Chondrosarcoma – Rokowania, prognozy i postęp choroby

Chondrosarcoma
Rokowania, prognozy i postęp choroby

Chondrosarcoma, stanowiący 10-15% pierwotnych nowotworów kości, to złośliwy guz tkanki chrzęstnej charakteryzujący się produkcją nieprawidłowej macierzy chrzęstnej. Rokowanie zależy głównie od stopnia histologicznego, lokalizacji, wielkości guza oraz stadium zaawansowania. Pięcioletnia przeżywalność wynosi ogólnie 79%, zróżnicowana w zależności od stadium: 91% dla choroby miejscowej, 76% dla regionalnej i 17% dla choroby z przerzutami. Stopień histologiczny jest kluczowym czynnikiem prognostycznym – dla stopnia I przeżywalność 5-letnia wynosi 90-95%, dla stopnia II 74-81%, a dla stopnia III tylko 29-31%. Lokalizacja w kręgosłupie i miednicy wiąże się z dwukrotnie gorszym rokowaniem (RR=2) w porównaniu do kończyn, a guzy >10 cm korelują z gorszym przeżyciem. W przypadku zdedyferencjonowanego chondrosarcoma (DCCS) kończyn, wielkość >8 cm oraz złamanie patologiczne są niezależnymi negatywnymi czynnikami prognostycznymi. Przerzuty znacząco pogarszają medianę całkowitego przeżycia (OS) do 14,0 ± 2,5 miesięcy, a 5-letnie przeżycie spada do 28,4%.

Prognostyka chondrosarcoma – wprowadzenie

Chondrosarcoma, będący drugim najczęstszym pierwotnym nowotworem kości, stanowi około 10-15% wszystkich pierwotnych nowotworów kostnych. Jest to złośliwy nowotwór pochodzący z tkanki chrzęstnej, charakteryzujący się produkcją nieprawidłowej macierzy chrzęstnej. ¹² Kliniczna prognoza wyników leczenia stanowi główne zmartwienie pacjentów z chorobą nowotworową, jednak w przypadku chondrosarcoma nie opracowano jeszcze dokładnych wskaźników predykcyjnych, pomimo że jest to drugi najczęstszy pierwotny nowotwór kości. ³ Większość chondrosarcomów rośnie powoli i rzadko daje przerzuty, a odpowiednie leczenie chirurgiczne prowadzi do dobrego rokowania, jednak w przypadku chondrosarcomów wysokiego stopnia złośliwości konieczna jest szeroka resekcja chirurgiczna ze względu na wysoką oporność tego guza na chemioterapię i radioterapię. ⁴

Wskaźniki przeżywalności w chondrosarcoma

Ogólnie rzecz biorąc, 79% pacjentów z chondrosarcoma przeżywa pięć lat po rozpoznaniu. Jednakże wskaźniki przeżywalności różnią się znacząco w zależności od lokalizacji guza i stopnia rozprzestrzenienia: ⁵

Choroba miejscowa (bez oznak rozprzestrzeniania się poza kość pierwotną): 91% przeżywalności 5-letniej
Choroba regionalna (rozprzestrzenianie się do okolicznych kości, tkanek, narządów lub węzłów chłonnych): 76% przeżywalności 5-letniej
Choroba odległa (przerzuty do płuc lub kości w innych częściach ciała): 17% przeżywalności 5-letniej

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Według danych Narodowego Instytutu Raka (NCI) ogólna 5-letnia przeżywalność dla mięsaków kości wynosi 66,9%. ⁷ Dla konwencjonalnego chondrosarcoma, wskaźniki przeżywalności są silnie powiązane ze stopniem złośliwości histologicznej: ⁸⁹

Stopień histologiczny	Wskaźnik przeżywalności 5-letniej	Wskaźnik przeżywalności 10-letniej	Ryzyko przerzutów
Stopień I	90-95%	Nie określono jednoznacznie	Wyjątkowe (praktycznie nie występują)
Stopień II	74-81%	62%	Około 10%
Stopień III	29-31%	26%	Do 70%

W przypadku pacjentów z chorobą zlokalizowaną (stadium I lub II), ogólna przeżywalność może przekraczać 80% w ciągu pięciu lat. Szczególnie dobre rokowanie dotyczy stadium I, gdzie szansa na przeżycie pięciu lat wynosi około 90-95%. ¹⁰

Czynniki prognostyczne w chondrosarcoma

Stopień histologiczny guza jest powszechnie uznawany za najważniejszy czynnik prognostyczny w chondrosarcoma. Wyższy stopień złośliwości wiąże się z gorszym rokowaniem i zwiększonym ryzykiem przerzutów. ¹¹¹²¹³ Stopień złośliwości określa się na podstawie atypii komórkowej, liczby mitoz i komórkowości. ¹⁴

Lokalizacja guza jest istotnym niezależnym czynnikiem prognostycznym. Guzy zlokalizowane w kręgosłupie i miednicy mają gorsze rokowanie (ryzyko względne RR = 2) w porównaniu do guzów kończyn. ¹⁵ Generalnie, chondrosarcoma głowy i szyi wiążą się z lepszą przeżywalnością niż chondrosarcoma zlokalizowane w innych miejscach. ¹⁶

Wielkość guza jest niezależnym predyktorem całkowitego przeżycia (OS) i specyficznego dla nowotworu przeżycia (CSS). Guzy o wielkości >10 cm wiążą się z gorszym rokowaniem. ¹⁷ W przypadku zdedyferencjonowanego centralnego chondrosarcoma (DCCS) kończyn, wielkość guza >8 cm była niezależnym czynnikiem prognostycznym gorszego całkowitego przeżycia. ¹⁸

Stadium zaawansowania według AJCC (American Joint Committee on Cancer) jest czynnikiem prognostycznym u pacjentów z chondrosarcoma kończyn, ale nie jest tak skuteczne u pacjentów z guzami szkieletu osiowego i miednicy. ¹⁹ W przypadku DCCS kończyn, stopień zaawansowania według Ennekinga okazał się niezależnym czynnikiem prognostycznym. ²⁰²¹

Przerzuty znacząco pogarszają rokowanie. Mediana OS i CSS pacjentów z przerzutami w momencie diagnozy wynosi odpowiednio 14,0 ± 2,5 miesięcy i 17,0 ± 2,6 miesięcy, co wskazuje na bardzo złe rokowanie. ²² 5-letnie i 10-letnie wskaźniki OS dla chondrosarcoma z przerzutami wynoszą odpowiednio 28,4% i 22,8%, a wskaźniki CSS wynoszą 31,2% i 26,6%. ²³

Złamanie patologiczne zostało zidentyfikowane jako niezależny czynnik prognostyczny gorszego całkowitego przeżycia i przeżycia wolnego od progresji w DCCS kończyn. ²⁴²⁵

Margines chirurgiczny jest istotnym czynnikiem prognostycznym. Negatywny margines chirurgiczny skutkuje mniejszą liczbą przerzutów odległych i poprawą wskaźników przeżywalności. ²⁶ W DCCS kończyn, margines chirurgiczny był niezależnym czynnikiem prognostycznym wpływającym na OS i PFS. ²⁷²⁸

