title: Multiple primary cutaneous melanomas in patients with FAMMM syndrome and sporadic atypical mole syndrome (AMS): what's worse?
abstract: Atypical Mole Syndrome is the most important phenotypic risk factor for cutaneous melanoma, a malignancy that accounts for about 80% of deaths from skin cancer. Since early diagnosis of melanoma is of great prognostic relevance, the identification of Atypical Mole Syndrome carriers (sporadic and familial) is essential, as well as the recommendation of preventative measures that must be undertaken by these patients.We report two rare cases concerning patients with multiple primary skin melanomas in the setting of a familial and a sporadic syndrome of dysplastic nevi: the first patient is a 67-year-old patient with a history of multiple superficial spreading melanomas localized on his back. The second patient presented with multiple primary melanomas in advanced stage in the context of the so-called sporadic form of the syndrome of dysplastic nevi-AMS (atypical mole syndrome). In the first case, excision of the melanomas was carried out with an uneventful post-operative period. In the second case, disseminated metastases were detected, involving the right fibula, the abdominal cavity as well as multiple lesions in the brain. The patient declined BRAF mutation tests as well as chemotherapy or targeted therapies, and suffered a rapid deterioration in his general condition leading to death. We classified the second case as a sporadic form of the atypical mole syndrome, associated with one nodular and two superficial spreading melanomas.There are no data in the literature to allow us to understand if, in patients with multiple primary melanomas, there is any difference in terms of prognosis between those with and without a family history of a similar phenotype. To answer this and other questions related to these rare cases, further studies with a significant number of patients should be carried out.
authors.affiliation: Policlinic for Dermatology and Venerology, Saint Kliment Ohridski University, Medical Faculty, University Hospital Lozenetz, Koziak street 1, 1407, Sofia, Bulgaria, georgi_tchernev@yahoo.de.
authors.forename: Georgi
authors.initials: G
authors.lastname: Tchernev
authors.forename: Julian
authors.initials: J
authors.lastname: Ananiev
authors.forename: José-Carlos
authors.initials: JC
authors.lastname: Cardoso
authors.forename: Anastasiya Atanasova
authors.initials: AA
authors.lastname: Chokoeva
authors.forename: Stanislav
authors.initials: S
authors.lastname: Philipov
authors.forename: Plamen Kolev
authors.initials: PK
authors.lastname: Penev
authors.forename: Torello
authors.initials: T
authors.lastname: Lotti
authors.forename: Uwe
authors.initials: U
authors.lastname: Wollina
mesh_terms: D000368:Aged; D001706:Biopsy; D046169:Dermoscopy; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008545:Melanoma / Q000175:diagnosis* / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000473:pathology; D009506:Nevus / Q000175:diagnosis* / Q000473:pathology; D011230:Precancerous Conditions / Q000175:diagnosis*; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000473:pathology; D013577:Syndrome; D000096142:Cutaneous Malignant Melanoma
references.citation: Int J Cancer. 2012 Apr 15;130(8):1715-25
references.pmid: 22025288
references.citation: Clin Exp Dermatol. 2005 Jan;30(1):68-70
references.pmid: 15663508
references.citation: Cancer. 2002 Jan 1;94(1):84-96
references.pmid: 11815963
references.citation: Br J Cancer. 1990 Jun;61(6):932-6
references.pmid: 2372499
references.citation: Int J Cancer. 2000 Sep 15;87(6):809-11
references.pmid: 10956390
references.citation: Clinics (Sao Paulo). 2011;66(3):493-9
references.pmid: 21552679
references.citation: J Am Acad Dermatol. 2012 Dec;67(6):1257-64
references.pmid: 22841127
references.citation: Cancer Epidemiol Biomarkers Prev. 2014 Jun;23(6):1080-9
references.pmid: 24876226
references.citation: J Med Genet. 2013 Apr;50(4):264-70
references.pmid: 23384855
references.citation: J Med Genet. 1978 Oct;15(5):352-6
references.pmid: 739524
references.citation: Tumori. 1976 Jan-Feb;62(1):127-30
references.pmid: 1014112
references.citation: Ann Surg. 2002 Dec;236(6):730-7
references.pmid: 12454511
references.citation: Arch Dermatol. 2003 Aug;139(8):1013-8
references.pmid: 12925389
references.citation: Br J Dermatol. 2011 Dec;165(6):1219-22
references.pmid: 21801156
references.citation: Can Med Assoc J. 1968 Jul 6;99(1):17-21
references.pmid: 5663000
references.citation: Br J Hosp Med (Lond). 2006 Jun;67(6):299-304
references.pmid: 16821732
references.citation: J Natl Cancer Inst. 2000 Aug 2;92(15):1260-6
references.pmid: 10922411
references.citation: J Am Acad Dermatol. 1995 Mar;32(3):479-94
references.pmid: 7868720
references.citation: J Med Genet. 1983 Oct;20(5):342-4
references.pmid: 6644764
references.citation: G Chir. 2012 Apr;33(4):132-5
references.pmid: 22668533
references.citation: Exp Dermatol. 2014 May;23(5):361-4
references.pmid: 24660985
references.citation: Arch Dermatol. 2010 Mar;146(3):257-64
references.pmid: 20231495
references.citation: Cancer Res. 2006 Oct 15;66(20):9818-28
references.pmid: 17047042
references.citation: Dermatol Clin. 1985 Apr;3(2):225-37
references.pmid: 3830486
references.citation: Dermatol Surg. 2004 Feb;30(2 Pt 2):291-6
references.pmid: 14871223
references.citation: Nat Genet. 2014 May;46(5):478-481
references.pmid: 24686849