Guzy drobne, okrągłe z desmoplastyczną stroma – Charakterystyka, pielęgnacja i opieka

Guzy drobne, okrągłe z desmoplastyczną stroma
Charakterystyka, pielęgnacja i opieka

Desmoplastyczny mięsak drobnokomórkowy z desmoplastyczną stromą (DSRCT) to rzadki, agresywny mięsak tkanek miękkich, najczęściej dotykający młodych mężczyzn, charakteryzujący się licznymi guzami w jamie brzusznej i miednicy. Pięcioletnie przeżycie wynosi jedynie 10-25%, a leczenie opiera się na wielomodalnym podejściu łączącym chemioterapię (schematy zawierające ifosfamid, doksorubicynę, cyklofosfamid, winkrystynę, etopozyd, daktynomycynę), chirurgię cytoredukcyjną oraz radioterapię. Chemioterapia neoadjuwantowa i adjuwantowa jest kluczowa ze względu na chemowrażliwość nowotworu, a radioterapia stosowana jest głównie w celu eliminacji pozostałości pooperacyjnych. W przypadku rozsianej choroby rozważa się także hipertermiczną chemioterapię dootrzewnową (HIPEC), choć jej skuteczność pozostaje niejednoznaczna. Kompleksowa opieka wymaga multidyscyplinarnego zespołu specjalistów, w tym chirurgów onkologicznych, onkologów medycznych, radioterapeutów oraz specjalistów od leczenia bólu i rehabilitacji.

Charakterystyka Guzów drobnych, okrągłych z desmoplastyczną stromą (DSRCT)

Guzy drobne, okrągłe z desmoplastyczną stromą (DSRCT) to rzadki, agresywny i złośliwy typ mięsaka tkanek miękkich, który najczęściej dotyka młodych mężczyzn i chłopców. Choroba charakteryzuje się powstawaniem wielu guzów w obrębie jamy brzusznej i miednicy, chociaż dokładne miejsce pochodzenia nowotworu zwykle pozostaje nieznane. DSRCT jest chorobą oporną na leczenie z wysokim ryzykiem nawrotu i niekorzystnym rokowaniem – pięcioletnie przeżycie wynosi około 10-25%.¹²

Ze względu na rzadkość występowania i ograniczoną wiedzę na temat patologicznej i klinicznej natury tej choroby, nie istnieje jednoznaczny konsensus dotyczący optymalnego postępowania terapeutycznego. Standardem pozostaje leczenie wielomodalne obejmujące chemioterapię, zabieg chirurgiczny i radioterapię.³⁴

Multidyscyplinarne podejście do opieki nad pacjentem z DSRCT

Kompleksowa opieka nad pacjentem z DSRCT wymaga zaangażowania zespołu specjalistów z różnych dziedzin. Taki zespół obejmuje doświadczonych chirurgów onkologicznych, onkologów medycznych, radioterapeutów, pielęgniarki onkologiczne oraz innych specjalistów wspierających proces leczenia.⁵⁶

Szczególnie istotna jest rola ośrodków o dużym doświadczeniu w leczeniu DSRCT, ponieważ badania sugerują, że pacjenci leczeni w szpitalach przeprowadzających dużą liczbę złożonych operacji osiągają lepsze wyniki leczenia. Wiele ośrodków onkologicznych posiada dedykowane zespoły specjalizujące się w leczeniu mięsaków, w tym DSRCT, które mają największe doświadczenie w leczeniu tej rzadkiej choroby.⁷

Przygotowanie indywidualnego planu leczenia

Plan leczenia DSRCT jest dostosowywany indywidualnie do każdego pacjenta. Zespół medyczny bierze pod uwagę lokalizację nowotworu oraz to, czy rozprzestrzenił się on na inne części ciała. Większość pacjentów z tym typem nowotworu otrzymuje kombinację różnych metod leczenia.⁸⁹

