Niezróżnicowany mięsak pleomorficzny – Rokowania, prognozy i postęp choroby

Niezróżnicowany mięsak pleomorficzny
Rokowania, prognozy i postęp choroby

Niezróżnicowany mięsak pleomorficzny (UPS) to agresywny, wysokiego stopnia złośliwości mięsak tkanek miękkich, stanowiący około 15% przypadków mięsaków. Leczenie standardowe obejmuje chirurgię z uzyskaniem marginesów ujemnych oraz radioterapię. Wskaźniki 5-letniego przeżycia całkowitego wynoszą około 60%, a 10-letnie około 48%, z medianą przeżycia 10,1 lat. Niekorzystne czynniki prognostyczne to starszy wiek (HR 1,03), obecność przerzutów w chwili diagnozy (HR 2,89), lokalizacja w kończynie dolnej (HR 2,30) oraz dodatnie marginesy chirurgiczne (HR 2,68). Duże guzy (≥5 cm) znacząco zwiększają ryzyko przerzutów odległych (HR do 6,80). Resekcja R0 jest kluczowa dla poprawy przeżycia wolnego od wznowy miejscowej, przerzutów i całkowitego przeżycia. Wysoka ekspresja COL6A3 koreluje z gorszym rokowaniem, a wskaźniki immunologiczne, takie jak CD276, mają prognostyczne znaczenie. Wczesne wykrycie i odpowiednia terapia są kluczowe dla poprawy wyników klinicznych.

Wprowadzenie do niezróżnicowanego mięsaka pleomorficznego

Niezróżnicowany mięsak pleomorficzny (Undifferentiated Pleomorphic Sarcoma, UPS) to rzadki złośliwy nowotwór pochodzenia mezenchymalnego, który powstał po reklasyfikacji włókniakomięsaka histiocytarnego złośliwego (malignant fibrous histiocytoma, MFH). Stanowi około 15% wszystkich przypadków mięsaków tkanek miękkich i charakteryzuje się agresywnym przebiegiem klinicznym, z częstym rozwojem wznowy miejscowej i przerzutów odległych.¹²

Nowotwór ten należy do wysokiego stopnia złośliwości mięsaków tkanek miękkich, w przypadku których leczeniem z wyboru jest zabieg chirurgiczny dążący do uzyskania marginesów ujemnych, w połączeniu z radioterapią. Wskaźniki 5-letniego przeżycia całkowitego (OS) wynoszą około 60%, a 10-letniego przeżycia – około 48%, z medianą czasu przeżycia 10,1 lat.³⁴

Czynniki prognostyczne wpływające na rokowanie

Czynniki kliniczne i patologiczne

Analiza wieloczynnikowa wykazała, że niekorzystnymi czynnikami prognostycznymi związanymi ze zmniejszonym całkowitym przeżyciem są:⁵

Starszy wiek (HR 1,03)
Obecność przerzutów w momencie diagnozy (HR 2,89)
Lokalizacja w kończynie dolnej (w przeciwieństwie do korzystnej prognozy dla guzów kończyny górnej, HR 2,30)

⁵

Złe rokowanie dla pooperacyjnej wznowy miejscowej wiąże się z:⁶

Starszym wiekiem (HR 1,03)
Dodatnimi marginesami chirurgicznymi (HR 2,68)

⁶

Zwiększone ryzyko pooperacyjnych przerzutów odległych obserwuje się u pacjentów z:⁷⁸

Dużymi guzami (5-9 cm, HR 4,42; ≥10 cm, HR 6,80) – w specyficznym dla UPS raporcie, występowanie przerzutów po leczeniu (37,6%) było istotnie związane z guzami ≥5 cm
Przerzutami w momencie diagnozy (HR 3,99)

⁷⁸

Leczenie uzupełniające wykazało korzystny wpływ, zmniejszając ryzyko przerzutów odległych (HR 0,34).⁹

Czynniki związane z przerzutami do węzłów chłonnych i przerzutami odległymi

Badania wykazały, że ogólne ryzyko śmiertelności pacjentów z UPS z przerzutami do węzłów chłonnych było 2,10 razy wyższe niż pacjentów bez przerzutów do węzłów chłonnych. Odpowiednie ryzyko zgonu u pacjentów z przerzutami odległymi było 4,79 i 4,51 razy wyższe niż u pacjentów bez przerzutów odległych.¹⁰

Lokalizacja guza i marginesy chirurgiczne

Badania wskazują, że rokowanie jest gorsze u pacjentów z głębszymi i większymi guzami ściany klatki piersiowej.¹¹ Większy rozmiar guza oraz bliskość ważnych struktur może ograniczać dostępne marginesy chirurgiczne, prowadząc do gorszych prognoz i wyników.¹²

