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B-K mole (nevus) syndrome

MedGen UID:
3937
•Concept ID:
C0013403
•
Neoplastic Process
Synonyms: B K Mole Syndrome; B-K Mole Syndrome; Dysplastic Nevus Syndrome; Nevus Syndrome, Dysplastic; Syndrome, B-K Mole; Syndrome, Dysplastic Nevus
SNOMED CT: Atypical mole syndrome (254819008); B-K mole (nevus) syndrome (254819008)
 
OMIM®: 155600

Definition

An autosomal dominant disorder defined by the presence of multiple dysplastic nevi and a history of melanoma in two family members. Patients are at an increased risk for the development of melanoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVB-K mole (nevus) syndrome

Recent clinical studies

Etiology

Dysplastic Nevi: Morphology and Molecular and the Controversies In-between.
Wiedemeyer K, Hartschuh W, Brenn T
Surg Pathol Clin 2021 Jun;14(2):341-357. Epub 2021 Apr 28 doi: 10.1016/j.path.2021.01.007. PMID: 34023110
Dysplastic nevi and melanoma.
Goldstein AM, Tucker MA
Cancer Epidemiol Biomarkers Prev 2013 Apr;22(4):528-32. doi: 10.1158/1055-9965.EPI-12-1346. PMID: 23549396Free PMC Article
Dysplastic nevi.
Farber MJ, Heilman ER, Friedman RJ
Dermatol Clin 2012 Jul;30(3):389-404. Epub 2012 Jun 8 doi: 10.1016/j.det.2012.04.004. PMID: 22800547
Childhood melanoma.
Jen M, Murphy M, Grant-Kels JM
Clin Dermatol 2009 Nov-Dec;27(6):529-36. doi: 10.1016/j.clindermatol.2008.09.011. PMID: 19880040
The "dysplastic" nevus.
Friedman RJ, Farber MJ, Warycha MA, Papathasis N, Miller MK, Heilman ER
Clin Dermatol 2009 Jan-Feb;27(1):103-15. doi: 10.1016/j.clindermatol.2008.09.008. PMID: 19095156

Diagnosis

Dysplastic nevus part I: Historical perspective, classification, and epidemiology.
Drozdowski R, Spaccarelli N, Peters MS, Grant-Kels JM
J Am Acad Dermatol 2023 Jan;88(1):1-10. Epub 2022 Aug 28 doi: 10.1016/j.jaad.2022.04.068. PMID: 36038073
Dysplastic Nevi: Morphology and Molecular and the Controversies In-between.
Wiedemeyer K, Hartschuh W, Brenn T
Surg Pathol Clin 2021 Jun;14(2):341-357. Epub 2021 Apr 28 doi: 10.1016/j.path.2021.01.007. PMID: 34023110
Atypical moles: diagnosis and management.
Perkins A, Duffy RL
Am Fam Physician 2015 Jun 1;91(11):762-7. PMID: 26034853
Dysplastic nevi.
Farber MJ, Heilman ER, Friedman RJ
Dermatol Clin 2012 Jul;30(3):389-404. Epub 2012 Jun 8 doi: 10.1016/j.det.2012.04.004. PMID: 22800547
Hereditary pancreatic cancer.
Grover S, Syngal S
Gastroenterology 2010 Oct;139(4):1076-80, 1080.e1-2. Epub 2010 Aug 19 doi: 10.1053/j.gastro.2010.08.012. PMID: 20727885Free PMC Article

Therapy

Beta-carotene in dermatology: Does it help?
Bayerl Ch
Acta Dermatovenerol Alp Pannonica Adriat 2008 Dec;17(4):160-2, 164-6. PMID: 19104740
Clinical practice. Dysplastic nevi.
Naeyaert JM, Brochez L
N Engl J Med 2003 Dec 4;349(23):2233-40. doi: 10.1056/NEJMcp023017. PMID: 14657431
Primary cutaneous malignant melanoma and its precursor lesions: diagnostic and therapeutic overview.
Kanzler MH, Mraz-Gernhard S
J Am Acad Dermatol 2001 Aug;45(2):260-76. doi: 10.1067/mjd.2001.116239. PMID: 11464189
The role of atypical mole syndrome and cutaneous naevi in the development of melanoma.
Easton D
Cancer Surv 1996;26:237-49. PMID: 8783577
The prevention of cutaneous malignant melanoma: high-risk groups, chemoprevention, education, and screening.
Greene MH
Cancer Treat Res 1993;65:103-40. doi: 10.1007/978-1-4615-3080-0_5. PMID: 8104019