Leczenie chirurgiczne istotnie wydłuża przeżycie pacjentów z chondrosarcoma z przerzutami. ²⁹ Jednocześnie warto podkreślić, że radioterapia nie wykazała korzyści w zakresie wydłużenia przeżycia w tej kohorcie. ³⁰

Chemioterapia w połączeniu z leczeniem chirurgicznym może być wiarygodną opcją terapeutyczną poprawiającą rokowanie pacjentów, szczególnie w przypadku chondrosarcoma wysokiego stopnia złośliwości. ³¹ Jednakże konwencjonalne i zdedyferencjonowane chondrosarcoma pozostają przede wszystkim chorobą chirurgiczną, która może odpowiadać na chemioterapię zawierającą doksorubicynę tylko w rzadkich przypadkach. ³²

Rokowanie w szczególnych podtypach chondrosarcoma

Zdedyferencjonowany chondrosarcoma

Zdedyferencjonowany chondrosarcoma (DCCS) ma szczególnie złe rokowanie. Mediana całkowitego przeżycia (OS) i przeżycia wolnego od progresji (PFS) wynosi odpowiednio 34 i 23 miesiące. ³³ Większość pacjentów umiera z powodu przerzutów odległych w ciągu 2 lat od początkowego rozpoznania, niezależnie od zastosowanych metod leczenia. ³⁴ Wskaźnik przeżywalności po 1 roku wynosi zaledwie 10%. ³⁵

Mezenchymalny chondrosarcoma

Mezenchymalny chondrosarcoma charakteryzuje się 5-letnim wskaźnikiem przeżywalności poniżej 50%, z ogólnym 10-letnim wskaźnikiem przeżywalności wynoszącym 28%. ³⁶ Mezenchymalne chondrosarcoma posiadają nawracające translokacje i są odrębnymi jednostkami patologicznymi, biologicznymi i klinicznymi. ³⁷

Clear cell chondrosarcoma

Chociaż clear cell chondrosarcoma są guzami o niskim stopniu złośliwości, mogą prowadzić do przerzutów odległych. Opisano późne nawroty (powyżej 10 lat). Ogólny wskaźnik nawrotów wynosi 16%. ³⁸

Przykortykowy chondrosarcoma

Przykortykowe (juxtacortical) chondrosarcoma są zmianami o niskim lub pośrednim stopniu złośliwości; rokowanie dla tych guzów jest lepsze niż dla innych typów. ³⁹

Biomarkery molekularne w prognozowaniu chondrosarcoma

Istnieje pilna potrzeba identyfikacji biomarkerów prognostycznych o wysokiej dokładności i swoistości, które pomogłyby określić najlepsze postępowanie kliniczne, agresywność guza i rokowanie u pacjentów z chondrosarcoma. ⁴⁰

Mutacje IDH

Heterozygotyczne mutacje dehydrogenazy izocytrynianowej (IDH) występują w około połowie konwencjonalnych centralnych chondrosarcomów kości (CCBC). Badania wykazały wyższą częstość mutacji IDH w guzach G3 (69%) w porównaniu do G2 (37%). Nie zaobserwowano jednak istotnych różnic w całkowitym przeżyciu (OS), przeżyciu wolnym od nawrotu (RFS) i przeżyciu wolnym od progresji (PFS) w zależności od statusu mutacyjnego IDH. ⁴¹⁴²

Mutacje TP53

Badanie przeprowadzone w MD Anderson wykazało, że pacjenci z konwencjonalnym i zdedyferencjonowanym chondrosarcoma, u których występuje mutacja genu TP53, mają gorsze rokowanie. Przez lata jedynym czynnikiem prognostycznym był stopień guza, ale badania udowodniły, że mutacja TP53 może również pomóc w określeniu rokowania. ⁴³

Inne biomarkery

Zwiększone stężenie CCL5 może być uznane za marker złego rokowania. ⁴⁴ Surwiwina może być potencjalnym nowym celem terapeutycznym dla chondrosarcoma i może służyć jako biomarker predykcyjny. ⁴⁵ Różnicowanie między enchondroma a chondrosarcoma niskiego stopnia złośliwości jest klinicznie istotniejsze niż stopniowanie chondrosarcoma ze względu na postępowanie kliniczne. ⁴⁶

Wpływ nawrotów i przerzutów na rokowanie

Zarówno rozwój nawrotu miejscowego, jak i rozwój przerzutów odległych prowadzą do znacznego spadku całkowitego przeżycia, niezależnie od stopnia złośliwości guza czy jego lokalizacji. ⁴⁷ Rokowanie staje się bardziej skomplikowane u pacjentów, u których rozwija się choroba nawrotowa. ⁴⁸

Im wyższy stopień złośliwości, tym większa szansa na nawrót chondrosarcoma. Guzy o niskim stopniu złośliwości rzadziej nawracają, a gdy to robią, często rosną ponownie jako guzy o niskim stopniu złośliwości. Jednak czasami sytuacja może się zmienić i z czasem guz o niskim stopniu złośliwości może przekształcić się i powrócić w bardziej agresywnej formie. ⁴⁹

Wskaźnik przerzutów dla pierwotnego chondrosarcoma jest wyższy niż dla wtórnego chondrosarcoma, a wskaźnik przerzutów odległych jest wyższy u pacjentów z nawrotem miejscowym niż u pacjentów bez nawrotu miejscowego. ⁵⁰ Najczęstszym miejscem przerzutów są płuca. Nawroty zwykle pojawiają się 5-10 lat lub dłużej po operacji. ⁵¹

Wykorzystanie uczenia maszynowego w prognozowaniu chondrosarcoma

Zastosowanie algorytmów uczenia maszynowego pomogło określić wzór, który dokładnie klasyfikuje pacjentów z chondrosarcoma do grup wysokiego i niskiego ryzyka według prawdopodobieństwa wystąpienia przerzutów odległych (DM). ⁵² Model XGBoost (o najlepszej wydajności predykcyjnej) sugerował, że wiek odgrywał nadzwyczajną rolę w DM chondrosarcoma. ⁵³

Badania potwierdziły, że podtyp chondrosarcoma, stopień zaawansowania T i N, obustronność oraz zastosowanie leczenia chirurgicznego i chemioterapii były niezależnymi czynnikami ryzyka DM. ⁵⁴ Rygorystyczna obserwacja jest zdecydowanie zalecana dla pacjentów z zaawansowanym stopniem patologicznym, zaawansowanym stopniem T i N, obustronnością oraz brakiem leczenia chirurgicznego i chemioterapii ze względu na wysokie ryzyko przerzutów. ⁵⁵

Rokowanie w szczególnych populacjach pacjentów

U dzieci i młodzieży chondrosarcoma kości ma bardzo dobre rokowanie i jest mniej agresywny niż u starszych pacjentów. ⁵⁶

Nie wszystkie choroby w stadium IV są takie same. Niektórzy pacjenci mogą mieć nawrót z 50 guzami, a niektórzy mogą mieć nawrót tylko z jednym guzem. Jednak jeśli znajduje się on w innym narządzie, jest klasyfikowany jako stadium IV. ⁵⁷ W przypadku pacjentów z nawrotem choroby z jednym lub dwoma zmianami w płucu, istnieje potencjalna możliwość wyleczenia. W przypadku pacjentów z nawrotem i bardziej zaawansowaną chorobą, wyleczenie może nie być możliwe, a celem staje się utrzymanie pacjentów przy życiu tak długo, jak to możliwe, przy zachowanej jakości życia. ⁵⁸