Resekcja R0 (całkowite usunięcie guza z marginesami wolnymi od nacieku nowotworowego) okazała się jedynym niezależnym korzystnym czynnikiem prognostycznym, który korelował z przeżyciem wolnym od wznowy miejscowej (LRFS), przeżyciem wolnym od przerzutów odległych (DMFS) i całkowitym przeżyciem (OS).¹³

Różnicowanie w kierunku mięśnia gładkiego

Znaczenie kliniczne dokładnego diagnozowania mięsaków pleomorficznych zostało wykazane, szczególnie w przypadkach niezróżnicowanych mięsaków pleomorficznych z różnicowaniem miogennym, które wydają się znacznie bardziej agresywne.¹⁴

Metoda klasyfikacji różnicowania mięśni gładkich, uwzględniająca nie tylko pozytywny wynik dla markerów mięśni gładkich, ale także intensywność ich znakowania, wykazała najsilniejszą wartość prognostyczną w porównaniu z innymi czynnikami prognostycznymi, w tym klasyfikacją histologiczną.¹⁵

Markery molekularne i genomiczne w rokowaniu UPS

Podpisy genomiczne

Niezróżnicowane mięsaki pleomorficzne wysokiego stopnia (UPS) wykazują najczęstsze zmiany w regionach genomowych obejmujące powielenie w 20q13.33 i 7q22.1 oraz utratę w 3p26.3.¹⁶

W przypadku mięsaków gładkokomórkowych (LMS), które są blisko spokrewnione z UPS, powielenie w regionie 1q21.3 było znacząco związane ze skróconym czasem całkowitego przeżycia. Analiza wieloczynnikowa wykazała, że powielenie w 1q21.3 było niezależnym markerem prognostycznym krótszego czasu przeżycia u pacjentów z LMS (HR=13,76; P=0,019).¹⁷

Ponadto, skrócony czas całkowitego przeżycia był znacząco związany z powieleniem w regionach:¹⁸

1q21.3 (P=0,002)
11q12.2-q12.3 (P=0,005)
16p11.2 (P=0,033)
19q13.12 (P=0,027)

¹⁸

Biomarkery prognostyczne

Badania wykazały, że COL6A3 (kolagen typu VI łańcuch alfa 3) jest markerem prognostycznym w UPS, który przewyższa skuteczność CINSARC (Complexity INdex in SARComas) w tym podtypie mięsaka.¹⁹

COL6A3, drugi najbardziej znaczący gen w danych scRNA-seq, był najbardziej predykcyjny dla całkowitego przeżycia u 46 pacjentów z UPS (p=0,00232), a następnie BGN (p=0,00843). Ekspresja COL6A3 w całym guzie jest prognostyczna dla całkowitego przeżycia w tej kohorcie.²⁰

Poprzez pojedynczą komórkę i przestrzenną transkryptomikę próbek FFPE, zidentyfikowano, że COL6A3 jest wyrażany w całej próbce guza, a jego wyższa ekspresja koreluje z gorszym całkowitym przeżyciem i przeżyciem wolnym od przerzutów w niezależnych kohortach niezróżnicowanego mięsaka pleomorficznego.²¹

Znaczenie mikrośrodowiska immunologicznego

Średnie wyniki immunologiczne przypadków UPS były najwyższe wśród wszystkich sześciu podtypów patologicznych, a następnie MFS, DDLPS, MPNST i LMS.²²

Krzywa przeżycia Kaplana-Meiera wykazała, że czas przeżycia grupy z wysokim wynikiem immunologicznym był dłuższy niż grupy z niskim wynikiem (P=0,001).²³

Pacjenci w grupie z niskim wynikiem stromalnym mieli znacząco gorsze czasy całkowitego przeżycia w porównaniu do grupy z wysokim wynikiem (P=0,035).²⁴

Wśród genów o podwyższonej regulacji w grupie z wysokim wynikiem immunologicznym, łącznie 528 genów było znacząco związanych z dobrym całkowitym przeżyciem, jak określono w analizie regresji Coxa.²⁵

Wyniki wskazały, że CD276 był niezależnym czynnikiem ryzyka, a indeks prognostyczny był obliczany w następujący sposób: Wynik ryzyka = -0,3529 × ekspTIGIT + 4,4598 × ekspCD276 − 3,0041 × ekspCD47 − 0,6090 × ekspIDO1.²⁶

Rokowanie w różnych typach UPS

Różnice w rokowaniu między podtypami

Niezróżnicowany mięsak pleomorficzny i pokrewne guzy obejmują zakres biologii z różnymi prognozami. Genetyczne wskaźniki prognostyczne, takie jak CINSARC, działają słabo w tym podtypie mięsaka, a histopatologiczne wskaźniki, takie jak głębokość inwazji, nie mogą być wykonane w momencie diagnozy.²⁷

