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familiair melanoom van huid (aandoening)
familiair melanoom van huid
familiair maligne melanoom van huid
familiaal maligne huidmelanoom
familiair melanoma maligna cutis
Familial malignant melanoma of skin
Familial cutaneous malignant melanoma
A rare inherited form of cutaneous melanoma with characteristics of development of histologically confirmed melanoma in two first degrees relatives or more relatives in an affected family. It is thought to account for about 10% of all cases of cutaneous melanoma. Tends to occur earlier than non-familial melanoma. The risk of familial melanoma is closely related to a wide range of genetic alterations in susceptibility genes but also appears to be influenced by phenotypic risk factors, such as pigmentation, freckling and sun reactions. Complex interactions between genetic and environmental factors are therefore thought to underlie the disease. The most common high-penetrance susceptibility gene implicated is CDKN2A, accounting for predisposition in approximately 20% of cases. In some affected families, susceptibility is consistent with autosomal dominant inheritance but in most cases, a polygenic mode of inheritance appears likely.
Id726019003
StatusDefined
Associated morphologymelanoom - categorie
Finding sitestructuur van huid
referentieset met complexe 'mapping' naar ICD-10
TargetC43.9
RuleTRUE
AdviceALWAYS C43.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified
TargetZ80.8
RuleTRUE
AdviceALWAYS Z80.8
CorrelationSNOMED CT source code to target map code correlation not specified