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Dermatofibrosarcoma protuberans (DFSP)

Zuletzt geändert von Thomas Brinkmeier am 2025/04/25 20:40

Dermatofibrosarcoma protuberans (DFSP)

Engl: dermatofibrosarcoma protuberans

Def: semimaligner bzw. intermediär maligner, rezidivfreudiger fibroblastischer Bindegewebstumor mit lokal invasivem Wachstum aber nur geringer Metastasierungsfrequenz

Histr: Erstbeschreibung durch Darier im Jahre 1924 und Bednar im Jahre 1957; metaphorische Beschreibung als Eisberg-Tumor, der über histologische Ausdehnungen verfügt, die zunächst visuell oder palpatorisch unterschätzt werden können

Vork: - zweithäufigster (semi-)maligner Weichteiltumor (nach atypischem Fibroxanthom bzw. pleomorphem Sarkom)

- Männer überwiegen Frauen

- mittleres Lebensalter (meist junge Erwachsene), aber auch Kinder

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Lit: - JAAD Case Rep. 2020 May 19;6(7):606-607

- Dermatol Online J. 2023 Jun 15;29(3). http://doi.org/10.5070/D329361440

- Clin Cosmet Investig Dermatol. 2024 Aug 27;17:1921-1930. http://doi.org/10.2147/CCID.S480616

-  

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Gen: - Auftreten von Ringchromosomen bestehend aus translozierten Segmenten der Chromosomen 17 und 22

- Fusion der Gene für Kollagen Typ I alpha1 (COL1A1) und für die Beta-Kette von PDGF (PDGFB)

Lit: J Dermatol Sci 2003; 33: 161-7

PPh: genetische Translokation führt zu einem Fusionsprotein, das den PDGF-Rezeptor stimuliert

Filia: - 5% d. F.

- vorwiegend hämatogen (Lunger, seltener Leber, Niere, Knochen), selten lymphogen

Lit:  

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KL: - mehrere Zentimeter große, derbe, plattenartige Infiltration

DD: von Frühstadien:

- Narbe

Lit:  

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- Morphea

- sklerodermiformes Basalzellkarzinom

- Porphyria cutanea tarda (PCT) mit sklerodermiformer Umwandlung

- Entwicklung von hautfarbenen oder pigmentierten keloidähnlichen Knoten in den o.g. Hautveränderungen

So: gestielt-noduläres DFSP

Lit:  

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DD: von Spätstadien:

- Keloide

- Erythema elevatum et diutinum

CV: Die Protuberanzen sind typisch, aber nicht obligat; die Klinik ist sehr variabel!

Lok: - Prädilektionsstelle: oberer Stamm

- seltener: proximale Extremitäten

- sehr selten: Kopf-Hals-Region

Lit:  

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Di: - Histologie und Immunhistologie

- ggf. präoperative Bildgebung (Sonographie, Computertomografie/MRT) zur Abschätzung der Tiefeninfiltration

- Lymphknotensonographie und Röntgen Thorax

Ind: obligat nur bei Rezidiven

ALM:  

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DD: Keloid, Histiozytom, kutane Lymphome, Sarkome anderer Art, sklerodermiformes Basalzellkarzinom, kutane Metastasen, plexiformer fibrohistiozytärer Tumor

Hi: - Bündel aus länglichen Fibroblasten/Fibrozyten und Kollagenfasern mit charakteristischer Anordnung im Speichenrad- bzw. Honigwabenmuster mit Umfließen der Fettgewebslolbuli durch spindelzellige Tumorzellen  

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Engl: storiform or cartwheel pattern

- häufig Riesenzell-Fibroblastom-ähnliche Areale und Infiltration des Subkutangewebes

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Engl: giant cell fibroblastoma-like areas

- selten Mitosen

DD: benigne atypische Dermatofibrome, pleomorphes dermales Sarkom, Dermatomyofibrom = plaqueförmige dermale Fibromatose

IHC: - CD34-Antigen positiv

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Eig: 115 kD

Note: Expression des Antigens auf:

- Hämatopoetischen Stammzellen

- Endothelzellen

- dendritischen Zellen

DD: - Expression unter anderem auch beim Kaposi-Sarkom und Glomustumor und Trichodiskom

- Eine neue Entität ist der rezidivierende superfizielle CD34-positive fibroblastische Tumor

