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Kongenitaler Nävuszellnävus

Zuletzt geändert von Thomas Brinkmeier am 2025/04/25 21:41

Kongenitaler Nävuszellnävus

Def: gutartiger, angeborener Tumor der epidermalen Melanozyten

Gen: multiple kongenitale NZN sollen auf postzygotischen N-RAS-Mutationen beruhen

Lit: Ann Dermatol Venereol. 2014 Apr;141(4):314-5 (Frankreich)

Etlg: Einteilung nach Größe:

- klein: < 1,5 cm Durchmesser; mittel: 1,5-20 cm Durchmesser

Gen: häufig BRAF-Mutationen (Prävalenz erworbenen melanozytären Nävi entsprechend)

- mittel: 1,5-20 cm Durchmesser

- groß: > 20-40 cm Durchmesser

Gen: NRAS-Mutationen sind entscheidend, um die benigne Proliferation in utero voranzutreiben (BRAF-Mutationen fehlen meist)

Vork: ca. 70-80% d. F.

Lit: J Invest Dermatol. 2013 Oct 15. http://doi.org/10.1038/jid.2013.429 (Frankreich)

Lit:  

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- riesig: > 40 cm Durchmesser

Syn: Naevus giganteus, Badehosennävus, Tierfellnävus

Lit:  

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KL: scharf begrenzter, variabel pigmentierter Nävus mit rauher papillomatöser Oberfläche und meist Hypertrichose in Form schwarzer Borstenhaare oder Langhaare

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Hi: bandförmige Ansammlung von Nävuszellen im oberen Korium  

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Note: Die regelmäßigen und bläschenförmigen Zellen des Nävuszellnävus sind melanozytären Ursprungs ohne den Dendritenbaum der normalen Melanozyten, wodurch sie Tendenz zeigen, entlang den epithelialen Strukturen ins Korium vorzudringen und sich zu Strängen oder Nestern um Blutgefäße und Nerven zusammenzulagern.

So: kombinierter kongenitaler Nävus

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Syn: kongenitaler Nävus mit zwei Zellpopulationen

Engl: combined congenital nevus, congenital nevus with two cell populations

Note: Ein morphologischer Subtyp kongenitaler Nävi lässt sich klinisch-dermatoskopisch nicht von einem dysplastischen NZN unterscheiden.

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Kopl: - Entstehung von Melanomen

Vork: - 1-10% absolutes (lebenslanges) Melanomrisiko bei großen kongenitalen NZN

Man: meist schon innerhalb der ersten Lebensjahre

CV: erschwerte und dadurch häufig verzögerte Diagnostik aufgrund der Entwicklung unterhalb der dermoepidermalen Junktionszone

Note: In ca. 1/3 d. F. entsteht das Melanom an einer Hautpartie entfernt vom großen kongenitalen NZN.

- 0,1% absolutes (lebenslanges) Melanomrisiko bei kleinen kongenitalen NZN

Man: meist erst im Erwachsenenalter

Note: dermatoskopisch durch die Entwicklung im Bereich der dermoepidermalen Junktionszone meist frühzeitig zu identifizieren

Risk: - akute, exzessive Sonnenexposition (bes. in der Jugend) kann zur Aktivierung von kongenitalen NZN führen

- große kongenitale NZN (> 20 cm) oder multiple mittelgroße kongenitale NZN (1,5 bis 20 cm) oder kongenitale NZN mit proliferierenden Knoten

- multiple Satelliten-Nävi (> 20 Stück)

Prop: jährliche Kontrolluntersuchungen (am häufigsten während der ersten Lj.)

Lit: - Case Rep Dermatol Med. 2013;2013:473635.  

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- Br J Dermatol. 2017 May;176(5):1114.  

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-  

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- ZNS-Beteiligung

Vork: ca. 10-20% bei großen kongenitalen NZN

Etlg: - neurokutane (leptomeningeale oder intrazerebrale) Melanozytose

- ZNS-Malformationen wie Hydrocephalus

- nichtmelanozytäre ZNS-Tumoren

Di: MRT von Schädel und Rückenmark innerhalb der ersten 6 Lebensmonate

Prog: schlecht (Letalität von bis zu 50%) bei klinischer Symptomatik (z. B. Epilepsie, Hirndruckzeichen); eher günstig bei Asymptomatik

- Entstehung von Rhabdomyosarkomen

Vork: extrem selten

Lit: Pediatr Dermatol. 2014 Jun 9. http://doi.org/10.1111/pde.12359 (USA)

Web: http://www.nevus.org, http://www.naevus-netzwerk.de

Th: - Exzision von kleinen und mittelgroßen NZN und jeglichen malignitätsverdächtigen Nävi(-Anteilen)

Meth: ggf. mehrzeitige Exzisionen

- (früher praktizierte) Dermabrasion in den ersten Wochen nach der Geburt bei großen NZN/Tierfellnävi

Altn: Erbium-YAG-Laser

Bed: obsolet

  
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