Obecnie prognoza dla włókniakomięsaka atypowego (AFX) i mięsaka pleomorficznego skóry (PDS) opiera się na histopatologicznych dowodach inwazji do tkanki podskórnej. Guzy inwazyjne (PDS) mają wyższe 5-letnie ryzyko wznowy miejscowej i przerzutów w porównaniu do guzów ograniczonych do skóry właściwej (AFX) (odpowiednio 17% vs. 10% i 16% vs. 0,8%).²⁸

Dlatego prognoza znacznie różni się w obrębie tego spektrum guzów, od bardzo rzadkiego wskaźnika przerzutów AFX do 50% wskaźnika przerzutów głębokiego UPS.²⁹

UPS związany z radiacją

Interesujące jest, że w jednej z publikacji 5-letnie przeżycie specyficzne dla choroby i wskaźniki nawrotów były znacznie gorsze w przypadku UPS związanego z promieniowaniem niż spontanicznego UPS, co sugeruje, że mogą istnieć odrębne profile mutacyjne dla tej choroby.³⁰

Badanie potwierdza również wcześniejsze doniesienia, że UPS związany z promieniowaniem częściej występuje w tułowiu.³¹

Wskaźniki przeżycia i monitorowanie

Wskaźniki przeżycia

Oczekiwana długość życia różni się w zależności od rozmiaru i stadium mięsaka:³²

Wskaźnik 5-letniego przeżycia dla UPS wysokiego stopnia wynosi około 60% (oznacza to, że około 60% osób zdiagnozowanych z tym schorzeniem żyje pięć lat później)
Wskaźnik 10-letniego przeżycia wynosi 48%

³²³³³⁴

Pięcioletni wskaźnik przeżycia całkowitego zazwyczaj waha się od 55% do 65%, a pacjenci z wyższymi stopniami UPS wykazują gorsze długoterminowe przeżycie w porównaniu z innymi mięsakami tkanek miękkich.³⁵

W retrospektywnym badaniu 166 pacjentów z UPS po medianie obserwacji wynoszącej 55 miesięcy wskaźnik wznowy miejscowej (LR) wynosił 22,9% (38/166), przerzutów odległych (DM) 32,5% (54/166), a całkowitego przeżycia (OS) 75,3% (125/166).³⁶

Monitorowanie i obserwacja

Wizyty kontrolne są ważne w celu wykrycia wznów miejscowych lub przerzutów. Badanie fizyczne guzów w stadium I powinno być przeprowadzane w odstępach 3-6 miesięcy przez pierwsze dwa lata, a następnie corocznie.³⁷

Możliwe jest wyleczenie UPS, szczególnie we wczesnych stadiach. Nadal mogą istnieć opcje leczenia w późniejszych stadiach, które mogą przedłużyć jakość życia. Podobnie jak w przypadku każdego rodzaju raka, wczesne wykrycie i leczenie zwiększają szanse na pomyślne wyniki.³⁸

Narzędzia prognostyczne

Nomogram predykcyjny

Stworzony nomogram może indywidualnie przewidywać 3-letnie i 5-letnie całkowite przeżycie pacjentów z UPS, z dobrą dyskryminacją, dokładnością i użytecznością kliniczną. Może on stanowić punkt odniesienia dla klinicystów i pacjentów do podejmowania lepszych decyzji klinicznych.³⁹

Wczesne rozpoznanie i strategie leczenia

Wczesne rozpoznanie choroby i odpowiednia strategia leczenia są najważniejszymi interwencjami poprawiającymi ogólne rokowanie. W bieżącym retrospektywnym badaniu 319 pacjentów z trzech ośrodków opieki trzeciorzędowej, nawroty i przerzuty wystąpiły odpowiednio w 14,1% i 7,8% przypadków.⁴⁰

Leczenie UPS obejmującego klatkę piersiową jest niezwykle złożone. Jedno z badań wykazało brak różnicy w nawrocie lub przerzutach między pacjentami leczonymi i nieleczonymi innymi terapiami.⁴¹

Istniejące badanie określa, że UPS w tułowiu, rozmiar guza ≥5 cm i margines resekcji R1/R2 są markerami prognostycznymi złego przeżycia całkowitego. Margines resekcji R1/R2 znacząco koreluje z wysokim wskaźnikiem wznowy miejscowej, a kobiety są bardziej podatne na wznowę miejscową. UPS w tułowiu i margines resekcji R1/R2 są znacząco skorelowane z przerzutami odległymi, a starsi pacjenci (≥60 lat) są bardziej podatni na przerzuty odległe.⁴²