Lit: - J Cutan Pathol. 2022 Sep 15. http://doi.org/10.1111/cup.14329

- Ann Dermatol. 2023 May;35(Suppl 1):S25-S29. http://doi.org/10.5021/ad.20.328

- Skin Res Technol. 2024 Sep;30(9):e70033. http://doi.org/10.1111/srt.70033

- Faktor XIIIa positiv

- Apolipoprotein D (Apo D) positiv

- Nestin

Lit: J Cutan Pathol. 2013 Jul 29. http://doi.org/10.1111/cup.12203 (Spanien)

Bed: hohe Nestin-Expression korreliert mit Invasivität

- Cathepsin K negativ

- Stromelysin-3 negativ Am J Dermatopathol. 2023 Dec 1;45(12):812-815. doi: 10.1097/DAD.0000000000002562

Lit: J Am Acad Dermatol 2002; 46: 408-13

Bed: Stromelysin ist ein "negativer" Marker für DFSP und verlässlicher in der Differenzierung vom benignen Dermatofibrom als CD34

- Connexin-43 negativ

Lit: Am J Dermatopathol. 2023 Dec 1;45(12):812-815. http://doi.org/10.1097/DAD.0000000000002562

So: - pigmentiertes DFSP

Syn: Bednar-Tumor

Hi: Einzelzellen mit Melanin-Einlagerung

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- myxoides DFSP

Pg: Akkumulation von sauren Glykosaminoglykanen

Lit:  

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- fibrosarkomatöses DFSP

Filia: höhere Metastasierungsrate von ca. 10-15%

Prog: schlechter als beim klassischen DFSP (auch deutlich niedrigere Ansprechrate auf Imatinib)

- myoides DFSP

- atrophes DFSP

- granuläres DFSP

Prog: - 45% Rezidive nach Exzision

CV: histologisch mit bis zu 6 cm langen pseudopodienartigen Ausläufern

Risk: Tumorgröße besonders bedeutsam

Lit: J Am Acad Dermatol. 2023 Jul 5:S0190-9622(23)01264-1. http://doi.org/10.1016/j.jaad.2023.06.044

- infaust bei Metastasierung

- erhöhtes Risiko für Zweitmalignome, insbesondere für nichtepithelialen Hautkrebs bis 10 Jahre nach Diagnosestellung

Lit: J Am Acad Dermatol. 2024 Feb 1:S0190-9622(24)00188-9. http://doi.org/10.1016/j.jaad.2023.12.068

Lit: -  

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- Dermatol Pract Concept. 2022 Jan 1;12(1):e2022033. http://doi.org/10.5826/dpc.1201a33

Th: Exzision

Bed: GS

Meth: - weit und tief exzidieren, möglichst unter Einschluss der Faszie

- bei konventioneller Exzision sollte der SA nach Möglichkeit mind. 3 cm, bei mikrographischer Chirurgie ca. 1 cm betragen

CV: histologische Randkontrolle; genaue Nachbeobachtung

Altn: - Radiatio

Ind: - primäre Inoperabilität

- bei positiven Schnitträndern (R1-Situation) oder makroskopischen Tumorresten (R2-Situation) oder mehrfachen Rezidiven

- ggf. auch bei R0-Resektion

Lit: J Eur Acad Dermatol Venereol. 2016 Feb 16. http://doi.org/10.1111/jdv.13601 (Taiwan)

PT: SR / MA

Dos: 5x/Woche 2 Gy ED; Gesamtdosis von 50 Gy (palliativ) bis 70 Gy (kurativ)

- Chemotherapie

Bed: nicht etabliert

So: Elektrochemotherapie

Lit: Eur J Dermatol. 2013 Apr 8. [Epub ahead of print]

- Tyrosinkinase-Inhibitoren

Stoff: - Imatinib

Bed: Ansprechraten um 50% in klinischen Studien

Lit: J Dtsch Dermatol Ges. 2019 May 22. http://doi.org/10.1111/ddg.13849

- Pazopanib

Lit: J Dermatol. 2016 Dec 18. http://doi.org/10.1111/1346-8138.13717 (Japan)

Ind: CR (metastasiertes, therapieresistentes fibrosarkomatöses DFSP ohne genetische Translokationen)

  
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