Melanoom Niet één diagnose, niet één standaardbehandeling

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1 Melanoom Niet één diagnose, niet één standaardbehandeling Wolter J. Mooi VU medisch centrum Amsterdam

2 Melanoomclassificatie Superficieel spreidend melanoom Nodulair melanoom Acrolentigineus melanoom Lentigo maligna melanoom Wallace Clark Vincent McGovern Richard Reed

3 Melanoomclassificatie Superficieel spreidend melanoom Nodulair melanoom Acrolentigineus melanoom Lentigo maligna melanoom Subunguaal melanoom Spitzoïd melanoom Kinderen volwassenen Desmoplastisch melanoom Poypoïd melanoom Kleincellig naevoïd melanoom Verruceus naevoïd melanoom Lentigineus naevoïd melanoom Balloncelmelanoom Myxoïd melanoom Maligne blauwe naevus Dermaal hypermelanotisch laaggradig melanoom

4 Melanoomclassificatie: relevantie Sterk verschillende presentaties en histologische beelden Verschillende subtypen hebben verschillende diagnostische pitfalls Soms verschil in klinisch gedrag.b.v.: Spitzoïd melanoom bij kinderen; Desmoplastisch melanoom; Dermaal hypermelanotisch laaggradig melanoom

5 Zoogdieren

6 Superficieel spreidend melanoom

7 Nodulair melanoom

8 Acraal lentigineus melanoom

9 Lentigo maligna melanoom

10 Desmoplastisch melanoom Dermal hypermelanotic low-grade melanoma Melanoom met regressie Subunguaal melanoom Amelanotisch melanoom

11 Melanoomclassificatie: relevantie Sterk verschillende presentaties en histologische beelden Verschillende subtypen hebben verschillende diagnostische pitfalls Soms verschil in klinisch gedrag.b.v.: Spitzoïd melanoom bij kinderen; Desmoplastisch melanoom; Dermaal hypermelanotisch laaggradig melanoom

12 11 cases, 1-10 years (10 years = upper limit for inclusion in study) All were referral cases Initial diagnoses: Spitz tumour of uncertain malignant potential (4 cases) Atypical Spitz naevus (2 cases) Spitz naevus (2 cases) Cellular blue naevus (2 cases) No diagnosis (1 case) All cases diagnosed as melanoma by the authors All cases: metastasis (inclusion criterion)

13 Case nr Thickness (mm) Site of metastasis Months to metastasis FU (months) 1 8,0 Satellite 0 14, alive 2 4,2 Regional LN 1 2, alive 3 3,5 Regional LN 11 35, alive 4 9,0 Regional LN 2 13, alive 5 8,0 Regional LN 9 23, alive 6 6,5 Regional LN 1 5, alive 7 7,0 Regional LN 13 37, alive 8 11,0 Widespread 32 33, DOD 9 7,0 Regional LN 0 37, alive 10 9,7 Regional LN 1 6, alive 11 1,9+ Regional LN 10 14, alive

14 Regional nodal metastasis was uncommon in patients who presented with clinically localized pdm (1%) compared with those with mdm (10%) or CM (6%) (P <.05,pDM vs. CM). Five-year melanoma-specific mortality was lower for patients who presented with pdm compared with mdm (11% vs. 31%; P <.01). Patients with pdm and CM had a similar melanoma-specific mortality despite a 3-fold difference in median tumor depth (3.6 vs. 1.2 mm, respectively). Conclusions: DMs can be divided into two subtypes based on a histological quantification of desmoplasia. Tumors with prominent fibrosis (pure subtype) are unlikely to disseminate to regional lymph nodes and are associated with a favorable outcome when compared with those with mixed desmoplasia or CM.

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17 Hypermelanotic low grade dermal melanoma = Animal type melanoma = Pigment synthesizing melanoma = Pigmented epithelioid melanocytoma Follow-up of 64 cases: Locoregional recurrence and/or spread to regional lymph nodes: at least 22 cases Spread beyond region: 4 cases Tumour related deaths: 2 reported Possible relationship to epithelioid blue naevus and deep penetrating naevus requires further study

18 Dermal low-grade hypermelanotic melanoma

19 Multiple melanotic tumours of p16 -/- mouse, with occasional metastasis (Krimpenfort et al., Nature 2001)

20 Dermal low-grade hypermelanotic melanoma Animal type melanoma Pigment synthesizing melanoma Atypical pigmented dermal melanocytoma Dadras, Arch Lab Pathol Med, 2011

21 Melanoom: geen graderingssysteem. Vreemd, eigenlijk...

22 Classificatie T Stadiëring voor melanoom Tumordikte (mm) Tis NVT NVT Ulceratie/mitosen T1 1.0 a: Zonder ulceratie en mitosen < 1/mm 2 b: Met ulceratie of mitosen > 1/mm 2 T2 > a: Zonder ulceratie b: Met ulceratie T a: Zonder ulceratie b: Met ulceratie T4 > 4.0 a: Zonder ulceratie b: Met ulceratie

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24 Taylor RC, Patel A, Panageas KS, Busam KJ, Brady MS, J Clin Oncol 2007; 25:

25 2005

26 CCND1 locus

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28 0 % 36 % 28 % 39 % 79% van tumoren met KIT mutaties en 53% met amplificaties toonden KIT overexpressie

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37 Optimale melanoomclassificatie: werk in uitvoering