Guzy desmoidowe – Rokowania, prognozy i postęp choroby

Guzy desmoidowe
Rokowania, prognozy i postęp choroby

Guzy desmoidowe to rzadkie, lokalnie inwazyjne nowotwory mezenchymalne, które nie dają przerzutów, ale charakteryzują się wysokim ryzykiem nawrotów po leczeniu. Pięcioletni wskaźnik przeżycia wynosi około 96%, a 15-letni 87%, jednak nawroty występują u 30-33% pacjentów w ciągu 5-10 lat, a w przypadku leczenia wyłącznie chirurgicznego wskaźniki te wzrastają do 34-38%. Nawroty są częstsze przy dodatnich marginesach resekcji (54% po 10 latach) w porównaniu do ujemnych (27%). Kluczowe czynniki prognostyczne to lokalizacja guza (gorsze rokowanie w kończynach, lepsze w ścianie jamy brzusznej), wiek pacjenta (młodszy wiek wiąże się z wyższym ryzykiem nawrotu) oraz rozmiar guza. Przebieg kliniczny jest nieprzewidywalny i może obejmować spontaniczną regresję, stabilizację lub progresję choroby, co utrudnia planowanie terapii.

Rokowanie w guzach desmoidowych (Desmoid tumors Prognosis)

Przeżywalność i nawroty
Czynniki prognostyczne
Nieprzewidywalny przebieg choroby
Wpływ metod leczenia na rokowanie
Nomogram prognostyczny
Wpływ guzów na jakość życia
Indywidualizacja prognozy

Wnioski dotyczące rokowania

Kolejne rozdziały

Rokowanie w guzach desmoidowych (Desmoid tumors Prognosis)

Guzy desmoidowe (łac. fibromatosis desmoides) to rzadkie mezenchymalne nowotwory, które charakteryzują się lokalną inwazyjnością, ale nie dają przerzutów. Mimo że rzadko zagrażają życiu, mogą być destrukcyjne i prowadzić do silnego bólu oraz upośledzenia funkcji narządów.¹ Rokowanie w przypadku guzów desmoidowych jest złożone i zależy od wielu czynników, które zostaną omówione poniżej.

Przeżywalność i nawroty

Guzy desmoidowe rzadko prowadzą do zgonu. Badania retrospektywne na grupie 189 pacjentów wykazały, że 5-letni wskaźnik przeżycia wynosi 96%, a 87% pacjentów nadal żyło po 15 latach. Jednak to, co stanowi największe wyzwanie w leczeniu tych guzów, to wysoki wskaźnik nawrotów. Aktuarialne wskaźniki nawrotów wynosiły 30% i 33% odpowiednio po 5 i 10 latach od leczenia.²³

W przypadku pacjentów leczonych wyłącznie chirurgicznie, wskaźniki nawrotów były wyższe i wynosiły 34% po 5 latach i 38% po 10 latach. Nawet wśród pacjentów z ujemnymi marginesami (całkowitą resekcją guza), 10-letni wskaźnik nawrotu wynosił 27%, podczas gdy u pacjentów z dodatnimi marginesami (niecałkowitą resekcją) 10-letni wskaźnik nawrotu sięgał 54% (P = 0,003).⁴⁵

W większym badaniu obejmującym 439 pacjentów poddanych całkowitej resekcji makroskopowej, u 100 (23%) wystąpił nawrót. Pięcioletnie przeżycie wolne od nawrotu miejscowego (LRFS) wynosiło 69%.⁶

Czynniki prognostyczne

Znanych jest kilka kluczowych czynników, które wpływają na rokowanie w guzach desmoidowych:

Lokalizacja guza – guzy zlokalizowane w kończynach mają gorsze rokowanie niż te znajdujące się w tułowiu. Guzy ściany jamy brzusznej mają znacznie niższy wskaźnik nawrotów w porównaniu z guzami kończyn i ściany klatki piersiowej.⁷⁸
Wiek pacjenta – młodszy wiek jest związany z wyższym ryzykiem nawrotu.⁹
Rozmiar guza – większe guzy charakteryzują się wyższym ryzykiem nawrotu.¹⁰
Margines chirurgiczny – chociaż niektóre badania wskazują na wpływ marginesu na rokowanie, w analizie wieloczynnikowej nie zawsze jest to czynnik niezależny.¹¹¹²

Nieprzewidywalny przebieg choroby

Jedną z najbardziej charakterystycznych cech guzów desmoidowych jest ich nieprzewidywalny przebieg kliniczny. Przebieg choroby może być bardzo zróżnicowany:¹³¹⁴¹⁵

Spontaniczna regresja – guzy mogą samoistnie się zmniejszać i ustępować bez leczenia
Stabilna choroba – guzy mogą pozostawać niezmienione przez długi czas
Progresja choroby – guzy mogą szybko rosnąć i powodować poważne powikłania

