593-9; discussion 599-600, 1990
                                          16. Blinder G et al: Widespread osteolytic lesions of the long bones in basal cell nevus syndrome. Skeletal Radiol. 12(3):196-8, 1984
                                          Related Anatomy
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                                          Related Differential Diagnoses
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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 syndrome
                                                    • Definitions

                                                      • Autosomal dominant disorder with multiple keratocystic odontogenic tumors (KOT), basal cell carcinoma (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

                                                                                  1. John AM et al: Basal cell naevus syndrome: an update on genetics and treatment. Br J Dermatol. 174(1):68-76, 2016
                                                                                  2. 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
                                                                                  3. Athar M et al: Sonic hedgehog signaling in basal cell nevus syndrome. Cancer Res. 74(18):4967-75, 2014
                                                                                  4. Sartip K et al: Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children. Pediatr Radiol. 43(5):620-7, 2013
                                                                                  5. Lam EW et al: The occurrence of keratocystic odontogenic tumours in nevoid basal cell carcinoma syndrome. Dentomaxillofac Radiol. 38(7):475-9, 2009
                                                                                  6. Efron PA et al: Pediatric basal cell carcinoma: case reports and literature review. J Pediatr Surg. 43(12):2277-80, 2008
                                                                                  7. Mitropoulos P et al: Nevoid basal cell carcinoma syndrome (Gorlin syndrome): updated review of minimally invasive treatments. Cutis. 81(1):53-60, 2008
                                                                                  8. Wallin JL et al: Sinonasal carcinoma after irradiation for medulloblastoma in nevoid basal cell carcinoma syndrome. Am J Otolaryngol. 28(5):360-2, 2007
                                                                                  9. Wang XX et al: Familial multiple odontogenic keratocysts. J Dent Child (Chic). 74(2):140-2, 2007
                                                                                  10. 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
                                                                                  11. 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
                                                                                  12. 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
                                                                                  13. Oda D et al: Odontogenic keratocyst: the northwestern USA experience. J Contemp Dent Pract. 1(2): 60-74, 2000
                                                                                  14. 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
                                                                                  15. Kakarantza-Angelopoulou E et al: Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg. 48(6): 593-9; discussion 599-600, 1990
                                                                                  16. Blinder G et al: Widespread osteolytic lesions of the long bones in basal cell nevus syndrome. Skeletal Radiol. 12(3):196-8, 1984