U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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]

Professional guidelines

PubMed

Ferreira A, Buckey TM, Grant-Kels JM
J Am Acad Dermatol 2024 Aug;91(2):402-403. Epub 2023 Jun 19 doi: 10.1016/j.jaad.2023.06.019. PMID: 37343828
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
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

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
Rieder H, Bartsch DK
Fam Cancer 2004;3(1):69-74. doi: 10.1023/B:FAME.0000026822.67291.a1. PMID: 15131409
Lynch HT, Deters CA, Hogg D, Lynch JF, Kinarsky Y, Gatalica Z
Cancer 2003 Nov 1;98(9):1947-57. doi: 10.1002/cncr.11743. PMID: 14584079

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...