Podsumowanie czynników prognostycznych

Główne czynniki wpływające na rokowanie w chondrosarcoma obejmują: ⁵⁹

Stopień histologiczny – najsilniejszy niezależny czynnik prognostyczny
Lokalizacja guza – guzy szkieletu osiowego i miednicy mają gorsze rokowanie
Wielkość guza – większe guzy wiążą się z gorszym rokowaniem
Stadium zaawansowania – wyższe stadium wiąże się z gorszym rokowaniem
Obecność przerzutów – znacząco pogarsza rokowanie
Wiek pacjenta – młodszy wiek wiąże się z lepszym rokowaniem
Typ histologiczny – różne podtypy mają odmienne rokowanie
Margines chirurgiczny – negatywny margines poprawia rokowanie
Złamanie patologiczne – wiąże się z gorszym rokowaniem
Mutacje molekularne – np. mutacja TP53 wiąże się z gorszym rokowaniem
Metoda leczenia – leczenie chirurgiczne pozostaje najważniejszym czynnikiem wpływającym na poprawę rokowania

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Materiały źródłowe

#1 Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options
https://www.mdpi.com/1422-0067/24/2/1361
Chondrosarcomas (CS) are rare mesenchymal neoplasms that are defined by the production of an abnormal cartilaginous matrix. They define a diverse group of sarcomas with varied morphological features and different clinical behaviors. CSs account for 25% of primary bone tumors, with about 150 new diagnosed cases per year in France. […] The prognosis of patients with dedifferentiated chondrosarcoma is uniformly poor, whatever the treatments used. Most patients die of distant metastases within 2 years of the initial diagnosis. […] Conventional, dedifferentiated, and mesenchymal CS are rare bone sarcomas with a very poor prognosis. Mesenchymal chondrosarcomas harbor recurrent translocations and are distinct pathological, biological, and clinical entities. […] Conventional chemotherapies fail to improve the survival of conventional and dedifferentiated CS patients. Conventional and dedifferentiated CS remains a primarily surgical disease at present which may respond to doxorubicin-containing chemotherapy in only rare cases.
#2 Treatment method and prognostic factors of chondrosarcoma: based on Surveillance, Epidemiology, and End Results (SEER) database – Hua – Translational Cancer Research
https://tcr.amegroups.org/article/view/41253/html
Chondrosarcoma is a malignant tumor originating from cartilage tissue. It is the second most malignant bone tumor, accounting for about 10% to 15% of all primary bone tumors. […] This study found that although non-surgical treatment of chondrosarcoma has made great progress, at present, it is still not considered that non-surgical treatment can significantly improve the prognosis. There are many factors affecting the prognosis of chondrosarcoma, including age, primary site, histological type, grade, tumor size, distant metastasis and treatment method. […] The results showed age, primary site, histological type, grade, tumor size, metastasis, and treatment method were independent factors affecting ACM and CSSM.
#3 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
Clinical outcome prediction is major concern to patients with cancer. […] However, accurate predictors in chondrosarcoma have not been developed, even though chondrosarcoma is the second most common primary bone tumour. […] The majority of chondrosarcoma grows slowly and rarely metastasises, and adequate surgery leads to a good prognosis. […] However, wide surgical excision is acquired in high-grade chondrosarcoma, because this tumour is highly resistant to chemotherapy and radiotherapy. […] Therefore, there is an urgent need to identify prognostic biomarkers with high accuracy and specificity to determine best clinical management, tumour aggressiveness and disease prognosis in patients. […] Increased CCL5 can be considered a marker of poor prognosis. […] Survivin could be a possible new therapeutic target for chondrosarcoma and could serve as a predictive biomarker.
#4 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
Clinical outcome prediction is major concern to patients with cancer. […] However, accurate predictors in chondrosarcoma have not been developed, even though chondrosarcoma is the second most common primary bone tumour. […] The majority of chondrosarcoma grows slowly and rarely metastasises, and adequate surgery leads to a good prognosis. […] However, wide surgical excision is acquired in high-grade chondrosarcoma, because this tumour is highly resistant to chemotherapy and radiotherapy. […] Therefore, there is an urgent need to identify prognostic biomarkers with high accuracy and specificity to determine best clinical management, tumour aggressiveness and disease prognosis in patients. […] Increased CCL5 can be considered a marker of poor prognosis. […] Survivin could be a possible new therapeutic target for chondrosarcoma and could serve as a predictive biomarker.
#5 Chondrosarcoma: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/22112-chondrosarcoma
Overall, 79% of people with chondrosarcoma are alive five years after diagnosis. […] Survival rates for chondrosarcoma vary depending on the tumor type and whether the tumor is spreading to other parts of your body. Overall, 79% of people with chondrosarcoma were alive five years after diagnosis. Here are specific survival rates based on tumor location: Local: There’s no sign of spreading from the bone where it started. 91% […] Regional: The tumor is spreading from your bone into nearby bones, tissues, organs or lymph nodes. 76% […] Distant: The tumor is in your lungs or in bones in other parts of your body. 17%. […] A survival rate is an estimate, not a prediction. They’re estimates based on the experiences of other people, and your situation may be very different. […] Survival rate estimates reflect what happened in the past. For example, the survival rates listed above are based on what happened to people with chondrosarcoma between 2012 and 2018. […] Survival rates don’t indicate how long you’ll live.
#6 Chondrosarcoma: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/22112-chondrosarcoma
Overall, 79% of people with chondrosarcoma are alive five years after diagnosis. […] Survival rates for chondrosarcoma vary depending on the tumor type and whether the tumor is spreading to other parts of your body. Overall, 79% of people with chondrosarcoma were alive five years after diagnosis. Here are specific survival rates based on tumor location: Local: There’s no sign of spreading from the bone where it started. 91% […] Regional: The tumor is spreading from your bone into nearby bones, tissues, organs or lymph nodes. 76% […] Distant: The tumor is in your lungs or in bones in other parts of your body. 17%. […] A survival rate is an estimate, not a prediction. They’re estimates based on the experiences of other people, and your situation may be very different. […] Survival rate estimates reflect what happened in the past. For example, the survival rates listed above are based on what happened to people with chondrosarcoma between 2012 and 2018. […] Survival rates don’t indicate how long you’ll live.
#7 Sarcoma – Wikipedia
https://en.wikipedia.org/wiki/Sarcoma
The AJCC has identified several factors that affect prognosis of bone sarcomas: […] For soft-tissue sarcomas other than GISTs, factors that affect prognosis include: […] For GISTs, the key factor that affects prognosis is: […] According to data published by the US National Cancer Institute (NCI), the overall 5-year survival for bone sarcomas is 66.9%. […] For soft-tissue sarcomas, the overall 5-year survival (irrespective of stage) is 64.5%, but survival is affected by many factors, including stage.
#8 Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options
https://www.mdpi.com/1422-0067/24/2/1361
The reported 5-year overall survival rate is 74% and 31% for grade 2 CS and grade 3 CS, respectively. The 10-year overall survival rate is 62% for grade 2 and 26% for grade 3 tumors. A high grade (2 or 3) was highly related to the increased risk of metastatic disease (29.3% at 5 years vs. 4.6% for Grade 1 tumors; p = 0.0001, log-rank test) in a Mayo Clinic descriptive cohort of 344 primary conventional CS patients. […] Understanding the different histological subtypes of CS is helpful in predicting biological behavior.
#9 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#10 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