Metody leczenia DSRCT

Chemioterapia

Chemioterapia stanowi podstawę leczenia DSRCT ze względu na jego chemowrażliwość. Stosuje się silne leki przeciwnowotworowe, które mogą być podawane przed operacją w celu zmniejszenia guza (chemioterapia neoadjuwantowa) lub po operacji w celu zniszczenia pozostałych komórek nowotworowych (chemioterapia adjuwantowa).¹⁰¹¹

Stosowane schematy chemioterapii obejmują:¹²

Schematy stosowane w mięsakach tkanek miękkich, zawierające ifosfamid i doksorubicynę
Schematy ustalone dla mięsaka Ewinga:
- Schemat P6 (cyklofosfamid, doksorubicyna, winkrystyna, ifosfamid i etopozyd)
- VAIA (winkrystyna, daktynomycyna, ifosfamid i doksorubicyna)

¹³

Ze względu na agresywny charakter DSRCT, ciągła chemioterapia z zastosowaniem odpowiedniego schematu oraz odpowiednia opieka wspomagająca są niezbędne dla długoterminowego przeżycia pacjentów.¹⁴

Leczenie chirurgiczne

Celem leczenia chirurgicznego jest całkowite usunięcie guza (cytoredukcja), co może nie być możliwe, jeśli nowotwór wrósł w pobliskie narządy. W takich przypadkach lekarze mogą najpierw zalecić chemioterapię, aby zmniejszyć guz przed operacją.¹⁵

Kompleksowa opieka pooperacyjna obejmuje:¹⁶

Kontrolę bólu i obrzęku wokół nacięcia chirurgicznego
Szczegółowe instrukcje dotyczące opieki w domu
Zalecenia odnośnie powrotu do normalnych aktywności

Istotną rolę odgrywa anestezjolog specjalizujący się w pediatrii, który wspiera zespół w kontroli bólu i dyskomfortu związanego z leczeniem DSRCT.¹⁷

Radioterapia

Radioterapia wykorzystuje silne wiązki energii do niszczenia komórek nowotworowych. W przypadku DSRCT zlokalizowanych w jamie brzusznej, radioterapia może być opcją leczenia komórek nowotworowych pozostałych po operacji.¹⁸¹⁹

Chociaż radioterapia całej jamy brzuszno-miedniczej w DSRCT nie była szeroko stosowana ze względu na ostrą toksyczność i niski wskaźnik odpowiedzi, badania potwierdzają, że może ona odgrywać znaczącą rolę w leczeniu zaawansowanego DSRCT jamy brzusznej i przyczynić się do trwałej remisji.²⁰²¹

Leczenie celowane

Leczenie celowane może być zalecane, jeśli nowotwór powraca po leczeniu lub gdy rozprzestrzenił się na inne części ciała. Leki celowane atakują określone substancje chemiczne obecne w komórkach nowotworowych, blokując je i powodując śmierć komórek nowotworowych.²²²³

Przed zastosowaniem tej metody leczenia, komórki nowotworowe pacjenta mogą być testowane w celu określenia, czy leki celowane mogą być skuteczne. Leczenie celowane może być stosowane samodzielnie lub w połączeniu z chemioterapią.²⁴

Chemioterapia dootrzewnowa w hipertermii (HIPEC)

W przypadku rozsianej choroby w jamie brzusznej, sama resekcja chirurgiczna może nie zapewnić trwałej kontroli, ponieważ mikroskopowe pozostałości choroby wydają się prawie nieuniknione. Teoretyczną korzyścią dodania chemioterapii dootrzewnowej w hipertermii (HIPEC) bezpośrednio po cytoredukcji chirurgicznej (CRS) jest eliminacja potencjalnej mikroskopowej choroby.²⁵