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Materiały źródłowe

#1 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#2 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK570612/
Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. In a current retrospective study of 319 patients from three tertiary care centers, recurrences and metastases occurred in 14.1% and 7.8% of the cases, respectively. […] The 5- and 10-year overall survival rates were 60% and 48%, respectively. Interestingly, in another publication, the 5-year disease-specific survival and recurrence rates were significantly worse in radiation-associated UPS than sporadic UPS, suggesting there might be distinct mutational profiles for the disease. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. […] In a UPS-specific report, post-treatment local recurrence (15%) was statistically associated with advanced age and inadequate surgical margins, whilst post-treatment metastatic disease (37.6%) was significantly relevant in tumors 5 cm.
#3 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#4 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20âyear experience of 266 cases | CoLab
https://colab.ws/articles/10.1111%2Fans.15348
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. Data of UPS tumours from 1996 to 2016 were collected, totalling 266 unique UPS patients. Median follow-up was 7.8 years. All tumours were retrospectively analysed for prognostic factors of the disease, including local recurrence (LR) and metastatic disease (MD) at diagnosis, tumour size, grade, location and depth, patient age, adjuvant therapy and surgical margin. Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age […] Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years.
#5 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#6 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#7 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#8 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK570612/
Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. In a current retrospective study of 319 patients from three tertiary care centers, recurrences and metastases occurred in 14.1% and 7.8% of the cases, respectively. […] The 5- and 10-year overall survival rates were 60% and 48%, respectively. Interestingly, in another publication, the 5-year disease-specific survival and recurrence rates were significantly worse in radiation-associated UPS than sporadic UPS, suggesting there might be distinct mutational profiles for the disease. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. […] In a UPS-specific report, post-treatment local recurrence (15%) was statistically associated with advanced age and inadequate surgical margins, whilst post-treatment metastatic disease (37.6%) was significantly relevant in tumors 5 cm.
#9 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#10 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text
https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z
Undifferentiated pleomorphic sarcoma (UPS) is considered one of the most common types of soft tissue sarcoma (STS). Current studies have shown that the prognosis of UPS is related to some of its clinical characteristics, but no survival prediction model for the overall survival (OS) of UPS patients has been reported. The purpose of this study is to construct and validate a nomogram for predicting OS in UPS patients at 3, 5 years after the diagnosis. […] With good discrimination, accuracy, and clinical practicability, the nomogram can individualize the prediction of 3-year and 5-year OS in patients with UPS, which can provide a reference for clinicians and patients to make better clinical decisions. […] The overall mortality risk of UPS patients with lymph node metastasis was 2.10 times higher than the patients without lymph node metastasis, respectively. The corresponding risks of death in the patients with distant metastasis were 4.79 and 4.51 times higher than that in the patients without distant metastasis. […] The nomogram can individually predict 3-year and 5-year OS of UPS patients, with good discrimination, accuracy and clinical utility. It can provide a reference for clinicians and patients to make better clinical decisions.
#11 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#12 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#13 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research
https://tcr.amegroups.org/article/view/62307/html
At the end of follow-up, the rate of LR, DM and OS in 166 UPS patients was 22.9% (38/166), 32.5% (54/166) and 75.3% (125/166) with a median follow-up time of 55 months. […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The existing study determines that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.
#14 Smooth muscle differentiation identifies two classes of poorly differentiated pleomorphic sarcomas with distinct outcome | Modern Pathology
https://www.nature.com/articles/modpathol2013205
The clinical relevance of accurately diagnosing pleomorphic sarcomas has been shown, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which appear significantly more aggressive. […] Collectively, our results show that the smooth muscle differentiation classification method may be a useful diagnostic tool as well as a relevant prognostic tool for undifferentiated pleomorphic sarcomas. […] Undifferentiated pleomorphic sarcomas are tumors with no specific line of differentiation. […] They are predominantly observed in the limbs and present a slightly better prognosis with a 5-year survival rate ranging between 50% and 70%, and local recurrence and metastasis rates of 35-50% and 30-50%, respectively. […] The clinical relevance of a better method of diagnosing undifferentiated pleomorphic sarcomas has been demonstrated, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which seem to be significantly more aggressive.
#15 Smooth muscle differentiation identifies two classes of poorly differentiated pleomorphic sarcomas with distinct outcome | Modern Pathology