How chondrosarcoma will affect you long-term depends on many factors, including the: […] Each patient is unique, so its important to discuss your prognosis with your doctor. […] Its more common to see patients with localized disease, which can be stage I or stage II, says Conley. Overall survival for these patients can exceed 80% at five years, depending on the situation. And if you have stage I chondrosarcoma, your chance of surviving five years is going to be closer to 90% to 95%. […] Prognosis becomes a bit trickier for patients who develop recurrent disease. […] The higher the grade, the higher the chance the chondrosarcoma will recur, says Bird. Low-grade tumors are less likely to recur, and when they do, they often grow back as low-grade. But sometimes things can change, and over time a low-grade tumor can transform and come back more aggressive.
#11 Survival and prognostic factors in chondrosarcoma
https://pmc.ncbi.nlm.nih.gov/articles/PMC3247897/
There have been few long-term studies on the outcome of chondrosarcoma and the findings regarding prognostic factors are controversial. […] Tumor grade and localization were found to be statistically significant independent predictors of disease-related deaths in multivariate analysis. […] The AJCC staging system was able to predict prognosis in patients with chondrosarcoma of the extremities, but not in those with tumors of the axial skeleton and pelvis. […] The outcome in patients with primary central chondrosarcoma of bone who present with localized disease is mostly affected by tumor-related parameters. […] Both the development of local recurrence and the development of distant metastases led to a significant decline in overall survival, regardless of tumor grade or localization.
#12 Survival and prognostic factors in chondrosarcoma
https://pmc.ncbi.nlm.nih.gov/articles/PMC3247897/
Significant independent predictors of death were high grade (relative risk (RR) = 5, 95% CI: 212; p 0.001) and a tumor localization in the axial skeleton and pelvis (RR = 2, 95% CI: 14; p = 0.04). […] In conclusion, the outcome of patients with primary central chondrosarcoma of bone who present with localized disease is mostly affected by tumor- and patient-related parameters.
#13 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Chondrosarcoma with metastatic disease has a very poor prognosis. […] The overall survival (OS) and cancer-survival specific (CSS) rates of the entire group at 5 years were 28.4% and 31.2%, respectively. […] Among patients with primary chondrosarcoma of bone and metastasis at presentation, low tumor grade, surgical treatment, tumor size 10 cm, and first primary tumor predict prolonged survival. […] Patients with chondrosarcoma and metastasis often have a poor prognosis. […] The 5- and 10-year OS rates of the metastatic chondrosarcoma were 28.4% and 22.8%, respectively, while the 5- and 10-year CSS rates were 31.2% and 26.6%. […] The median OS and CSS of our cohort were 14.0 2.5 months and 17.0 2.6 months, respectively, suggesting a pretty poor prognosis. […] Tumor grade was usually recognized as a very important predictor of chondrosarcoma.
#14 IDH mutations in G2-3 conventional central bone chondrosarcoma: a mono institutional experience | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-11396-y
Tumor grade is the main prognostic factor. It is defined by cellular atypia, mitoses, and cellularity. Metastasis occurs exceptionally in grade 1 lesions, 10% in grade 2, but up to 70% in grade III lesions. The 5-year overall survival (OS) is 80-90% for grade I but drops to about 50-60% for grade II-III conventional chondrosarcoma. […] The impact of IDH mutation on chondrosarcoma prognosis is still unclear. Stage, grade, size, and surgical margins are the main factors to consider for survival prediction in CCBC. In our series grade and stage were confirmed to be prognostic, but not the mutational status. The 5-year OS was 51%, but dropped to 7% in metastatic patients, and to 29% in G3 patients, but did not differ significantly by mutational status. The 5-year RFS was worse in IDH mut localized patients, but not statistically significant.
#15 Survival and prognostic factors in chondrosarcoma
https://pmc.ncbi.nlm.nih.gov/articles/PMC3247897/
Significant independent predictors of death were high grade (relative risk (RR) = 5, 95% CI: 212; p 0.001) and a tumor localization in the axial skeleton and pelvis (RR = 2, 95% CI: 14; p = 0.04). […] In conclusion, the outcome of patients with primary central chondrosarcoma of bone who present with localized disease is mostly affected by tumor- and patient-related parameters.
#16 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
The prognosis is correlated with the grade and stage of the lesion at the time of diagnosis. The location of the lesion is also important because tumors in areas where complete wide resection is possible are associated with better prognoses. In general, chondrosarcomas of the head and neck are associated with better disease-specific survival and overall survival rates than chondrosarcomas located elsewhere. […] Recurrence and distant metastasis may develop. The metastasis rate for primary chondrosarcoma is higher than that for secondary chondrosarcoma, and the rate of distant metastasis is higher in patients with local recurrence than in those without local recurrence. […] In children and adolescents, chondrosarcoma of bone has a very good prognosis and is less aggressive than in older patients.
#17 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Therefore, tumor grade is an important prognostic factor for chondrosarcoma patients with metastasis, whether at presentation or otherwise. […] Surgical resection of primary tumors significantly prolonged the survival of metastatic chondrosarcoma patients. […] The median OS and CSS of patients receiving radiotherapy tended to be lower than those receiving no radiotherapy, suggesting that radiation treatment was not beneficial for prolonging survival in this cohort. […] Independent predictors of OS include tumor grade, tumor size, surgery and tumor sequence. Tumor grade, tumor size, and surgery were calculated as independent predictors of CSS.
#18 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
Univariate analysis indicated that metastasis (OS p=0.007, PFS p=0.005), pathological fracture (OS p=0.010, PFS p=0.005), Enneking staging (OS p<0.001, PFS p<0.001) and surgical margin (OS p=0.025, PFS p=0.001) were associated with worse overall survival and progression-free survival in extremity dedifferentiated central chondrosarcomas. [...] Multivariate analysis showed that metastasis (OS p=0.002, PFS p=0.005), pathological fracture (OS p=0.001, PFS p=0.012), Enneking staging (OS p=0.036), surgical margin (OS p=0.025, PFS p=0.011) and tumor size >8 cm (OS p=0.046) were independent prognostic factors of worse overall survival and progression-free survival. […] The former studies reported a 5-year overall survival rate ranging from 6 to 24%. […] In our study, we reported the largest extremity DCCS cohort in Asian and found extremity DCCS still had a grave prognosis.
#19 Survival and prognostic factors in chondrosarcoma
https://pmc.ncbi.nlm.nih.gov/articles/PMC3247897/
There have been few long-term studies on the outcome of chondrosarcoma and the findings regarding prognostic factors are controversial. […] Tumor grade and localization were found to be statistically significant independent predictors of disease-related deaths in multivariate analysis. […] The AJCC staging system was able to predict prognosis in patients with chondrosarcoma of the extremities, but not in those with tumors of the axial skeleton and pelvis. […] The outcome in patients with primary central chondrosarcoma of bone who present with localized disease is mostly affected by tumor-related parameters. […] Both the development of local recurrence and the development of distant metastases led to a significant decline in overall survival, regardless of tumor grade or localization.
#20 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
This study was to analyze the clinical outcomes and prognostic factors of dedifferentiated central chondrosarcomas (DCCS) in extremities. […] The median overall survival (OS) and progression-free survival (PFS) were 34 and 23 months, respectively. […] Multiple variate analyses indicated metastasis, pathological fracture, Enneking staging and surgical margin were independent prognostic factors in extremity dedifferentiated central chondrosarcomas. […] Dedifferentiated central chondrosarcomas in extremities still had a grave prognosis. Metastasis, pathological fracture, Enneking staging, and surgical margin were independent risk factors for prognosis. […] The 2-year overall survival rate and progression-free survival rate were 100.0% and 100.0% in the good response group and 69.2% and 53.8% in the bad response group, respectively.