Chociaż HIPEC była stosowana po CRS, jej korzyści pozostają niejasne, a niewiele badań oceniło tę opcję. Jednak ze względu na wysokie ryzyko nawrotu, leczenie pooperacyjne jest kluczowe. Po operacji stosowane są konsolidacyjne schematy z radioterapią i leczeniem systemowym, aby zwalczyć mikroskopową chorobę.²⁶²⁷

Opieka nad pacjentem z DSRCT

Kontrola objawów i skutków ubocznych leczenia

Leczenie DSRCT wiąże się z licznymi skutkami ubocznymi, które wymagają odpowiedniej opieki. Pacjenci poddawani chemioterapii mogą doświadczać:²⁸

Kataru
Bólu kości
Utraty włosów
Innych objawów związanych z konkretnym schematem chemioterapii

Zespół medyczny powinien zapewnić kompleksową opiekę w zakresie kontroli bólu i innych objawów. Istotną rolę odgrywają tu specjaliści od leczenia bólu i opieki paliatywnej, którzy poprzez leki, medycynę integracyjną i inne sprawdzone metody mogą zwiększyć komfort i jakość życia pacjenta podczas i po leczeniu.²⁹

Wsparcie psychiczne i emocjonalne

Poza radzeniem sobie z fizycznymi wyzwaniami związanymi z DSRCT, niezwykle ważne jest dbanie o zdrowie psychiczne. Aktywności takie jak spacery na świeżym powietrzu i rozmowy z terapeutą mogą znacząco przyczynić się do poprawy samopoczucia.³⁰

Pacjenci z DSRCT podkreślają znaczenie:³¹

Utrzymywania nadziei i pozytywnego nastawienia
Skupienia się na czynnościach, które przynoszą radość
Życia dzień po dniu
Budowania sieci wsparcia
Czerpania siły zarówno ze społeczności, jak i z perspektywy

Rehabilitacja i powrót do zdrowia

Po leczeniu DSRCT, pacjenci mogą potrzebować rehabilitacji, w tym fizjoterapii i terapii zajęciowej, aby odzyskać siłę i funkcje oraz pomóc w codziennych czynnościach życiowych.³²

Zespół rehabilitacyjny współpracuje z zespołem onkologicznym, aby zapewnić kompleksowe podejście do odzyskiwania sprawności po leczeniu. Program rehabilitacji może obejmować ćwiczenia wzmacniające, terapię zajęciową oraz inne interwencje dostosowane do indywidualnych potrzeb pacjenta.³³

Opieka długoterminowa i obserwacja

Wizyty kontrolne i monitorowanie

Życie pacjenta po leczeniu DSRCT obejmuje regularne wizyty u zespołu onkologicznego w celu sprawdzenia nawrotu choroby i radzenia sobie z wszelkimi skutkami ubocznymi choroby lub jej leczenia.³⁴

Zespół medyczny współpracuje z lekarzem podstawowej opieki zdrowotnej i innymi świadczeniodawcami w celu monitorowania wzrostu i rozwoju pacjenta, a także oferuje edukację i doradztwo w zakresie odżywiania, ćwiczeń i zdrowego trybu życia.³⁵

Medycyna integracyjna w opiece długoterminowej

Zespół medycyny integracyjnej oferuje terapie oparte na dowodach, skupione na fizycznym i emocjonalnym komforcie oraz dobrostanie pacjenta. Programy takie jak:³⁶

Arteterapia i muzykoterapia
Terapia ruchowa
Masaż terapeutyczny

Mogą pomóc pacjentowi rozładować napięcie i przezwyciężyć wyzwania związane z chorobą nowotworową.³⁷

Dieta i aktywność fizyczna

Dietetycy i fizjolodzy wysiłku współpracują z zespołem medycznym pacjenta w celu stworzenia planu żywieniowego i ćwiczeń, który może pomóc pacjentowi czuć się lepiej i pozostać silniejszym, a także potencjalnie zmniejszyć przyszłe ryzyko raka lub powikłań.³⁸

