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.
Autosomal dominant inheritance (Orphanet)
 
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

Dermatologists' ethical obligations to patients and their family members regarding genetic testing.
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
Diagnosis and Management of Hereditary Pancreatic Cancer.
Humphris JL, Biankin AV
Recent Results Cancer Res 2016;205:61-83. doi: 10.1007/978-3-319-29998-3_5. PMID: 27075349
Pancreatic ductal adenocarcinoma: risk factors, screening, and early detection.
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

Skin Cancer: Precancers.
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Skin Cancer Associated Genodermatoses: A Literature Review.
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Familial atypical multiple mole melanoma (FAMMM) syndrome: history, genetics, and heterogeneity.
Lynch HT, Shaw TG
Fam Cancer 2016 Jul;15(3):487-91. doi: 10.1007/s10689-016-9888-2. PMID: 26892865
Hereditary melanoma: Update on syndromes and management: Genetics of familial atypical multiple mole melanoma syndrome.
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
Familial pancreatic cancer.
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

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
Skin Cancer Associated Genodermatoses: A Literature Review.
Schierbeck J, Vestergaard T, Bygum A
Acta Derm Venereol 2019 Apr 1;99(4):360-369. doi: 10.2340/00015555-3123. PMID: 30653245
Familial atypical multiple mole melanoma (FAMMM) syndrome: history, genetics, and heterogeneity.
Lynch HT, Shaw TG
Fam Cancer 2016 Jul;15(3):487-91. doi: 10.1007/s10689-016-9888-2. PMID: 26892865
Familial pancreatic cancer.
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

Early detection of pancreatic cancer: why, who, and how to screen.
Klapman J, Malafa MP
Cancer Control 2008 Oct;15(4):280-7. doi: 10.1177/107327480801500402. PMID: 18813195

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
Pancreatic ductal adenocarcinoma: risk factors, screening, and early detection.
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
Familial pancreatic cancer.
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
Familial pancreatic cancer.
Rieder H, Bartsch DK
Fam Cancer 2004;3(1):69-74. doi: 10.1023/B:FAME.0000026822.67291.a1. PMID: 15131409
Familial sarcoma: challenging pedigrees.
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

Skin Cancer: Precancers.
Bruner P, Bashline B
FP Essent 2019 Jun;481:23-27. PMID: 31188549
Pancreatic ductal adenocarcinoma: risk factors, screening, and early detection.
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
Familial pancreatic cancer.
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
Atypical mole syndrome: risk factor for cutaneous malignant melanoma and implications for management.
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
A review of hereditary malignant melanoma including biomarkers in familial atypical multiple mole melanoma syndrome.
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.

    Reviews

    Related information

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...