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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

Skin Cancer: Precancers.
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Melanoma progression.
Elder DE
Pathology 2016 Feb;48(2):147-54. Epub 2016 Jan 16 doi: 10.1016/j.pathol.2015.12.002. PMID: 27020387
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
Dysplastic nevi.
Clarke LE
Clin Lab Med 2011 Jun;31(2):255-65. doi: 10.1016/j.cll.2011.03.003. PMID: 21549239
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

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
Skin Cancer: Precancers.
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Melanoma progression.
Elder DE
Pathology 2016 Feb;48(2):147-54. Epub 2016 Jan 16 doi: 10.1016/j.pathol.2015.12.002. PMID: 27020387
Atypical genital nevus.
Brenn T
Arch Pathol Lab Med 2011 Mar;135(3):317-20. doi: 10.5858/2009-0382-RA.1. PMID: 21366454
Dermoscopy: basic concepts.
Campos-do-Carmo G, Ramos-e-Silva M
Int J Dermatol 2008 Jul;47(7):712-9. doi: 10.1111/j.1365-4632.2008.03556.x. PMID: 18613881

Therapy

Eruptive Melanocytic Nevi: A Review.
Burian EA, Jemec GBE
Am J Clin Dermatol 2019 Oct;20(5):669-682. doi: 10.1007/s40257-019-00444-8. PMID: 31119650
Dysplastic/Clark naevus in the era of molecular pathology.
Mesbah Ardakani N
Australas J Dermatol 2019 Aug;60(3):186-191. Epub 2019 Mar 10 doi: 10.1111/ajd.13019. PMID: 30854639
Trajectories of premalignancy during the journey from melanocyte to melanoma.
Colebatch AJ, Scolyer RA
Pathology 2018 Jan;50(1):16-23. Epub 2017 Nov 10 doi: 10.1016/j.pathol.2017.09.002. PMID: 29132722
Epidemiology of melanoma.
Rigel DS
Semin Cutan Med Surg 2010 Dec;29(4):204-9. doi: 10.1016/j.sder.2010.10.005. PMID: 21277533
Precursors to skin cancer.
Sober AJ, Burstein JM
Cancer 1995 Jan 15;75(2 Suppl):645-50. doi: 10.1002/1097-0142(19950115)75:2+<645::aid-cncr2820751405>3.0.co;2-1. PMID: 7804989

Prognosis

Overview of familial syndromes with increased skin malignancies.
Juan HY, Zhou AE, Hoegler KM, Khachemoune A
Arch Dermatol Res 2023 May;315(4):707-727. Epub 2022 Nov 7 doi: 10.1007/s00403-022-02447-8. PMID: 36342513
Eruptive Melanocytic Nevi: A Review.
Burian EA, Jemec GBE
Am J Clin Dermatol 2019 Oct;20(5):669-682. doi: 10.1007/s40257-019-00444-8. PMID: 31119650
Melanoma progression.
Elder DE
Pathology 2016 Feb;48(2):147-54. Epub 2016 Jan 16 doi: 10.1016/j.pathol.2015.12.002. PMID: 27020387
Dysplastic nevi.
Clarke LE
Clin Lab Med 2011 Jun;31(2):255-65. doi: 10.1016/j.cll.2011.03.003. PMID: 21549239
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

Clinical prediction guides

Skin Cancer: Precancers.
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Trajectories of premalignancy during the journey from melanocyte to melanoma.
Colebatch AJ, Scolyer RA
Pathology 2018 Jan;50(1):16-23. Epub 2017 Nov 10 doi: 10.1016/j.pathol.2017.09.002. PMID: 29132722
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
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
Skin precancer.
Brash DE, Pontén J
Cancer Surv 1998;32:69-113. PMID: 10489624

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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