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Fibromatosen

Zuletzt geändert von Thomas Brinkmeier am 2025/04/25 21:47

Fibromatosen

Def: Gruppe von gutartigen zellarmen Bindegewebsproliferationen mit graugelber Farbe und einer Größe von meist mehreren cm sowie invasivem Wachstum und Rezidivneigung

Etlg: - oberflächliche Fibromatosen

Pg: von Faszien ausgehend

KL: - geringere Größe

- langsameres Wachstum

Etlg: - Palmarfibromatose (Dupuytren-Kontraktur)

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TF: Vemurafenib?  

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Th: - Radiatio

Ind: insbes. in Frühstadien

- Kollagenase von Clostridium histolyticum

Appl: intraläsionale Injektionen

- Nadel-Fasziotomie

- komplexe Handchirurgie

- extrakorporale Stoßwellen (ESWL)

Lit: Lasers Med Sci. 2021 Jan 23. http://doi.org/10.1007/s10103-021-03254-9

Ind: Schmerzlinderung in den Nodi

- fraktionierter CO2-Laser

Lit: Dermatol Surg. 2019 Sep 24. http://doi.org/10.1097/DSS.0000000000002159

- Plantarfibromatose (M. Ledderhose)

Hi: 

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- Penisfibromatose

Def: chronisch entzündliche Fibromatose der Tunica albuginea des Penis mit dem Risiko von Verbiegungen und Knickbildung

Syn: Induratio penis plastica, Morbus Peyronie

Engl: Peyronie`s disease

Risk: Dyslipidämie, Adipositas, chronische Prostatitis, benigne Prostatahyperplasie, Autoimmunkrankheiten

Ass: Depression, erektile Dysfunktion

Th: - Röntgenbestrahlung

Bed: obsolet

- intraläsionale Injektion von Glukokortikoiden

Bed: umstritten

Altn: - oraler Steroidstoß, z. B. beginnend mit 60 mg Prednisolon und schrittweiser Dosisreduktion

Ind: Frühstadium

Bed: niedrige Evidenz

- intraläsionale Injektion von Verapamil

- intraläsionale Injektion von Botulinumtoxin

- NSAR wie Ibuprofen oder Diclofenac

Ind: symptomatisch im schmerzhaften Frühstadium

- Vitamin E

Bed: im Akutstadium

Dos: ca. 400 mg/Tag

NW: erhöhtes Risiko für Prostatakarzinom und zerebrovaskuläre Ereignisse

Co: ggf. Kombination mit Bromelain oder Omega-3-Fettsäuren (Leinöl, Fischölkapseln)

- Colchicin

Bed: umstritten

Dos: 0,5-2,5 mg/Tag über Monate

- PDE-5-Inhibitoren

Bed: im Akutstadium über Monate bis Jahre

Stoff: - Sildenafil

- Tadalafil

Dos: 2,5-5,0 mg/Tag

Wirk: Erhöhung von cGMP und NO mit Verstärkung der Fibroblasten-Apoptose und Hemmung der Kollagen-Synthese

- L-Arginin

Wirk: Stimulation der NO-Synthetase, Erhöhung von NO, Förderung der Fibroblasten-Apoptose

Dos: 3 g/Tag

Co: ggf. Kombination mit Citrullin

Dos: 3 g/Tag

- Pentoxyfillin

Wirk: PDE-Inhibitor, Hemmung der Fibroblasten-Proliferation, Erhöhung der fibrinolytischen Aktivität

Dos: 3x400 mg/Tag über Monate bis Jahre

- Extrakorporale Stoßwellenlithotripsie

Ind: kalzifizierte Plaques

- operativ-plastische Behandlung

Ind: chronische Phase (> 12 Monate nach Erstmanifestation)

- Pachydermodaktylie

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Vork: selten

Ät: repetitive mechanische Faktoren, insbes. im Rahmen von Zwangsneurosen

DD: degenerative Kollagenplaques der Hände

Lit: Dermatol Online J. 2022 Mar 15;28(2). http://doi.org/10.5070/D328257403

- infantile digitale Fibromatose

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Syn: (echte) Fingerknöchelpolster

Engl: knuckle pads, infantile digital fibromatosis

Lok: Metacarpophalangeal- oder proximale Interphalangealgelenke

Hi:  

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Verl: keine Tendenz zur Spontanremission

Th: ggf. Glukokortikoide intraläsional

Appl: - intraläsional

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- topisch

Co: Imiquimod 5% Creme

Lit: Dermatol Ther. 2020 Jul 13:e14012. http://doi.org/10.1111/dth.14012

- tiefe Fibromatosen

Syn: Desmoidtumoren

Pg: von Muskelaponeurosen ausgehend

KL: - größere Ausdehnung

- rascheres Wachstum

Etlg: - extraabdominelle Fibromatose/extraabdominelles Desmoid

Vork: meist Männer oder Kinder

Lok: meist Kopf, Hals, Rücken

- abdominelle Fibromatose/abdominelles Desmoid

Vork: meist Frauen im gebärfähigen Alter

Lok: meist Unterbauch

- intraabdominelle Fibromatose/intraabdominelles Desmoid

Etlg: - Beckenfibromatose

- Mesenterialfibromatose

- Desmoid bei Gardner-Syndrom

Prog: hohe Rezidivquote

DD: andere Tumoren des tendofaszialen Gewebes:

- Riesenzelltumor der Sehnenscheiden

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Syn: tenosynovialer Riesenzelltumor

Pg: Beginn als lokalisierte noduläre Synovialitis

Hi: 

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- Synovialsarkom

Pa: maligne

Pg: ausgehend von den Zellen des paraartikulären und peritendinösen Weichteilgewebes

  
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