https://www.nature.com/articles/modpathol2013205
The smooth muscle differentiation classification method, taking into account not only positivity for smooth muscle markers but also their labeling intensity, exhibited the strongest prognostic value compared with other prognostic factors, including histological classification. […] The cumulative incidence of metastasis in smooth muscle differentiation reclassified tumors was intermediate between tumors equally classified by both methods as undifferentiated pleomorphic sarcomas and as leiomyosarcomas, although it was closer to incidence for tumors classified as leiomyosarcomas by both methods. […] These results show that the smooth muscle differentiation classification method could not only be a useful diagnostic tool but also a relevant prognostic tool for undifferentiated pleomorphic sarcomas.
#16 Genomic Signatures Predict Poor Outcome in Undifferentiated Pleomorphic Sarcomas and Leiomyosarcomas | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0067643
Genomic signatures predict poor outcome in undifferentiated pleomorphic sarcomas and leiomyosarcomas. Undifferentiated high-grade pleomorphic sarcomas (UPSs) display aggressive clinical behavior and frequently develop local recurrence and distant metastasis. The most frequently altered UPS regions involved gains at 20q13.33 and 7q22.1 and losses at 3p26.3. Gains at 1q21.3 were significantly associated with reduced overall survival times in LMS patients. A multivariate analysis revealed that gains at 1q21.3 were an independent prognostic marker of shorter survival times in LMS patients (HR=13.76; P=0.019). Gains at 1q21.3, 11q12.2-q12.3, and 19q13.12 were significantly associated with death caused by LMS. Furthermore, reduced overall survival time was significantly associated with gains at 1q21.3 (P=0.002), 11q12.2-q12.3 (P=0.005), 16p11.2 (P=0.033), and 19q13.12 (P=0.027). Gains at 1q21.3 were shown to be an independent prognostic marker for shorter survival times in LMS patients, suggesting that genes mapped to this region may be involved in the aggressiveness of LMS tumors.
#17 Genomic Signatures Predict Poor Outcome in Undifferentiated Pleomorphic Sarcomas and Leiomyosarcomas | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0067643
Genomic signatures predict poor outcome in undifferentiated pleomorphic sarcomas and leiomyosarcomas. Undifferentiated high-grade pleomorphic sarcomas (UPSs) display aggressive clinical behavior and frequently develop local recurrence and distant metastasis. The most frequently altered UPS regions involved gains at 20q13.33 and 7q22.1 and losses at 3p26.3. Gains at 1q21.3 were significantly associated with reduced overall survival times in LMS patients. A multivariate analysis revealed that gains at 1q21.3 were an independent prognostic marker of shorter survival times in LMS patients (HR=13.76; P=0.019). Gains at 1q21.3, 11q12.2-q12.3, and 19q13.12 were significantly associated with death caused by LMS. Furthermore, reduced overall survival time was significantly associated with gains at 1q21.3 (P=0.002), 11q12.2-q12.3 (P=0.005), 16p11.2 (P=0.033), and 19q13.12 (P=0.027). Gains at 1q21.3 were shown to be an independent prognostic marker for shorter survival times in LMS patients, suggesting that genes mapped to this region may be involved in the aggressiveness of LMS tumors.
#18 Genomic Signatures Predict Poor Outcome in Undifferentiated Pleomorphic Sarcomas and Leiomyosarcomas | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0067643
Among the 15 minimally recurrent altered regions identified in the LMS samples, four regions showing gains were significantly associated with reduced overall survival time (1q21.3, 11q12.2-q12.3, 16p11.2, and 19q13.12). To our knowledge, this signature of poor prognosis has not been previously reported for LMS.
#19 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
While seven of these nine genes trended toward worse survival with high expression, only two reached significance. COL6A3, the second most significant gene in our scRNA-seq data, was most predictive of overall survival in the 46 patients with UPS (p=0.00232), followed by BGN (p=0.00843). […] Nevertheless, expression of COL6A3 in bulk tumor is prognostic of overall survival in this cohort. […] We therefore propose that COL6A3 is a prognostic marker in UPS, and, within this soft-tissue sarcoma subtype, outperforms CINSARC. […] In summary, undifferentiated pleomorphic sarcomas and related tumors encompass a range of biology with varying prognoses. Genetic prognostic indicators such as CINSARC perform poorly on this sarcoma subtype and histopathologic indicators such as depth of invasion cannot be performed at the time of diagnosis. Through single-cell and spatial transcriptomics of retrospective FFPE samples, we identified that COL6A3 is expressed throughout the tumor specimen and that higher expression is correlated with poor overall and metastasis-free survival in independent cohorts of undifferentiated pleomorphic sarcoma.
#20 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
While seven of these nine genes trended toward worse survival with high expression, only two reached significance. COL6A3, the second most significant gene in our scRNA-seq data, was most predictive of overall survival in the 46 patients with UPS (p=0.00232), followed by BGN (p=0.00843). […] Nevertheless, expression of COL6A3 in bulk tumor is prognostic of overall survival in this cohort. […] We therefore propose that COL6A3 is a prognostic marker in UPS, and, within this soft-tissue sarcoma subtype, outperforms CINSARC. […] In summary, undifferentiated pleomorphic sarcomas and related tumors encompass a range of biology with varying prognoses. Genetic prognostic indicators such as CINSARC perform poorly on this sarcoma subtype and histopathologic indicators such as depth of invasion cannot be performed at the time of diagnosis. Through single-cell and spatial transcriptomics of retrospective FFPE samples, we identified that COL6A3 is expressed throughout the tumor specimen and that higher expression is correlated with poor overall and metastasis-free survival in independent cohorts of undifferentiated pleomorphic sarcoma.