#21 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
Univariate analysis indicated that metastasis (OS p=0.007, PFS p=0.005), pathological fracture (OS p=0.010, PFS p=0.005), Enneking staging (OS p<0.001, PFS p<0.001) and surgical margin (OS p=0.025, PFS p=0.001) were associated with worse overall survival and progression-free survival in extremity dedifferentiated central chondrosarcomas. [...] Multivariate analysis showed that metastasis (OS p=0.002, PFS p=0.005), pathological fracture (OS p=0.001, PFS p=0.012), Enneking staging (OS p=0.036), surgical margin (OS p=0.025, PFS p=0.011) and tumor size >8 cm (OS p=0.046) were independent prognostic factors of worse overall survival and progression-free survival. […] The former studies reported a 5-year overall survival rate ranging from 6 to 24%. […] In our study, we reported the largest extremity DCCS cohort in Asian and found extremity DCCS still had a grave prognosis.
#22 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Chondrosarcoma with metastatic disease has a very poor prognosis. […] The overall survival (OS) and cancer-survival specific (CSS) rates of the entire group at 5 years were 28.4% and 31.2%, respectively. […] Among patients with primary chondrosarcoma of bone and metastasis at presentation, low tumor grade, surgical treatment, tumor size 10 cm, and first primary tumor predict prolonged survival. […] Patients with chondrosarcoma and metastasis often have a poor prognosis. […] The 5- and 10-year OS rates of the metastatic chondrosarcoma were 28.4% and 22.8%, respectively, while the 5- and 10-year CSS rates were 31.2% and 26.6%. […] The median OS and CSS of our cohort were 14.0 2.5 months and 17.0 2.6 months, respectively, suggesting a pretty poor prognosis. […] Tumor grade was usually recognized as a very important predictor of chondrosarcoma.
#23 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Chondrosarcoma with metastatic disease has a very poor prognosis. […] The overall survival (OS) and cancer-survival specific (CSS) rates of the entire group at 5 years were 28.4% and 31.2%, respectively. […] Among patients with primary chondrosarcoma of bone and metastasis at presentation, low tumor grade, surgical treatment, tumor size 10 cm, and first primary tumor predict prolonged survival. […] Patients with chondrosarcoma and metastasis often have a poor prognosis. […] The 5- and 10-year OS rates of the metastatic chondrosarcoma were 28.4% and 22.8%, respectively, while the 5- and 10-year CSS rates were 31.2% and 26.6%. […] The median OS and CSS of our cohort were 14.0 2.5 months and 17.0 2.6 months, respectively, suggesting a pretty poor prognosis. […] Tumor grade was usually recognized as a very important predictor of chondrosarcoma.
#24 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
This study was to analyze the clinical outcomes and prognostic factors of dedifferentiated central chondrosarcomas (DCCS) in extremities. […] The median overall survival (OS) and progression-free survival (PFS) were 34 and 23 months, respectively. […] Multiple variate analyses indicated metastasis, pathological fracture, Enneking staging and surgical margin were independent prognostic factors in extremity dedifferentiated central chondrosarcomas. […] Dedifferentiated central chondrosarcomas in extremities still had a grave prognosis. Metastasis, pathological fracture, Enneking staging, and surgical margin were independent risk factors for prognosis. […] The 2-year overall survival rate and progression-free survival rate were 100.0% and 100.0% in the good response group and 69.2% and 53.8% in the bad response group, respectively.
#25 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
Univariate analysis indicated that metastasis (OS p=0.007, PFS p=0.005), pathological fracture (OS p=0.010, PFS p=0.005), Enneking staging (OS p<0.001, PFS p<0.001) and surgical margin (OS p=0.025, PFS p=0.001) were associated with worse overall survival and progression-free survival in extremity dedifferentiated central chondrosarcomas. [...] Multivariate analysis showed that metastasis (OS p=0.002, PFS p=0.005), pathological fracture (OS p=0.001, PFS p=0.012), Enneking staging (OS p=0.036), surgical margin (OS p=0.025, PFS p=0.011) and tumor size >8 cm (OS p=0.046) were independent prognostic factors of worse overall survival and progression-free survival. […] The former studies reported a 5-year overall survival rate ranging from 6 to 24%. […] In our study, we reported the largest extremity DCCS cohort in Asian and found extremity DCCS still had a grave prognosis.
#26 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The XGB model also occupied the largest area in the ROC of the external validation group, indicating that it was still the most efficient equation. […] Our study found that high histologic grades was a key factor in DM and revealed statistical differences between the two populations. […] Negative margin surgery resulted in fewer DM and improved survival rates. […] The XGB model (best predictive performance) suggested that age played an extraordinary role in DM of CS. […] In summary, the use of machine learning algorithms helped determine a formula that accurately classified CS patients into high-risk and low-risk groups according to the probability of DM. […] In conclusion, rigorous follow-up was strongly recommended for patients with advanced pathological grade, advanced T-stage and N-stage, laterality and failure to undergo surgery and chemotherapy, due to the high risk of metastasis.
#27 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
This study was to analyze the clinical outcomes and prognostic factors of dedifferentiated central chondrosarcomas (DCCS) in extremities. […] The median overall survival (OS) and progression-free survival (PFS) were 34 and 23 months, respectively. […] Multiple variate analyses indicated metastasis, pathological fracture, Enneking staging and surgical margin were independent prognostic factors in extremity dedifferentiated central chondrosarcomas. […] Dedifferentiated central chondrosarcomas in extremities still had a grave prognosis. Metastasis, pathological fracture, Enneking staging, and surgical margin were independent risk factors for prognosis. […] The 2-year overall survival rate and progression-free survival rate were 100.0% and 100.0% in the good response group and 69.2% and 53.8% in the bad response group, respectively.
#28 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
Univariate analysis indicated that metastasis (OS p=0.007, PFS p=0.005), pathological fracture (OS p=0.010, PFS p=0.005), Enneking staging (OS p<0.001, PFS p<0.001) and surgical margin (OS p=0.025, PFS p=0.001) were associated with worse overall survival and progression-free survival in extremity dedifferentiated central chondrosarcomas. [...] Multivariate analysis showed that metastasis (OS p=0.002, PFS p=0.005), pathological fracture (OS p=0.001, PFS p=0.012), Enneking staging (OS p=0.036), surgical margin (OS p=0.025, PFS p=0.011) and tumor size >8 cm (OS p=0.046) were independent prognostic factors of worse overall survival and progression-free survival. […] The former studies reported a 5-year overall survival rate ranging from 6 to 24%. […] In our study, we reported the largest extremity DCCS cohort in Asian and found extremity DCCS still had a grave prognosis.
#29 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Therefore, tumor grade is an important prognostic factor for chondrosarcoma patients with metastasis, whether at presentation or otherwise. […] Surgical resection of primary tumors significantly prolonged the survival of metastatic chondrosarcoma patients. […] The median OS and CSS of patients receiving radiotherapy tended to be lower than those receiving no radiotherapy, suggesting that radiation treatment was not beneficial for prolonging survival in this cohort. […] Independent predictors of OS include tumor grade, tumor size, surgery and tumor sequence. Tumor grade, tumor size, and surgery were calculated as independent predictors of CSS.
#30 Predictors of the survival of patients with chondrosarcoma of bone and metastatic disease at diagnosis
https://www.jcancer.org/v10p2457.htm
