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Familial atypical multiple mole melanoma syndrome(FAMMM; DNS; MLM)

MedGen UID:
389220
Concept ID:
C2314896
Neoplastic Process
Synonyms: Familial Atypical Mole Melanoma Syndrome; FAMILIAL ATYPICAL MULTIPLE MOLE MELANOMA-PANCREATIC CARCINOMA SYNDROME
SNOMED CT: FAMMM - Familial atypical mole malignant melanoma syndrome (254819008); Familial atypical mole malignant melanoma syndrome (254819008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Related genes: STK11, CDKN2A, BRAF
 
Monarch Initiative: MONDO:0018453
OMIM®: 155600
Orphanet: ORPHA404560

Definition

Familial atypical multiple mole melanoma (FAMMM) syndrome is an inherited genodermatosis characterized by the presence of multiple melanocytic nevi (often >50) and a family history of melanoma as well as, in a subset of patients, an increased risk of developing pancreatic cancer (see this term) and other malignancies. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Humphris JL, Biankin AV
Recent Results Cancer Res 2016;205:61-83. doi: 10.1007/978-3-319-29998-3_5. PMID: 27075349
Becker AE, Hernandez YG, Frucht H, Lucas AL
World J Gastroenterol 2014 Aug 28;20(32):11182-98. doi: 10.3748/wjg.v20.i32.11182. PMID: 25170203Free PMC Article

Recent clinical studies

Etiology

Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Lynch HT, Shaw TG
Fam Cancer 2016 Jul;15(3):487-91. doi: 10.1007/s10689-016-9888-2. PMID: 26892865
Soura E, Eliades PJ, Shannon K, Stratigos AJ, Tsao H
J Am Acad Dermatol 2016 Mar;74(3):395-407; quiz 408-10. doi: 10.1016/j.jaad.2015.08.038. PMID: 26892650Free PMC Article
Shi C, Hruban RH, Klein AP
Arch Pathol Lab Med 2009 Mar;133(3):365-74. doi: 10.5858/133.3.365. PMID: 19260742Free PMC Article

Diagnosis

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
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Lynch HT, Shaw TG
Fam Cancer 2016 Jul;15(3):487-91. doi: 10.1007/s10689-016-9888-2. PMID: 26892865
Soura E, Eliades PJ, Shannon K, Stratigos AJ, Tsao H
J Am Acad Dermatol 2016 Mar;74(3):395-407; quiz 408-10. doi: 10.1016/j.jaad.2015.08.038. PMID: 26892650Free PMC Article

Therapy

Klapman J, Malafa MP
Cancer Control 2008 Oct;15(4):280-7. doi: 10.1177/107327480801500402. PMID: 18813195

Prognosis

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
Becker AE, Hernandez YG, Frucht H, Lucas AL
World J Gastroenterol 2014 Aug 28;20(32):11182-98. doi: 10.3748/wjg.v20.i32.11182. PMID: 25170203Free PMC Article
Shi C, Hruban RH, Klein AP
Arch Pathol Lab Med 2009 Mar;133(3):365-74. doi: 10.5858/133.3.365. PMID: 19260742Free PMC Article
Klapman J, Malafa MP
Cancer Control 2008 Oct;15(4):280-7. doi: 10.1177/107327480801500402. PMID: 18813195
Rieder H, Bartsch DK
Fam Cancer 2004;3(1):69-74. doi: 10.1023/B:FAME.0000026822.67291.a1. PMID: 15131409

Clinical prediction guides

Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Becker AE, Hernandez YG, Frucht H, Lucas AL
World J Gastroenterol 2014 Aug 28;20(32):11182-98. doi: 10.3748/wjg.v20.i32.11182. PMID: 25170203Free PMC Article
Shi C, Hruban RH, Klein AP
Arch Pathol Lab Med 2009 Mar;133(3):365-74. doi: 10.5858/133.3.365. PMID: 19260742Free PMC Article
Slade J, Marghoob AA, Salopek TG, Rigel DS, Kopf AW, Bart RS
J Am Acad Dermatol 1995 Mar;32(3):479-94. doi: 10.1016/0190-9622(95)90073-x. PMID: 7868720
Lynch HT, Fusaro RM, Danes BS, Kimberling WJ, Lynch JF
Cancer Genet Cytogenet 1983 Apr;8(4):325-58. doi: 10.1016/0165-4608(83)90075-4. PMID: 6403218

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