#21 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
While seven of these nine genes trended toward worse survival with high expression, only two reached significance. COL6A3, the second most significant gene in our scRNA-seq data, was most predictive of overall survival in the 46 patients with UPS (p=0.00232), followed by BGN (p=0.00843). […] Nevertheless, expression of COL6A3 in bulk tumor is prognostic of overall survival in this cohort. […] We therefore propose that COL6A3 is a prognostic marker in UPS, and, within this soft-tissue sarcoma subtype, outperforms CINSARC. […] In summary, undifferentiated pleomorphic sarcomas and related tumors encompass a range of biology with varying prognoses. Genetic prognostic indicators such as CINSARC perform poorly on this sarcoma subtype and histopathologic indicators such as depth of invasion cannot be performed at the time of diagnosis. Through single-cell and spatial transcriptomics of retrospective FFPE samples, we identified that COL6A3 is expressed throughout the tumor specimen and that higher expression is correlated with poor overall and metastasis-free survival in independent cohorts of undifferentiated pleomorphic sarcoma.
#22 Comprehensive analysis of immune infiltration and gene expression for predicting survival in patients with sarcomas | Aging
https://www.aging-us.com/article/202229/text
The average immune scores of the UPS cases were ranked highest among all six pathological subtypes, followed by those of MFS, DDLPS, MPNST and LMS. […] A Kaplan-Meier survival curve showed that the survival time of the high-immune-score group was longer than that of the low-score group (Figure 1J, P=0.001). […] Patients in the low-stromal-score group were significantly worse off in their overall survival times compared to the high-score group (Figure 1K, P=0.035). […] Among the DEGs upregulated in the high-immune-score group, a total of 528 DEGs were significantly related to good overall survival, as determined by univariate Cox regression analysis. […] After performing log-rank tests in the TCGA SARC cohort and validating these good-prognosis-related genes with the GEO dataset GSE17679, we extracted information indicating that NR1H3, VAMP5, GIMAP2, GBP2, HLA-E and CRIP1 were highly expressed in the immune microenvironment and were most significantly associated with predicting good outcomes in sarcoma patients.
#23 Comprehensive analysis of immune infiltration and gene expression for predicting survival in patients with sarcomas | Aging
https://www.aging-us.com/article/202229/text
The average immune scores of the UPS cases were ranked highest among all six pathological subtypes, followed by those of MFS, DDLPS, MPNST and LMS. […] A Kaplan-Meier survival curve showed that the survival time of the high-immune-score group was longer than that of the low-score group (Figure 1J, P=0.001). […] Patients in the low-stromal-score group were significantly worse off in their overall survival times compared to the high-score group (Figure 1K, P=0.035). […] Among the DEGs upregulated in the high-immune-score group, a total of 528 DEGs were significantly related to good overall survival, as determined by univariate Cox regression analysis. […] After performing log-rank tests in the TCGA SARC cohort and validating these good-prognosis-related genes with the GEO dataset GSE17679, we extracted information indicating that NR1H3, VAMP5, GIMAP2, GBP2, HLA-E and CRIP1 were highly expressed in the immune microenvironment and were most significantly associated with predicting good outcomes in sarcoma patients.
#24 Comprehensive analysis of immune infiltration and gene expression for predicting survival in patients with sarcomas | Aging
https://www.aging-us.com/article/202229/text
The average immune scores of the UPS cases were ranked highest among all six pathological subtypes, followed by those of MFS, DDLPS, MPNST and LMS. […] A Kaplan-Meier survival curve showed that the survival time of the high-immune-score group was longer than that of the low-score group (Figure 1J, P=0.001). […] Patients in the low-stromal-score group were significantly worse off in their overall survival times compared to the high-score group (Figure 1K, P=0.035). […] Among the DEGs upregulated in the high-immune-score group, a total of 528 DEGs were significantly related to good overall survival, as determined by univariate Cox regression analysis. […] After performing log-rank tests in the TCGA SARC cohort and validating these good-prognosis-related genes with the GEO dataset GSE17679, we extracted information indicating that NR1H3, VAMP5, GIMAP2, GBP2, HLA-E and CRIP1 were highly expressed in the immune microenvironment and were most significantly associated with predicting good outcomes in sarcoma patients.
#25 Comprehensive analysis of immune infiltration and gene expression for predicting survival in patients with sarcomas | Aging
https://www.aging-us.com/article/202229/text
The average immune scores of the UPS cases were ranked highest among all six pathological subtypes, followed by those of MFS, DDLPS, MPNST and LMS. […] A Kaplan-Meier survival curve showed that the survival time of the high-immune-score group was longer than that of the low-score group (Figure 1J, P=0.001). […] Patients in the low-stromal-score group were significantly worse off in their overall survival times compared to the high-score group (Figure 1K, P=0.035). […] Among the DEGs upregulated in the high-immune-score group, a total of 528 DEGs were significantly related to good overall survival, as determined by univariate Cox regression analysis. […] After performing log-rank tests in the TCGA SARC cohort and validating these good-prognosis-related genes with the GEO dataset GSE17679, we extracted information indicating that NR1H3, VAMP5, GIMAP2, GBP2, HLA-E and CRIP1 were highly expressed in the immune microenvironment and were most significantly associated with predicting good outcomes in sarcoma patients.