Wyzwania w leczeniu DSRCT i kierunki rozwoju

Leczenie DSRCT pozostaje wyzwaniem klinicznym i brakuje standardowej strategii leczenia. Potencjalne strategie terapeutyczne obejmują operację usunięcia guza, radioterapię i chemioterapię, jednak do tej pory nie ustalono standardowego schematu chemioterapeutycznego w leczeniu DSRCT.³⁹

Nowe kierunki badań obejmują poszukiwanie biomarkerów prognostycznych i predykcyjnych oraz testowanie nowych terapii celowanych, takich jak koniugaty przeciwciało-lek. Przykładem jest enfortumab-vedotin, nowatorski koniugat przeciwciało-lek skierowany przeciwko nektynie-4, który wykazał potencjał w leczeniu DSRCT.⁴⁰

Ekspresja nektyny-4 powinna być badana w DSRCT i innych mięsakach jako potencjalny biomarker prognostyczny i predykcyjny, co może otworzyć nowe możliwości terapeutyczne dla pacjentów z tym rzadkim i agresywnym nowotworem.⁴¹

Wnioski

DSRCT jest rzadkim i agresywnym nowotworem wymagającym kompleksowego, wielomodalnego podejścia terapeutycznego. Chemioterapia, chirurgia i radioterapia stanowią podstawę leczenia, jednak wyniki pozostają niezadowalające, co podkreśla potrzebę dalszych badań i opracowania nowych strategii terapeutycznych.⁴²⁴³

Opieka nad pacjentem z DSRCT wymaga zaangażowania multidyscyplinarnego zespołu specjalistów, którzy zapewniają nie tylko leczenie samego nowotworu, ale także kompleksową opiekę wspierającą obejmującą kontrolę objawów, wsparcie psychologiczne, rehabilitację oraz opiekę długoterminową.⁴⁴⁴⁵

Pomimo wyzwań związanych z leczeniem DSRCT, istnieją przypadki długotrwałego przeżycia pacjentów leczonych terapią wielomodalną, co daje nadzieję na poprawę wyników w przyszłości dzięki zaawansowanym badaniom i nowym metodom leczenia.⁴⁶

