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Tom 9, Nr 4 (2023)
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Rozpoznanie i leczenie mięsaków prążkowanokomórkowych

Michał Łomiak12, Tomasz Świtaj1, Mateusz Spałek1, Joanna Radzikowska3, Marzanna Chojnacka4, Sławomir Falkowski1, Michał Wągrodzki5, Wojciech Kukwa3, Anna Szumera-Ciećkiewicz5, Piotr Rutkowski1, Anna M. Czarnecka1
Onkol Prakt Klin Edu 2023;9(4):264-294.
Afiliacje
  1. Klinika Nowotworów Tkanek Miękkich, Kości i Czerniaków, Narodowy Instytut Onkologii im. Marii Skłodowskiej-Curie — Państwowy Instytut Badawczy w Warszawie
  2. Wydział Lekarski, Warszawski Uniwersytet Medyczny, Warszawa
  3. Klinika Otorynolaryngologii, Wydział Lekarsko-Dentystyczny, Warszawski Uniwersytet Medyczny, Warszawa
  4. Klinika Onkologii i Radioterapii, Narodowy Instytut Onkologii im. Marii Skłodowskiej-Curie — Państwowy Instytut Badawczy w Warszawie
  5. Zakład Patomorfologii Nowotworów, Narodowy Instytut Onkologii im. Marii Skłodowskiej-Curie — Państwowy Instytut Badawczy w Warszawie

dostęp płatny

Tom 9, Nr 4 (2023)
PRACE PRZEGLĄDOWE (REVIEW ARTICLES)
Opublikowany online: 2022-09-01

Streszczenie

Mięśniakomięsak prążkowanokomórkowy (RMS) należy do mięsaków tkanek miękkich. Guz pierwotny najczęściej zlokalizowany jest w rejonie głowy i szyi, układu moczowo-płciowego oraz kończyn. Klasyfikacja Światowej Organizacji Zdrowia wyodrębniła cztery podtypy histopatologiczne RMS: zarodkowy, pęcherzykowy, pleomorficzny oraz wrzecionowatokomórkowy/ szkliwiejący. Diagnostyka różnicowa RMS obejmuje czerniaka, złośliwy nowotwór z osłonek nerwów obwodowych, tłuszczakomięsaka oraz PEComa. Do typowych zmian cytogenetycznych w RMS zalicza się translokacje chromosomowe t(2;13)(q35;q14) oraz t(1;13)(p36;q14). Prowadzą one do powstania genów fuzyjnych, które cechuje wartość prognostyczna. W przebiegu RMS obecne mogą być także zmiany w szlakach sygnałowych, do których należą: RAS-PI3K, Wnt/b-katenina, ścieżki receptorów kinaz tyrozynowych i regulacji miogenezy. U 30% chorych w momencie rozpoznania RMS stwierdza się obecność przerzutów odległych, najczęściej do płuc, węzłów chłonnych, kości i szpiku kostnego. Leczenie chorych na RMS wymaga podejścia wielodyscyplinarnego, a wciąż doskonalone techniki diagnostyczne przyczyniają się do indywidualizacji strategii terapeutycznych. Optymalne leczenie RMS w postaci zlokalizowanej bazuje na chirurgii skojarzonej z radioterapią i chemioterapią. W przypadku obecności przerzutów odległych, podstawową metodę terapeutyczną stanowi wielolekowa chemioterapia, najczęściej oparta na: winkrystynie, daktynomycynie, ifosfamidzie/cyklofosfamidzie i etopozydzie. Pomimo intensywnego leczenia, wskaźnik 5-letniego przeżycia u dorosłych chorych na RMS nie przekracza 50%. Wciąż brakuje jednoznacznych wytycznych dotyczących postępowania u chorych z nawrotem miejscowym bądź odległym.

Streszczenie

Mięśniakomięsak prążkowanokomórkowy (RMS) należy do mięsaków tkanek miękkich. Guz pierwotny najczęściej zlokalizowany jest w rejonie głowy i szyi, układu moczowo-płciowego oraz kończyn. Klasyfikacja Światowej Organizacji Zdrowia wyodrębniła cztery podtypy histopatologiczne RMS: zarodkowy, pęcherzykowy, pleomorficzny oraz wrzecionowatokomórkowy/ szkliwiejący. Diagnostyka różnicowa RMS obejmuje czerniaka, złośliwy nowotwór z osłonek nerwów obwodowych, tłuszczakomięsaka oraz PEComa. Do typowych zmian cytogenetycznych w RMS zalicza się translokacje chromosomowe t(2;13)(q35;q14) oraz t(1;13)(p36;q14). Prowadzą one do powstania genów fuzyjnych, które cechuje wartość prognostyczna. W przebiegu RMS obecne mogą być także zmiany w szlakach sygnałowych, do których należą: RAS-PI3K, Wnt/b-katenina, ścieżki receptorów kinaz tyrozynowych i regulacji miogenezy. U 30% chorych w momencie rozpoznania RMS stwierdza się obecność przerzutów odległych, najczęściej do płuc, węzłów chłonnych, kości i szpiku kostnego. Leczenie chorych na RMS wymaga podejścia wielodyscyplinarnego, a wciąż doskonalone techniki diagnostyczne przyczyniają się do indywidualizacji strategii terapeutycznych. Optymalne leczenie RMS w postaci zlokalizowanej bazuje na chirurgii skojarzonej z radioterapią i chemioterapią. W przypadku obecności przerzutów odległych, podstawową metodę terapeutyczną stanowi wielolekowa chemioterapia, najczęściej oparta na: winkrystynie, daktynomycynie, ifosfamidzie/cyklofosfamidzie i etopozydzie. Pomimo intensywnego leczenia, wskaźnik 5-letniego przeżycia u dorosłych chorych na RMS nie przekracza 50%. Wciąż brakuje jednoznacznych wytycznych dotyczących postępowania u chorych z nawrotem miejscowym bądź odległym.

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Słowa kluczowe

mięśniakomięsak prążkowanokomórkowy; mięsak; mięsaki tkanek miękkich; RMS; RAS; translokacja

Informacje o artykule
Tytuł

Rozpoznanie i leczenie mięsaków prążkowanokomórkowych

Czasopismo

Onkologia w Praktyce Klinicznej - Edukacja

Numer

Tom 9, Nr 4 (2023)

Typ artykułu

Artykuł przeglądowy

Strony

264-294

Opublikowany online

2022-09-01

Wyświetlenia strony

612

Wyświetlenia/pobrania artykułu

112

Rekord bibliograficzny

Onkol Prakt Klin Edu 2023;9(4):264-294.

Słowa kluczowe

mięśniakomięsak prążkowanokomórkowy
mięsak
mięsaki tkanek miękkich
RMS
RAS
translokacja

Autorzy

Michał Łomiak
Tomasz Świtaj
Mateusz Spałek
Joanna Radzikowska
Marzanna Chojnacka
Sławomir Falkowski
Michał Wągrodzki
Wojciech Kukwa
Anna Szumera-Ciećkiewicz
Piotr Rutkowski
Anna M. Czarnecka

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