#26 Comprehensive analysis of immune infiltration and gene expression for predicting survival in patients with sarcomas | Aging
https://www.aging-us.com/article/202229/text
The results indicated that CD276 was an independent risk factor. […] The prognosis index was calculated as follows: Risk score = -0.3529 expTIGIT + 4.4598 expCD276 3.0041 expCD47 0.6090 expIDO1. […] The results showed that 11 kinds of highly infiltrating immune cells were predictors of longer overall survival, most of which have been confirmed in previous studies.
#27 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
Undifferentiated pleomorphic sarcoma (UPS) and related tumors are the most common type of soft tissue sarcoma. However, this spectrum of tumors has different etiologies with varying rates of metastasis and survival. […] Therefore, prognosis varies greatly within this spectrum of tumors, from a very rare metastasis rate of AFX to 50% metastasis rate of deep UPS. […] Currently, prognosis for AFX and PDS is based on histopathologic evidence of invasion into the subcutis. Invasive tumors (PDS) have a higher 5-year risk of local recurrence and metastasis compared to tumors confined to the dermis (AFX) (17% vs. 10% and 16% vs. 0.8%, respectively). […] However, in an independent cohort that we analyzed, CINSARC does not perform well on UPS. […] We were particularly interested in whether our findings apply to UPS, which falls in the broader family of dedifferentiated sarcomas with AFX and PDS, although they are now considered biologically distinct tumors.
#28 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
Undifferentiated pleomorphic sarcoma (UPS) and related tumors are the most common type of soft tissue sarcoma. However, this spectrum of tumors has different etiologies with varying rates of metastasis and survival. […] Therefore, prognosis varies greatly within this spectrum of tumors, from a very rare metastasis rate of AFX to 50% metastasis rate of deep UPS. […] Currently, prognosis for AFX and PDS is based on histopathologic evidence of invasion into the subcutis. Invasive tumors (PDS) have a higher 5-year risk of local recurrence and metastasis compared to tumors confined to the dermis (AFX) (17% vs. 10% and 16% vs. 0.8%, respectively). […] However, in an independent cohort that we analyzed, CINSARC does not perform well on UPS. […] We were particularly interested in whether our findings apply to UPS, which falls in the broader family of dedifferentiated sarcomas with AFX and PDS, although they are now considered biologically distinct tumors.
#29 Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma | Molecular Cancer | Full Text
https://molecular-cancer.biomedcentral.com/articles/10.1186/s12943-024-02168-8
Undifferentiated pleomorphic sarcoma (UPS) and related tumors are the most common type of soft tissue sarcoma. However, this spectrum of tumors has different etiologies with varying rates of metastasis and survival. […] Therefore, prognosis varies greatly within this spectrum of tumors, from a very rare metastasis rate of AFX to 50% metastasis rate of deep UPS. […] Currently, prognosis for AFX and PDS is based on histopathologic evidence of invasion into the subcutis. Invasive tumors (PDS) have a higher 5-year risk of local recurrence and metastasis compared to tumors confined to the dermis (AFX) (17% vs. 10% and 16% vs. 0.8%, respectively). […] However, in an independent cohort that we analyzed, CINSARC does not perform well on UPS. […] We were particularly interested in whether our findings apply to UPS, which falls in the broader family of dedifferentiated sarcomas with AFX and PDS, although they are now considered biologically distinct tumors.
#30 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK570612/
Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. In a current retrospective study of 319 patients from three tertiary care centers, recurrences and metastases occurred in 14.1% and 7.8% of the cases, respectively. […] The 5- and 10-year overall survival rates were 60% and 48%, respectively. Interestingly, in another publication, the 5-year disease-specific survival and recurrence rates were significantly worse in radiation-associated UPS than sporadic UPS, suggesting there might be distinct mutational profiles for the disease. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. […] In a UPS-specific report, post-treatment local recurrence (15%) was statistically associated with advanced age and inadequate surgical margins, whilst post-treatment metastatic disease (37.6%) was significantly relevant in tumors 5 cm.
#31 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#32 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma
Life expectancy varies depending on the size and stage of the sarcoma. The five-year survival rate for high-grade UPS is about 60%. That means that around 60% of people diagnosed with this condition are still alive five years later. The 10-year survival rate is 48%. […] Its possible to cure UPS, especially during the early stages. There still may be treatment options in later stages, which can prolong your quality of life. Like any type of cancer, early detection and treatment increase your chances for successful outcomes.
#33 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases – PubMed
https://pubmed.ncbi.nlm.nih.gov/31364245/
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. […] The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P 0.001; 4.42), 10 cm (P 0.001; 6.80)) and MD at diagnosis (P 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P 0.001; 0.34). […] UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.
#34 Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20âyear experience of 266 cases | CoLab
https://colab.ws/articles/10.1111%2Fans.15348
Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. Data of UPS tumours from 1996 to 2016 were collected, totalling 266 unique UPS patients. Median follow-up was 7.8 years. All tumours were retrospectively analysed for prognostic factors of the disease, including local recurrence (LR) and metastatic disease (MD) at diagnosis, tumour size, grade, location and depth, patient age, adjuvant therapy and surgical margin. Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age […] Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years.
#35 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#36 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research
https://tcr.amegroups.org/article/view/62307/html
At the end of follow-up, the rate of LR, DM and OS in 166 UPS patients was 22.9% (38/166), 32.5% (54/166) and 75.3% (125/166) with a median follow-up time of 55 months. […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The existing study determines that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.
#37 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK570612/
Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. In a current retrospective study of 319 patients from three tertiary care centers, recurrences and metastases occurred in 14.1% and 7.8% of the cases, respectively. […] The 5- and 10-year overall survival rates were 60% and 48%, respectively. Interestingly, in another publication, the 5-year disease-specific survival and recurrence rates were significantly worse in radiation-associated UPS than sporadic UPS, suggesting there might be distinct mutational profiles for the disease. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. […] In a UPS-specific report, post-treatment local recurrence (15%) was statistically associated with advanced age and inadequate surgical margins, whilst post-treatment metastatic disease (37.6%) was significantly relevant in tumors 5 cm.
#38 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma
Life expectancy varies depending on the size and stage of the sarcoma. The five-year survival rate for high-grade UPS is about 60%. That means that around 60% of people diagnosed with this condition are still alive five years later. The 10-year survival rate is 48%. […] Its possible to cure UPS, especially during the early stages. There still may be treatment options in later stages, which can prolong your quality of life. Like any type of cancer, early detection and treatment increase your chances for successful outcomes.
#39 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text
https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z
Undifferentiated pleomorphic sarcoma (UPS) is considered one of the most common types of soft tissue sarcoma (STS). Current studies have shown that the prognosis of UPS is related to some of its clinical characteristics, but no survival prediction model for the overall survival (OS) of UPS patients has been reported. The purpose of this study is to construct and validate a nomogram for predicting OS in UPS patients at 3, 5 years after the diagnosis. […] With good discrimination, accuracy, and clinical practicability, the nomogram can individualize the prediction of 3-year and 5-year OS in patients with UPS, which can provide a reference for clinicians and patients to make better clinical decisions. […] The overall mortality risk of UPS patients with lymph node metastasis was 2.10 times higher than the patients without lymph node metastasis, respectively. The corresponding risks of death in the patients with distant metastasis were 4.79 and 4.51 times higher than that in the patients without distant metastasis. […] The nomogram can individually predict 3-year and 5-year OS of UPS patients, with good discrimination, accuracy and clinical utility. It can provide a reference for clinicians and patients to make better clinical decisions.
#40 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK570612/
Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. In a current retrospective study of 319 patients from three tertiary care centers, recurrences and metastases occurred in 14.1% and 7.8% of the cases, respectively. […] The 5- and 10-year overall survival rates were 60% and 48%, respectively. Interestingly, in another publication, the 5-year disease-specific survival and recurrence rates were significantly worse in radiation-associated UPS than sporadic UPS, suggesting there might be distinct mutational profiles for the disease. […] Follow-up visits are important to detect local recurrences or metastasis. Physical examination of stage I tumors should be performed at 3-to-6-month intervals for the first two years and then annually. […] In a UPS-specific report, post-treatment local recurrence (15%) was statistically associated with advanced age and inadequate surgical margins, whilst post-treatment metastatic disease (37.6%) was significantly relevant in tumors 5 cm.
#41 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology
https://cco.amegroups.org/article/view/119459/html
Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] The five-year overall survival rate typically ranges from 55% to 65%, and patients with higher grades of UPS exhibit worse long-term survival as compared with other STS counterparts. […] Larger tumor size as well as proximity to important structures may limit accessible margin distances, leading to poorer prognoses and outcomes. […] A final point to note is that this study also corroborates previous reports that radiation-associated UPS is more commonly found in the trunk as well.
#42 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research
https://tcr.amegroups.org/article/view/62307/html
At the end of follow-up, the rate of LR, DM and OS in 166 UPS patients was 22.9% (38/166), 32.5% (54/166) and 75.3% (125/166) with a median follow-up time of 55 months. […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The existing study determines that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.