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 What are Desmoplastic Small Round Cell Tumors (DSRCT)? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center
https://www.mdanderson.org/cancer-types/desmoplastic-small-round-cell-tumors.html
Desmoplastic small round cell tumors (DSRCT) are a type of soft-tissue sarcoma. DSRCT causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. […] DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment. […] A team of highly focused renowned physicians, including surgeons, oncologists and radiologists, as well as a specialized support staff of nurses, physicians assistants and others follow your child from diagnosis through treatment. […] Childrens Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and familys experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child. […] DSRCT is treated in our Children’s Cancer Hospital.
#2
https://link.springer.com/article/10.1007/s00432-025-06102-3
Desmoplastic small round cell tumor is a rare sarcoma type with poor prognosis for which there is no standard of care. It primarily affects adolescents and young adults and treatment is both multimodal and challenging due to the aggressive nature of the disease. […] Treatment is multimodal and involves peri-operative chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide and may also include adjuvant radiation. […] The standard of care for DSRCT is constantly evolving as we explore new lines of therapy for an aggressive tumor with a generally poor prognosis; overall survival at five years is roughly 10-25%. […] Since 1996, the mainstay of treatment has been aggressive chemotherapy followed by surgical resection where possible and radiotherapy to high-risk sites. […] In our case, the patient underwent chemotherapy regimens for urothelial carcinoma before trying a DSRCT-targeted regimen.
#3 A Case of Intra-Abdominal Desmoplastic Small Round Cell Tumor in a Young Girl Patient
https://clinmedjournals.org/articles/ogcr/obstetrics-and-gynaecology-cases-reviews-ogcr-5-128.php?jid=ogcr
The main aim of this case report was to present the method of diagnosis, management, and prognosis of a young adolescent diagnosed with a desmoplastic small round cell tumor (DSRCT). […] Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm and a multimodal treatment with surgery, chemotherapy and radiotherapy is used for these rare cases. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis. […] The prognosis remains bleak and evolution is usually done to death, despite a heavy therapeutic care including surgery, high-dose chemotherapy and sometimes radiotherapy.
#4 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
https://www.mdpi.com/1718-7729/30/4/299
Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. […] There is no consensus on the best treatment approach for DSRCT, but several studies have reported improved survival with multimodal treatment that consists of systemic therapy combined with surgery and radiotherapy. Although hyperthermic intraperitoneal chemotherapy (HIPEC) has been employed after cytoreductive surgery (CRS), its benefit remains unclear. The aim of this review is to identify the role of HIPEC after CRS in patients with DSRCT.
#5 Desmoplastic Small Round Cell Tumor Treatments for Your Child | MSK Kids | Memorial Sloan Kettering Cancer Center
https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts/treatment
Chemotherapy and surgery are common treatments for desmoplastic small round cell tumor (DSRCT) in children. MSK Kids offers all treatments for DSRCT. […] We will talk with you about the best treatment plan for your child. We will answer all your questions, so you will know what to expect during your child’s treatment. […] MSK Kids cancer surgeons in New York City have improved DSRCT treatment. They have developed new ways to remove these tumors. They work as part of a very experienced team that includes medical and radiation oncologists, nurses, and others. […] Our surgeons do more desmoplastic small round cell tumors operations in a year than many others do in a career. Research suggests that patients at hospitals that do a high volume of a complex surgery have better treatment results.
#6 What are Desmoplastic Small Round Cell Tumors (DSRCT)? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center
https://www.mdanderson.org/cancer-types/desmoplastic-small-round-cell-tumors.html
Desmoplastic small round cell tumors (DSRCT) are a type of soft-tissue sarcoma. DSRCT causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. […] DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment. […] A team of highly focused renowned physicians, including surgeons, oncologists and radiologists, as well as a specialized support staff of nurses, physicians assistants and others follow your child from diagnosis through treatment. […] Childrens Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and familys experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child. […] DSRCT is treated in our Children’s Cancer Hospital.
#7 Desmoplastic Small Round Cell Tumor Treatments for Your Child | MSK Kids | Memorial Sloan Kettering Cancer Center
https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts/treatment
Chemotherapy and surgery are common treatments for desmoplastic small round cell tumor (DSRCT) in children. MSK Kids offers all treatments for DSRCT. […] We will talk with you about the best treatment plan for your child. We will answer all your questions, so you will know what to expect during your child’s treatment. […] MSK Kids cancer surgeons in New York City have improved DSRCT treatment. They have developed new ways to remove these tumors. They work as part of a very experienced team that includes medical and radiation oncologists, nurses, and others. […] Our surgeons do more desmoplastic small round cell tumors operations in a year than many others do in a career. Research suggests that patients at hospitals that do a high volume of a complex surgery have better treatment results.
