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Diagnostyka i leczenie chorych na mięsaka maziówkowego

Magdalena Zielińska12, Katarzyna Kozak1, Tomasz Świtaj1, Aneta Borkowska1, Bartłomiej Szostakowski13, Anna Szumera-Ciećkiewicz45, Elżbieta Bylina16, Paweł Sobczuk17, Sławomir Falkowski1, Piotr Rutkowski1, Anna M. Czarnecka18
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
  3. Samodzielny Publiczny Szpital Kliniczny im. prof. Adama Grucy CMKP w Otwocku
  4. Zakład Patomorfologii Nowotworów, Narodowy Instytut Onkologii im. Marii Skłodowskiej-Curie — Państwowy Instytut Badawczy w Warszawie
  5. Zakład Diagnostyki Hematologicznej, Instytut Hematologii i Transfuzjologii w Warszawie
  6. Dział Badań Klinicznych, Narodowy Instytut Onkologii im. Marii Skłodowskiej-Curie — Państwowy Instytut Badawczy w Warszawie
  7. Zakład Fizjologii Doświadczalnej i Klinicznej, Laboratorium Centrum Badań Przedklinicznych, Warszawski Uniwersytet Medyczny
  8. Zakład Farmakologii Doświadczalnej, Instytut Medycyny Doświadczalnej i Klinicznej im. M. Mossakowskiego PAN w Warszawie

dostęp płatny

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PRACE PRZEGLĄDOWE (REVIEW ARTICLES)
Opublikowany online: 2023-07-06

Streszczenie

Mięsak maziówkowy (SaSy, sarcoma synoviale) jest nowotworem złośliwym, wywodzącym się z prymitywnych komórek mezenchymalnych. Nowotwory te stanowią około 5–10% wszystkich mięsaków tkanek miękkich (MTM). Zajmują jednak 3. miejsce wśród MTM, jeśli chodzi o częstość występowania w lokalizacji kończynowej. Występują najczęściej u młodych dorosłych w 3. i 4. dekadzie życia, jako powoli rosnąca zmiana, która staje się agresywna od pewnego etapu choroby. Nowotwór ten charakteryzuje się specyficzną translokacją między genem na chromosomie 18 (SS18) a genem na chromosomie X (SSX1, SSX2 lub SSX4). Leczenie chorych na SaSy powinno być prowadzone w ośrodku referencyjnym. Podstawą leczenia mięsaka maziówkowego pozostaje chirurgiczne wycięcie z ujemnymi marginesami z dodatkiem radioterapii i/lub chemioterapii w zależności od charakterystyki pacjenta i guza. Chemioterapię pierwszego rzutu stanowi obecnie leczenie skojarzone doksorubicyną z ifosfamidem, które jest stosowane także jako terapia podstawowa choroby uogólnionej. Alternatywną opcję dla pacjentów z chorobą uogólnioną, którzy nie mogą być leczeni doksorubicyną, stanowi monoterapia ifosfamidem w wysokich dawkach. Nowe pojawiające się metody leczenia chorych na SaSy obejmują regorafenib i terapie celowane przeciwko NYESO- 1 i MAGE-4 z użyciem genetycznie modyfikowanych limfocytów T. Nowe terapie ukierunkowane na geny oraz leczenie immunomodulujące mogą w przyszłości poprawić wyniki leczenia chorych na mięsaka maziówkowego w stadium zaawansowanym i obecnością przerzutów odległych. Obecnie jednak pazopanib jest jedynym lekiem celowanym dopuszczonym do leczenia SaSy. Według ClinicalTrials.gov po 2016 roku rozpoczęło się ponad 30 badań klinicznych nad leczeniem chorych z przerzutową i zaawansowaną postacią SaSy.

Streszczenie

Mięsak maziówkowy (SaSy, sarcoma synoviale) jest nowotworem złośliwym, wywodzącym się z prymitywnych komórek mezenchymalnych. Nowotwory te stanowią około 5–10% wszystkich mięsaków tkanek miękkich (MTM). Zajmują jednak 3. miejsce wśród MTM, jeśli chodzi o częstość występowania w lokalizacji kończynowej. Występują najczęściej u młodych dorosłych w 3. i 4. dekadzie życia, jako powoli rosnąca zmiana, która staje się agresywna od pewnego etapu choroby. Nowotwór ten charakteryzuje się specyficzną translokacją między genem na chromosomie 18 (SS18) a genem na chromosomie X (SSX1, SSX2 lub SSX4). Leczenie chorych na SaSy powinno być prowadzone w ośrodku referencyjnym. Podstawą leczenia mięsaka maziówkowego pozostaje chirurgiczne wycięcie z ujemnymi marginesami z dodatkiem radioterapii i/lub chemioterapii w zależności od charakterystyki pacjenta i guza. Chemioterapię pierwszego rzutu stanowi obecnie leczenie skojarzone doksorubicyną z ifosfamidem, które jest stosowane także jako terapia podstawowa choroby uogólnionej. Alternatywną opcję dla pacjentów z chorobą uogólnioną, którzy nie mogą być leczeni doksorubicyną, stanowi monoterapia ifosfamidem w wysokich dawkach. Nowe pojawiające się metody leczenia chorych na SaSy obejmują regorafenib i terapie celowane przeciwko NYESO- 1 i MAGE-4 z użyciem genetycznie modyfikowanych limfocytów T. Nowe terapie ukierunkowane na geny oraz leczenie immunomodulujące mogą w przyszłości poprawić wyniki leczenia chorych na mięsaka maziówkowego w stadium zaawansowanym i obecnością przerzutów odległych. Obecnie jednak pazopanib jest jedynym lekiem celowanym dopuszczonym do leczenia SaSy. Według ClinicalTrials.gov po 2016 roku rozpoczęło się ponad 30 badań klinicznych nad leczeniem chorych z przerzutową i zaawansowaną postacią SaSy.

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Słowa kluczowe

mięsak maziówkowy; sarcoma synoviale; MTM; SS18; mięsak

Informacje o artykule
Tytuł

Diagnostyka i leczenie chorych na mięsaka maziówkowego

Czasopismo

Onkologia w Praktyce Klinicznej - Edukacja

Numer

Ahead of print

Typ artykułu

Artykuł przeglądowy

Opublikowany online

2023-07-06

Wyświetlenia strony

416

Wyświetlenia/pobrania artykułu

44

Słowa kluczowe

mięsak maziówkowy
sarcoma synoviale
MTM
SS18
mięsak

Autorzy

Magdalena Zielińska
Katarzyna Kozak
Tomasz Świtaj
Aneta Borkowska
Bartłomiej Szostakowski
Anna Szumera-Ciećkiewicz
Elżbieta Bylina
Paweł Sobczuk
Sławomir Falkowski
Piotr Rutkowski
Anna M. Czarnecka

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