Co istotne, lekarze nie dysponują wiarygodnymi i zwalidowanymi czynnikami predykcyjnymi wzrostu guza, co utrudnia planowanie leczenia i prognozowanie przebiegu choroby.¹⁶

Wpływ metod leczenia na rokowanie

Różne metody leczenia oferują odmienne rezultaty, co przekłada się na rokowanie:

Leczenie chirurgiczne – szerokie wycięcie miejscowe z ujemnymi marginesami patologicznymi jest leczeniem z wyboru dla większości guzów desmoidowych, choć nawet wtedy wskaźniki nawrotów są znaczące.¹⁷
Radioterapia – w przypadku pacjentów leczonych radioterapią z powodu makroskopowej choroby, 10-letni aktuarialny wskaźnik nawrotu wynosił 24%.¹⁸
Leczenie skojarzone (chirurgia + radioterapia) – dla pacjentów leczonych skojarzonym wycięciem i radioterapią, 10-letni aktuarialny wskaźnik nawrotu wynosił 25%. Dodanie radioterapii niwelowało niekorzystny wpływ dodatnich marginesów obserwowany w grupie leczonej wyłącznie chirurgicznie.¹⁹²⁰
Nowe terapie – pojawiają się nowe opcje leczenia, które wykazują obiecujące wyniki. W jednym z badań odnotowano 71% redukcję ryzyka progresji w porównaniu z placebo, co stanowi znaczący postęp w leczeniu tych guzów.²¹

Nomogram prognostyczny

W celu lepszego przewidywania ryzyka nawrotu, opracowano pooperacyjny nomogram prognostyczny. Uwzględnia on tylko trzy czynniki: rozmiar guza, jego lokalizację oraz wiek pacjenta. Wszystkie te czynniki można ocenić przedoperacyjnie, co pozwala na skuteczną przedoperacyjną ocenę ryzyka i poradnictwo dla pacjentów.²²²³

Wpływ guzów na jakość życia

Chociaż guzy desmoidowe rzadko wpływają na długość życia, mogą znacząco obniżać jego jakość. Są trudne do całkowitego wyleczenia i mogą powodować przewlekły ból. Guzy rosnące w jamie brzusznej mogą powodować dodatkowe problemy, takie jak niedrożność jelit. Dlatego kluczowe jest, aby lekarz uważnie monitorował wzrost tych guzów.²⁴

Indywidualizacja prognozy

Każdy przypadek guza desmoidowego jest unikalny, a prognoza zależy od wielu czynników, w tym:²⁵

Lokalizacji guza w organizmie
Tempa wzrostu guza
Zakresu resekcji chirurgicznej

Warto podkreślić, że ze względu na rzadkość występowania guzów desmoidowych, wskaźniki przeżycia mogą nie być bardzo dokładne. Ponadto dane statystyczne nie uwzględniają nowszych, rozwijanych obecnie metod leczenia.²⁶

Wnioski dotyczące rokowania

Guzy desmoidowe stanowią wyjątkowe wyzwanie prognostyczne ze względu na ich nieprzewidywalny przebieg kliniczny. Mimo wysokiego wskaźnika przeżycia, znaczący odsetek pacjentów doświadcza nawrotów, nawet po całkowitej resekcji. Lokalizacja guza, wiek pacjenta i rozmiar guza są kluczowymi czynnikami prognostycznymi. Leczenie skojarzone, zwłaszcza łączenie chirurgii z radioterapią, może poprawić rokowanie, szczególnie w przypadkach z dodatnimi marginesami chirurgicznymi. Indywidualizacja podejścia terapeutycznego, oparta na czynnikach prognostycznych, jest niezbędna do optymalizacji wyników leczenia.²⁷²⁸²⁹