Therefore, tumor grade is an important prognostic factor for chondrosarcoma patients with metastasis, whether at presentation or otherwise. […] Surgical resection of primary tumors significantly prolonged the survival of metastatic chondrosarcoma patients. […] The median OS and CSS of patients receiving radiotherapy tended to be lower than those receiving no radiotherapy, suggesting that radiation treatment was not beneficial for prolonging survival in this cohort. […] Independent predictors of OS include tumor grade, tumor size, surgery and tumor sequence. Tumor grade, tumor size, and surgery were calculated as independent predictors of CSS.
#31 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The occurrence of distant metastases (DM) limits the overall survival (OS) of patients with chondrosarcoma (CS). […] Identifying the risk of distal metastasis in patients with chondrosarcoma is very important as it affects the patients treatment plan and prognosis. […] Previous studies have concluded that the combination of surgery and chemotherapy is a reliable treatment option to improve patient prognosis. […] In this study, combined with adequate SEER case database and external validation with data from multicenter institutions in different geographic regions, we confirmed that CS, T, N, laterality, and grading of surgery and chemotherapy were independent risk factors for DM. […] The ROC curves assessed the predictive ability of the six ML algorithm models in the validation group.
#32 Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options
https://www.mdpi.com/1422-0067/24/2/1361
Chondrosarcomas (CS) are rare mesenchymal neoplasms that are defined by the production of an abnormal cartilaginous matrix. They define a diverse group of sarcomas with varied morphological features and different clinical behaviors. CSs account for 25% of primary bone tumors, with about 150 new diagnosed cases per year in France. […] The prognosis of patients with dedifferentiated chondrosarcoma is uniformly poor, whatever the treatments used. Most patients die of distant metastases within 2 years of the initial diagnosis. […] Conventional, dedifferentiated, and mesenchymal CS are rare bone sarcomas with a very poor prognosis. Mesenchymal chondrosarcomas harbor recurrent translocations and are distinct pathological, biological, and clinical entities. […] Conventional chemotherapies fail to improve the survival of conventional and dedifferentiated CS patients. Conventional and dedifferentiated CS remains a primarily surgical disease at present which may respond to doxorubicin-containing chemotherapy in only rare cases.
#33 The clinical outcomes and prognostic factors of dedifferentiated central chondrosarcoma in extremities | Journal of Orthopaedic Surgery and Research | Full Text
https://josr-online.biomedcentral.com/articles/10.1186/s13018-024-05098-9
This study was to analyze the clinical outcomes and prognostic factors of dedifferentiated central chondrosarcomas (DCCS) in extremities. […] The median overall survival (OS) and progression-free survival (PFS) were 34 and 23 months, respectively. […] Multiple variate analyses indicated metastasis, pathological fracture, Enneking staging and surgical margin were independent prognostic factors in extremity dedifferentiated central chondrosarcomas. […] Dedifferentiated central chondrosarcomas in extremities still had a grave prognosis. Metastasis, pathological fracture, Enneking staging, and surgical margin were independent risk factors for prognosis. […] The 2-year overall survival rate and progression-free survival rate were 100.0% and 100.0% in the good response group and 69.2% and 53.8% in the bad response group, respectively.
#34 Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options
https://www.mdpi.com/1422-0067/24/2/1361
Chondrosarcomas (CS) are rare mesenchymal neoplasms that are defined by the production of an abnormal cartilaginous matrix. They define a diverse group of sarcomas with varied morphological features and different clinical behaviors. CSs account for 25% of primary bone tumors, with about 150 new diagnosed cases per year in France. […] The prognosis of patients with dedifferentiated chondrosarcoma is uniformly poor, whatever the treatments used. Most patients die of distant metastases within 2 years of the initial diagnosis. […] Conventional, dedifferentiated, and mesenchymal CS are rare bone sarcomas with a very poor prognosis. Mesenchymal chondrosarcomas harbor recurrent translocations and are distinct pathological, biological, and clinical entities. […] Conventional chemotherapies fail to improve the survival of conventional and dedifferentiated CS patients. Conventional and dedifferentiated CS remains a primarily surgical disease at present which may respond to doxorubicin-containing chemotherapy in only rare cases.
#35 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#36 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#37 Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options
https://www.mdpi.com/1422-0067/24/2/1361
Chondrosarcomas (CS) are rare mesenchymal neoplasms that are defined by the production of an abnormal cartilaginous matrix. They define a diverse group of sarcomas with varied morphological features and different clinical behaviors. CSs account for 25% of primary bone tumors, with about 150 new diagnosed cases per year in France. […] The prognosis of patients with dedifferentiated chondrosarcoma is uniformly poor, whatever the treatments used. Most patients die of distant metastases within 2 years of the initial diagnosis. […] Conventional, dedifferentiated, and mesenchymal CS are rare bone sarcomas with a very poor prognosis. Mesenchymal chondrosarcomas harbor recurrent translocations and are distinct pathological, biological, and clinical entities. […] Conventional chemotherapies fail to improve the survival of conventional and dedifferentiated CS patients. Conventional and dedifferentiated CS remains a primarily surgical disease at present which may respond to doxorubicin-containing chemotherapy in only rare cases.
#38 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#39 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#40 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
Clinical outcome prediction is major concern to patients with cancer. […] However, accurate predictors in chondrosarcoma have not been developed, even though chondrosarcoma is the second most common primary bone tumour. […] The majority of chondrosarcoma grows slowly and rarely metastasises, and adequate surgery leads to a good prognosis. […] However, wide surgical excision is acquired in high-grade chondrosarcoma, because this tumour is highly resistant to chemotherapy and radiotherapy. […] Therefore, there is an urgent need to identify prognostic biomarkers with high accuracy and specificity to determine best clinical management, tumour aggressiveness and disease prognosis in patients. […] Increased CCL5 can be considered a marker of poor prognosis. […] Survivin could be a possible new therapeutic target for chondrosarcoma and could serve as a predictive biomarker.
#41 IDH mutations in G2-3 conventional central bone chondrosarcoma: a mono institutional experience | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-11396-y
Heterozygous isocitrate dehydrogenase (IDH) mutations occur in about half of conventional central bone chondrosarcomas (CCBC). Aim of this study was to assess the frequency and prognostic impact of IDH mutations in high grade CCBC patients. […] The frequency of mutations was 37% in G2 vs. 69% in G3 (p=0.039), and 100% in three Ollier disease associated chondrosarcoma. 5-year overall survival (OS) at 124 months (range 1-166) was 51%, with no significant difference based on the IDH mutational status: 61% in IDH mut vs. 44% in IDH wild type (IDH wt). The 5-year relapse free survival (RFS) was 33% (95% CI:1057) for IDH mut vs. 57% (95% CI: 3077) for IDH wt. Progression free survival (PFS) was 25% (95% CI:165) IDH mut vs. 16% (95% CI: 0.752) IDH wt. […] This study shows a higher frequency of IDH mutations in G3 CCBC as compared with G2. No significant differences in OS, RFS, and PFS by mutational status were detected. After relapse, a higher rate of G3 for IDH mutated CCBC was observed.
#42 IDH mutations in G2-3 conventional central bone chondrosarcoma: a mono institutional experience | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-11396-y
No different clinical characteristics were found between IDH wt and IDH mut high grade CCBC, except a higher frequency of mutations in syndromic patients and in G3 lesions. No statistically significant difference in prognosis was detected between IDH wt and IDH mut. A different trend in histologic evolution was detected, with IDH mut G2 tumors having a higher rate of grade progression at relapse, as compared to IDH wt.