#8 Desmoplastic small round cell tumors – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/dsrct/diagnosis-treatment/drc-20449484
Our caring team of Mayo Clinic experts can help you with your desmoplastic small round cell tumors-related health concerns […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation. […] For desmoplastic small round cell tumors that affect the abdomen, radiation might be an option to kill cancer cells that remain after surgery.
#9 Mayo Clinic Health Library – Desmoplastic small round cell tumors | Swiss Medical Network
https://www.swissmedical.net/en/healtcare-library/con-20248516
Desmoplastic small round cell tumors can happen to anyone, but they’re more common in young men and boys. […] Treatment for desmoplastic small round cell tumors typically involves a combination of treatments. Options might include surgery, chemotherapy and radiation therapy. […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] The goal of surgery is to remove all of the cancer. It might not be possible if the cancer has grown into nearby organs. If that happens, your healthcare professional may recommend chemotherapy with powerful drugs to shrink the cancer first. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation.
#10 Mayo Clinic Health Library – Desmoplastic small round cell tumors | Swiss Medical Network
https://www.swissmedical.net/en/healtcare-library/con-20248516
Desmoplastic small round cell tumors can happen to anyone, but they’re more common in young men and boys. […] Treatment for desmoplastic small round cell tumors typically involves a combination of treatments. Options might include surgery, chemotherapy and radiation therapy. […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] The goal of surgery is to remove all of the cancer. It might not be possible if the cancer has grown into nearby organs. If that happens, your healthcare professional may recommend chemotherapy with powerful drugs to shrink the cancer first. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation.
#11 Desmoplastic small round cell tumors – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/dsrct/diagnosis-treatment/drc-20449484
Our caring team of Mayo Clinic experts can help you with your desmoplastic small round cell tumors-related health concerns […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation. […] For desmoplastic small round cell tumors that affect the abdomen, radiation might be an option to kill cancer cells that remain after surgery.
#12 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
https://www.mdpi.com/1718-7729/30/4/299
Given the chemosensitivity of DSRCT and the high proportion of metastatic disease, initial treatment is usually systemic chemotherapy. The regimens employed include those for soft tissue sarcoma which incorporate ifosfamide and doxorubicin or those established for Ewing sarcoma which are most commonly the P6 regimen (cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide) or VAIA (vincristine, dactinomycin, ifosfamide, and doxorubicin). […] There are no clear guidelines on patient selection for surgery in DSRCT. Almost all patients receive systemic chemotherapy prior to surgery, where progression on chemotherapy indicates poor tumor biology and precludes resection. […] The ultimate goal of surgery is complete cytoreduction but the potential risks of morbidity should be considered since this could prevent or delay postoperative treatments such as chemotherapy and/or radiotherapy. If the expected surgical morbidity and mortality from optimal cytoreduction are excessive, the surgeon may consider debulking surgery as an alternative.
#13
https://link.springer.com/article/10.1007/s00432-025-06102-3
Desmoplastic small round cell tumor is a rare sarcoma type with poor prognosis for which there is no standard of care. It primarily affects adolescents and young adults and treatment is both multimodal and challenging due to the aggressive nature of the disease. […] Treatment is multimodal and involves peri-operative chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide and may also include adjuvant radiation. […] The standard of care for DSRCT is constantly evolving as we explore new lines of therapy for an aggressive tumor with a generally poor prognosis; overall survival at five years is roughly 10-25%. […] Since 1996, the mainstay of treatment has been aggressive chemotherapy followed by surgical resection where possible and radiotherapy to high-risk sites. […] In our case, the patient underwent chemotherapy regimens for urothelial carcinoma before trying a DSRCT-targeted regimen.
#14 A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy
https://www.spandidos-publications.com/10.3892/ol.2011.457
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that predominantly affects young males. No standard therapy is currently available for patients with DSRCT and the prognosis remains extremely poor. […] This is a long-term survival case of metastatic DSRCT treated with multimodal therapy including 15 courses of multiagent chemotherapy, radiation therapy for the hepatic portal region using 42.5 Gy, and four instances of therapeutic endoscopy. […] DSRCT is an aggressive but chemo-sensitive disease, and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for long-term survival. […] In conclusion, DSRCT is an aggressive but chemosensitive disease and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for the long-term survival of these patients.