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

Materiały źródłowe

#1 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adultsâthe incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] These tumors are rarely fatal. In a retrospective study of 189 patients, the 5-year survival rate was 96%, and 87% of patients were still alive at 15 years. However, the actuarial relapse rate following surgical excision was 38% at 5 years. Even among patients with negative margins, the 10-year recurrence rate was 27%. […] The disease course is unpredictable. Patients can have spontaneous regression, long-lasting stable disease, or disease progression. Furthermore, physicians lack reliable and validated predictive factors for tumor growth.
#2 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adultsâthe incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] These tumors are rarely fatal. In a retrospective study of 189 patients, the 5-year survival rate was 96%, and 87% of patients were still alive at 15 years. However, the actuarial relapse rate following surgical excision was 38% at 5 years. Even among patients with negative margins, the 10-year recurrence rate was 27%. […] The disease course is unpredictable. Patients can have spontaneous regression, long-lasting stable disease, or disease progression. Furthermore, physicians lack reliable and validated predictive factors for tumor growth.
#3 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#4 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#5 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adultsâthe incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] These tumors are rarely fatal. In a retrospective study of 189 patients, the 5-year survival rate was 96%, and 87% of patients were still alive at 15 years. However, the actuarial relapse rate following surgical excision was 38% at 5 years. Even among patients with negative margins, the 10-year recurrence rate was 27%. […] The disease course is unpredictable. Patients can have spontaneous regression, long-lasting stable disease, or disease progression. Furthermore, physicians lack reliable and validated predictive factors for tumor growth.
#6 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#7 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#8 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
All of these predictors of recurrence can be evaluated preoperatively, allowing efficient preoperative estimation of risk and counseling of patients using the nomogram. […] Given the very different rates of local recurrence of abdominal wall lesions compared to extremity and chest wall tumors, these tumors may have a different biology or a different host-tumor-site interaction.
#9 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#10 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#11 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#12 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#13 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#14 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adultsâthe incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] These tumors are rarely fatal. In a retrospective study of 189 patients, the 5-year survival rate was 96%, and 87% of patients were still alive at 15 years. However, the actuarial relapse rate following surgical excision was 38% at 5 years. Even among patients with negative margins, the 10-year recurrence rate was 27%. […] The disease course is unpredictable. Patients can have spontaneous regression, long-lasting stable disease, or disease progression. Furthermore, physicians lack reliable and validated predictive factors for tumor growth.
#15 Desmoid Tumor – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: Where the tumor is in your body, How fast the tumor grows, How much of the tumor was taken out during surgery. […] Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Given that there are so few desmoid tumor patients, survival rates may not be very accurate. They also dont consider newer treatments being developed. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.
#16 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adultsâthe incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] These tumors are rarely fatal. In a retrospective study of 189 patients, the 5-year survival rate was 96%, and 87% of patients were still alive at 15 years. However, the actuarial relapse rate following surgical excision was 38% at 5 years. Even among patients with negative margins, the 10-year recurrence rate was 27%. […] The disease course is unpredictable. Patients can have spontaneous regression, long-lasting stable disease, or disease progression. Furthermore, physicians lack reliable and validated predictive factors for tumor growth.
#17 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. […] Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. […] Unresectable disease should be treated with definitive radiation therapy.
#18 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#19 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. […] For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. […] Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). […] Tumors located in an extremity also had a poorer prognosis than did those in the trunk. […] For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. […] For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. […] The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
#20 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. […] Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. […] Unresectable disease should be treated with definitive radiation therapy.
#21 Desmoid Tumors: The Tumor That Isnât a Cancer
https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer
Despite surgical resection, these tumors have a high rate of local recurrenceâup to 30% of patients see their disease return. […] There was a 71% reduction in risk of progression, which is huge, Shepard said. That’s a really big response in terms of being able to give a therapy and have a really long period of time before the patient starts to progress compared with placebo.
#22 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#23 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
All of these predictors of recurrence can be evaluated preoperatively, allowing efficient preoperative estimation of risk and counseling of patients using the nomogram. […] Given the very different rates of local recurrence of abdominal wall lesions compared to extremity and chest wall tumors, these tumors may have a different biology or a different host-tumor-site interaction.
#24 Desmoid Tumor – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: Where the tumor is in your body, How fast the tumor grows, How much of the tumor was taken out during surgery. […] Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Given that there are so few desmoid tumor patients, survival rates may not be very accurate. They also dont consider newer treatments being developed. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.
#25 Desmoid Tumor – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: Where the tumor is in your body, How fast the tumor grows, How much of the tumor was taken out during surgery. […] Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Given that there are so few desmoid tumor patients, survival rates may not be very accurate. They also dont consider newer treatments being developed. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.
#26 Desmoid Tumor – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: Where the tumor is in your body, How fast the tumor grows, How much of the tumor was taken out during surgery. […] Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Given that there are so few desmoid tumor patients, survival rates may not be very accurate. They also dont consider newer treatments being developed. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.
#27 Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy – PubMed
https://pubmed.ncbi.nlm.nih.gov/10458229/
Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. […] Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. […] Unresectable disease should be treated with definitive radiation therapy.
#28 A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC4096320/
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. […] Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival (LRFS) was 69%. […] A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. […] In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. […] The clinical course of desmoid fibromatosis varies widely; in some instances, the lesions remain stable for long periods with no intervention, while in others the tumors grow rapidly. […] A multivariate analysis demonstrated that age, tumor size, and tumor site are independent predictors of recurrence.
#29 Desmoid Tumor – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: Where the tumor is in your body, How fast the tumor grows, How much of the tumor was taken out during surgery. […] Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Given that there are so few desmoid tumor patients, survival rates may not be very accurate. They also dont consider newer treatments being developed. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.