#43 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
Not all stage IV disease is the same. […] Some people can relapse with 50 lumps, and some may relapse with only one lump. But, if its in a different organ, its classified as stage IV, explains Conley. The most common site of metastasis is the lung. If I have a relapsed patient with one or two spots on their lung, I believe we can potentially cure them. For relapsed patients with more advanced disease, curing the disease may not be possible. In those cases, our goal is to keep patients alive as long as possible with a preserved quality of life. […] An MD Anderson study published in 2023 found that patients with conventional and dedifferentiated chondrosarcoma who have the TP53 gene mutation have a poorer prognosis. […] For years, the only prognostic factor was the grade of the tumor, says Conley. But what weve proven through research is that the TP53 mutation can help determine prognosis as well. This helps us counsel patients during treatment.
#44 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
Clinical outcome prediction is major concern to patients with cancer. […] However, accurate predictors in chondrosarcoma have not been developed, even though chondrosarcoma is the second most common primary bone tumour. […] The majority of chondrosarcoma grows slowly and rarely metastasises, and adequate surgery leads to a good prognosis. […] However, wide surgical excision is acquired in high-grade chondrosarcoma, because this tumour is highly resistant to chemotherapy and radiotherapy. […] Therefore, there is an urgent need to identify prognostic biomarkers with high accuracy and specificity to determine best clinical management, tumour aggressiveness and disease prognosis in patients. […] Increased CCL5 can be considered a marker of poor prognosis. […] Survivin could be a possible new therapeutic target for chondrosarcoma and could serve as a predictive biomarker.
#45 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
Clinical outcome prediction is major concern to patients with cancer. […] However, accurate predictors in chondrosarcoma have not been developed, even though chondrosarcoma is the second most common primary bone tumour. […] The majority of chondrosarcoma grows slowly and rarely metastasises, and adequate surgery leads to a good prognosis. […] However, wide surgical excision is acquired in high-grade chondrosarcoma, because this tumour is highly resistant to chemotherapy and radiotherapy. […] Therefore, there is an urgent need to identify prognostic biomarkers with high accuracy and specificity to determine best clinical management, tumour aggressiveness and disease prognosis in patients. […] Increased CCL5 can be considered a marker of poor prognosis. […] Survivin could be a possible new therapeutic target for chondrosarcoma and could serve as a predictive biomarker.
#46 Biomarkers of chondrosarcoma | Journal of Clinical Pathology
https://jcp.bmj.com/content/71/7/579
The differential diagnosis between enchondroma and low-grade chondrosarcoma is more important than chondrosarcoma staging because of clinical management. […] This finding promises the possibility for a specific differential diagnostic marker. Further studies on the role of periostin in chondrosarcoma may lead to new knowledge of sarcoma oncogenesis. […] In chondrosarcoma, nothing is reliable yet. […] Follow-up study for validation of candidates should be facilitated.
#47 Survival and prognostic factors in chondrosarcoma
https://pmc.ncbi.nlm.nih.gov/articles/PMC3247897/
There have been few long-term studies on the outcome of chondrosarcoma and the findings regarding prognostic factors are controversial. […] Tumor grade and localization were found to be statistically significant independent predictors of disease-related deaths in multivariate analysis. […] The AJCC staging system was able to predict prognosis in patients with chondrosarcoma of the extremities, but not in those with tumors of the axial skeleton and pelvis. […] The outcome in patients with primary central chondrosarcoma of bone who present with localized disease is mostly affected by tumor-related parameters. […] Both the development of local recurrence and the development of distant metastases led to a significant decline in overall survival, regardless of tumor grade or localization.
#48 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
How chondrosarcoma will affect you long-term depends on many factors, including the: […] Each patient is unique, so its important to discuss your prognosis with your doctor. […] Its more common to see patients with localized disease, which can be stage I or stage II, says Conley. Overall survival for these patients can exceed 80% at five years, depending on the situation. And if you have stage I chondrosarcoma, your chance of surviving five years is going to be closer to 90% to 95%. […] Prognosis becomes a bit trickier for patients who develop recurrent disease. […] The higher the grade, the higher the chance the chondrosarcoma will recur, says Bird. Low-grade tumors are less likely to recur, and when they do, they often grow back as low-grade. But sometimes things can change, and over time a low-grade tumor can transform and come back more aggressive.
#49 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
How chondrosarcoma will affect you long-term depends on many factors, including the: […] Each patient is unique, so its important to discuss your prognosis with your doctor. […] Its more common to see patients with localized disease, which can be stage I or stage II, says Conley. Overall survival for these patients can exceed 80% at five years, depending on the situation. And if you have stage I chondrosarcoma, your chance of surviving five years is going to be closer to 90% to 95%. […] Prognosis becomes a bit trickier for patients who develop recurrent disease. […] The higher the grade, the higher the chance the chondrosarcoma will recur, says Bird. Low-grade tumors are less likely to recur, and when they do, they often grow back as low-grade. But sometimes things can change, and over time a low-grade tumor can transform and come back more aggressive.
#50 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
The prognosis is correlated with the grade and stage of the lesion at the time of diagnosis. The location of the lesion is also important because tumors in areas where complete wide resection is possible are associated with better prognoses. In general, chondrosarcomas of the head and neck are associated with better disease-specific survival and overall survival rates than chondrosarcomas located elsewhere. […] Recurrence and distant metastasis may develop. The metastasis rate for primary chondrosarcoma is higher than that for secondary chondrosarcoma, and the rate of distant metastasis is higher in patients with local recurrence than in those without local recurrence. […] In children and adolescents, chondrosarcoma of bone has a very good prognosis and is less aggressive than in older patients.
#51 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
Evans et al showed that the survival rate depends on the histologic grade of the tumor, as follows: Grade I tumors – 90% survival at 5 years; Grade II tumors – 81% survival at 5 years; Grade III tumors – 29% survival at 5 years. […] Overall, the 5-year survival rate for conventional chondrosarcomas is 48-60%. […] Grade I tumors do not metastasize, whereas 66% of grade III tumors do. The most common sites for metastases are the lungs. Recurrences typically appear 5-10 years or longer after surgery. […] Dedifferentiated chondrosarcoma is highly lethal. It is associated with a 10% survival rate after 1 year. Even with early surgical treatment, disseminated hematogenous metastasis occurs in most patients. […] Although clear cell chondrosarcomas are low-grade tumors, they can lead to distant metastasis. Late recurrences (10 years) have been described. Overall, the recurrence rate is 16%. […] The 5-year survival rate for mesenchymal chondrosarcoma is less than 50%, with an overall 10-year survival rate of 28%. […] Juxtacortical chondrosarcomas are low- to intermediate-grade lesions; the prognosis for these tumors is better than that for other tumors.