Prognosis

Pregnancy as a driver for melanoma.
Richtig G, Byrom L, Kupsa R, Schaider H, Hofmann-Wellenhof R, Wolf IH, Soyer HP, Richtig E
Br J Dermatol 2017 Sep;177(3):854-857. Epub 2017 Jul 12 doi: 10.1111/bjd.15124. PMID: 27718538
Practical application of new technologies for melanoma diagnosis: Part II. Molecular approaches.
March J, Hand M, Truong A, Grossman D
J Am Acad Dermatol 2015 Jun;72(6):943-58; quiz 959-60. doi: 10.1016/j.jaad.2015.02.1140. PMID: 25980999
Childhood melanoma.
Jen M, Murphy M, Grant-Kels JM
Clin Dermatol 2009 Nov-Dec;27(6):529-36. doi: 10.1016/j.clindermatol.2008.09.011. PMID: 19880040
Dysplastic pointillist nevus.
Moreno-Ramirez D, Ferrandiz L, Rios-Martin JJ, Camacho FM
Arch Dermatol 2005 Jun;141(6):763-4. doi: 10.1001/archderm.141.6.763. PMID: 15967924
Familial cutaneous melanoma.
Lucchina LC, Barnhill RL, Duke DM, Sober AJ
Melanoma Res 1995 Dec;5(6):413-8. doi: 10.1097/00008390-199512000-00004. PMID: 8589615

Clinical prediction guides

Noninvasive diagnosis in dermatology.
Welzel J, Schuh S
J Dtsch Dermatol Ges 2017 Oct;15(10):999-1016. doi: 10.1111/ddg.13347. PMID: 28976087
Practical application of new technologies for melanoma diagnosis: Part II. Molecular approaches.
March J, Hand M, Truong A, Grossman D
J Am Acad Dermatol 2015 Jun;72(6):943-58; quiz 959-60. doi: 10.1016/j.jaad.2015.02.1140. PMID: 25980999
Dermatopathology updates on melanocytic lesions.
Cheung WL, Smoller BR
Dermatol Clin 2012 Oct;30(4):617-22, vi. Epub 2012 Aug 3 doi: 10.1016/j.det.2012.06.014. PMID: 23021049
Dysplastic pointillist nevus.
Moreno-Ramirez D, Ferrandiz L, Rios-Martin JJ, Camacho FM
Arch Dermatol 2005 Jun;141(6):763-4. doi: 10.1001/archderm.141.6.763. PMID: 15967924
The dysplastic naevus.
Mooi WJ
J Clin Pathol 1997 Sep;50(9):711-5. doi: 10.1136/jcp.50.9.711. PMID: 9389970Free PMC Article

Recent systematic reviews

Prognosis of CDKN2A germline mutation in patients with familial melanoma: a systematic review and meta-analysis.
Taibo A, Paradela S, Suanzes-Hernández J, Balboa-Barreiro V, Amado-Bouza J, Fonseca E
Melanoma Res 2024 Feb 1;34(1):9-15. Epub 2023 Nov 2 doi: 10.1097/CMR.0000000000000920. PMID: 37924530
Surgical re-excision vs. observation for histologically dysplastic naevi: a systematic review of associated clinical outcomes.
Vuong KT, Walker J, Powell HB, Thomas NE, Jonas DE, Adamson AS
Br J Dermatol 2018 Sep;179(3):590-598. Epub 2018 Jun 21 doi: 10.1111/bjd.16557. PMID: 29570779
A meta-analysis of nevus-associated melanoma: Prevalence and practical implications.
Pampena R, Kyrgidis A, Lallas A, Moscarella E, Argenziano G, Longo C
J Am Acad Dermatol 2017 Nov;77(5):938-945.e4. Epub 2017 Aug 29 doi: 10.1016/j.jaad.2017.06.149. PMID: 28864306
Characterization, treatment, and outcome of uveal melanoma in the first two years of life.
Yousef YA, Alkilany M
Hematol Oncol Stem Cell Ther 2015 Mar;8(1):1-5. Epub 2014 Oct 6 doi: 10.1016/j.hemonc.2014.09.004. PMID: 25300563

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