Melanoom - WIN O · Melanoom Niet één diagnose, niet één standaardbehandeling Wolter J. Mooi VU...

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Melanoom Niet één diagnose, niet één

standaardbehandeling

Wolter J. Mooi

VU medisch centrum Amsterdam

Melanoomclassificatie

• Superficieel spreidend melanoom

• Nodulair melanoom

• Acrolentigineus melanoom

• Lentigo maligna melanoom

Wallace Clark

Richard Reed

Vincent McGovern

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

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

Superficieel spreidend melanoom

Nodulair melanoom

Acraal lentigineus melanoom

Lentigo maligna melanoom

Desmoplastisch

melanoom

Melanoom met

regressie Subunguaal

melanoom Amelanotisch

melanoom

Dermal hypermelanotic

low-grade melanoma

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

• 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)

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

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.

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

Dermal low-grade

hypermelanotic

melanoma

Multiple melanotic tumours of p16 -/- mouse, with occasional metastasis

(Krimpenfort et al., Nature 2001)

Dadras, Arch Lab Pathol Med, 2011

• Dermal low-grade

hypermelanotic melanoma

• Animal type melanoma

• Pigment synthesizing

melanoma

• Atypical pigmented dermal

melanocytoma

Melanoom: geen graderingssysteem.

Vreemd, eigenlijk....

T Stadiëring voor melanoom

Classificatie Tumordikte

(mm)

Ulceratie/mitosen

Tis NVT NVT

T1 ≤ 1.0 a: Zonder ulceratie en

mitosen < 1/mm2

b: Met ulceratie of

mitosen > 1/mm2

T2 >1.0-2.0 a: Zonder ulceratie

b: Met ulceratie

T3 2.1-4.0 a: Zonder ulceratie

b: Met ulceratie

T4 > 4.0 a: Zonder ulceratie

b: Met ulceratie

Taylor RC, Patel A, Panageas KS, Busam KJ,

Brady MS, J Clin Oncol 2007; 25: 869-75

2005

CCND1

locus

79% van tumoren met KIT mutaties en

53% met amplificaties toonden KIT

overexpressie

0 %

28 %

36 %

39 %

Optimale melanoomclassificatie:

werk in uitvoering