#52 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The XGB model also occupied the largest area in the ROC of the external validation group, indicating that it was still the most efficient equation. […] Our study found that high histologic grades was a key factor in DM and revealed statistical differences between the two populations. […] Negative margin surgery resulted in fewer DM and improved survival rates. […] The XGB model (best predictive performance) suggested that age played an extraordinary role in DM of CS. […] In summary, the use of machine learning algorithms helped determine a formula that accurately classified CS patients into high-risk and low-risk groups according to the probability of DM. […] In conclusion, rigorous follow-up was strongly recommended for patients with advanced pathological grade, advanced T-stage and N-stage, laterality and failure to undergo surgery and chemotherapy, due to the high risk of metastasis.
#53 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The XGB model also occupied the largest area in the ROC of the external validation group, indicating that it was still the most efficient equation. […] Our study found that high histologic grades was a key factor in DM and revealed statistical differences between the two populations. […] Negative margin surgery resulted in fewer DM and improved survival rates. […] The XGB model (best predictive performance) suggested that age played an extraordinary role in DM of CS. […] In summary, the use of machine learning algorithms helped determine a formula that accurately classified CS patients into high-risk and low-risk groups according to the probability of DM. […] In conclusion, rigorous follow-up was strongly recommended for patients with advanced pathological grade, advanced T-stage and N-stage, laterality and failure to undergo surgery and chemotherapy, due to the high risk of metastasis.
#54 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The occurrence of distant metastases (DM) limits the overall survival (OS) of patients with chondrosarcoma (CS). […] Identifying the risk of distal metastasis in patients with chondrosarcoma is very important as it affects the patients treatment plan and prognosis. […] Previous studies have concluded that the combination of surgery and chemotherapy is a reliable treatment option to improve patient prognosis. […] In this study, combined with adequate SEER case database and external validation with data from multicenter institutions in different geographic regions, we confirmed that CS, T, N, laterality, and grading of surgery and chemotherapy were independent risk factors for DM. […] The ROC curves assessed the predictive ability of the six ML algorithm models in the validation group.
#55 A machine learning-based model for clinical prediction of distal metastasis in chondrosarcoma: a multicenter, retrospective study [PeerJ]
https://peerj.com/articles/16485/
The XGB model also occupied the largest area in the ROC of the external validation group, indicating that it was still the most efficient equation. […] Our study found that high histologic grades was a key factor in DM and revealed statistical differences between the two populations. […] Negative margin surgery resulted in fewer DM and improved survival rates. […] The XGB model (best predictive performance) suggested that age played an extraordinary role in DM of CS. […] In summary, the use of machine learning algorithms helped determine a formula that accurately classified CS patients into high-risk and low-risk groups according to the probability of DM. […] In conclusion, rigorous follow-up was strongly recommended for patients with advanced pathological grade, advanced T-stage and N-stage, laterality and failure to undergo surgery and chemotherapy, due to the high risk of metastasis.
#56 Chondrosarcoma: Practice Essentials, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1258236-overview
The prognosis is correlated with the grade and stage of the lesion at the time of diagnosis. The location of the lesion is also important because tumors in areas where complete wide resection is possible are associated with better prognoses. In general, chondrosarcomas of the head and neck are associated with better disease-specific survival and overall survival rates than chondrosarcomas located elsewhere. […] Recurrence and distant metastasis may develop. The metastasis rate for primary chondrosarcoma is higher than that for secondary chondrosarcoma, and the rate of distant metastasis is higher in patients with local recurrence than in those without local recurrence. […] In children and adolescents, chondrosarcoma of bone has a very good prognosis and is less aggressive than in older patients.
#57 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
Not all stage IV disease is the same. […] Some people can relapse with 50 lumps, and some may relapse with only one lump. But, if its in a different organ, its classified as stage IV, explains Conley. The most common site of metastasis is the lung. If I have a relapsed patient with one or two spots on their lung, I believe we can potentially cure them. For relapsed patients with more advanced disease, curing the disease may not be possible. In those cases, our goal is to keep patients alive as long as possible with a preserved quality of life. […] An MD Anderson study published in 2023 found that patients with conventional and dedifferentiated chondrosarcoma who have the TP53 gene mutation have a poorer prognosis. […] For years, the only prognostic factor was the grade of the tumor, says Conley. But what weve proven through research is that the TP53 mutation can help determine prognosis as well. This helps us counsel patients during treatment.
#58 Understanding chondrosarcoma: symptoms, treatment and prognosis | MD Anderson Cancer Center
https://www.mdanderson.org/cancerwise/understanding-chondrosarcoma–symptoms–treatment-and-prognosis.h00-159699123.html
Not all stage IV disease is the same. […] Some people can relapse with 50 lumps, and some may relapse with only one lump. But, if its in a different organ, its classified as stage IV, explains Conley. The most common site of metastasis is the lung. If I have a relapsed patient with one or two spots on their lung, I believe we can potentially cure them. For relapsed patients with more advanced disease, curing the disease may not be possible. In those cases, our goal is to keep patients alive as long as possible with a preserved quality of life. […] An MD Anderson study published in 2023 found that patients with conventional and dedifferentiated chondrosarcoma who have the TP53 gene mutation have a poorer prognosis. […] For years, the only prognostic factor was the grade of the tumor, says Conley. But what weve proven through research is that the TP53 mutation can help determine prognosis as well. This helps us counsel patients during treatment.
#59 Treatment method and prognostic factors of chondrosarcoma: based on Surveillance, Epidemiology, and End Results (SEER) database – Hua – Translational Cancer Research
https://tcr.amegroups.org/article/view/41253/html
Chondrosarcoma is a malignant tumor originating from cartilage tissue. It is the second most malignant bone tumor, accounting for about 10% to 15% of all primary bone tumors. […] This study found that although non-surgical treatment of chondrosarcoma has made great progress, at present, it is still not considered that non-surgical treatment can significantly improve the prognosis. There are many factors affecting the prognosis of chondrosarcoma, including age, primary site, histological type, grade, tumor size, distant metastasis and treatment method. […] The results showed age, primary site, histological type, grade, tumor size, metastasis, and treatment method were independent factors affecting ACM and CSSM.
#60 Treatment method and prognostic factors of chondrosarcoma: based on Surveillance, Epidemiology, and End Results (SEER) database – Hua – Translational Cancer Research
https://tcr.amegroups.org/article/view/41253/html
Chondrosarcoma is a malignant tumor originating from cartilage tissue. It is the second most malignant bone tumor, accounting for about 10% to 15% of all primary bone tumors. […] This study found that although non-surgical treatment of chondrosarcoma has made great progress, at present, it is still not considered that non-surgical treatment can significantly improve the prognosis. There are many factors affecting the prognosis of chondrosarcoma, including age, primary site, histological type, grade, tumor size, distant metastasis and treatment method. […] The results showed age, primary site, histological type, grade, tumor size, metastasis, and treatment method were independent factors affecting ACM and CSSM.