#15 Mayo Clinic Health Library – Desmoplastic small round cell tumors | Swiss Medical Network
https://www.swissmedical.net/en/healtcare-library/con-20248516
Desmoplastic small round cell tumors can happen to anyone, but they’re more common in young men and boys. […] Treatment for desmoplastic small round cell tumors typically involves a combination of treatments. Options might include surgery, chemotherapy and radiation therapy. […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] The goal of surgery is to remove all of the cancer. It might not be possible if the cancer has grown into nearby organs. If that happens, your healthcare professional may recommend chemotherapy with powerful drugs to shrink the cancer first. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation.
#16 Desmoplastic Small Round Cell Tumor Treatments for Your Child | MSK Kids | Memorial Sloan Kettering Cancer Center
https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts/treatment
MSK Kids surgeons take on cases that other centers may turn away because they’re too complex. Many people bring their child to MSK Kids because of our surgeons’ reputations as experts in pediatric sarcoma care. […] Your child’s surgeon will talk with you about how long your child will be in the hospital. The recovery from DSRCT surgery can take several weeks. Often, there’s pain and swelling around the incision (surgical cut). We do all we can do make your child feel comfortable. […] Your care team will tell you how to care for your child at home. They will talk with you about when your child can go back to their regular activities. […] An anesthesiologist uses anesthesia (medicine to make your child sleep) during surgery. MSK Kids anesthesiologists are all pediatric anesthesiologists, with special training in the care of children. As part of your child’s care team, anesthesiologists help with pain or discomfort related to DSRCT treatments. They will help control your child’s pain.
#17 Desmoplastic Small Round Cell Tumor Treatments for Your Child | MSK Kids | Memorial Sloan Kettering Cancer Center
https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts/treatment
MSK Kids surgeons take on cases that other centers may turn away because they’re too complex. Many people bring their child to MSK Kids because of our surgeons’ reputations as experts in pediatric sarcoma care. […] Your child’s surgeon will talk with you about how long your child will be in the hospital. The recovery from DSRCT surgery can take several weeks. Often, there’s pain and swelling around the incision (surgical cut). We do all we can do make your child feel comfortable. […] Your care team will tell you how to care for your child at home. They will talk with you about when your child can go back to their regular activities. […] An anesthesiologist uses anesthesia (medicine to make your child sleep) during surgery. MSK Kids anesthesiologists are all pediatric anesthesiologists, with special training in the care of children. As part of your child’s care team, anesthesiologists help with pain or discomfort related to DSRCT treatments. They will help control your child’s pain.
#18 Mayo Clinic Health Library – Desmoplastic small round cell tumors | Swiss Medical Network
https://www.swissmedical.net/en/healtcare-library/con-20248516
Radiation therapy uses powerful beams of energy to kill cancer cells. For desmoplastic small round cell tumors that affect the abdomen, radiation might be an option to kill cancer cells that remain after surgery. […] Targeted drug treatments attack specific chemicals present within cancer cells. By blocking these chemicals, targeted drug treatments can cause cancer cells to die.
#19 Desmoplastic small round cell tumors – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/dsrct/diagnosis-treatment/drc-20449484
Our caring team of Mayo Clinic experts can help you with your desmoplastic small round cell tumors-related health concerns […] Treatment for desmoplastic small round cell tumor depends on your situation. Your healthcare team considers your cancer’s location and whether it has spread to other parts of the body. Most people with this type of cancer receive a combination of treatments. […] Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink the cancer. This makes it easier to remove with surgery. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation. […] For desmoplastic small round cell tumors that affect the abdomen, radiation might be an option to kill cancer cells that remain after surgery.
#20 Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report
https://www.spandidos-publications.com/10.3892/ol.2015.4024
Desmoplastic small round cell tumor (DSRCT) is a rare tumor that mainly affects adolescents, and typically involves the abdominal and pelvic peritoneum. […] DSRCT is a rare malignancy requiring multidisciplinary treatment, including surgery, chemotherapy and radiotherapy. […] The results of the present study confirm that radiotherapy has a significant role in the treatment of advanced abdominal DSRCT and may contribute to durable remission. […] The standard treatment protocol for DSRCT has not been well established. […] The treatment of DSRCT remains clinically challenging and lacks a standard treatment strategy. Potential therapeutic strategies include, debulking surgery, radiotherapy and chemotherapy. […] To date, there is no standard chemotherapeutic regimen for the treatment of DSRCT.
#21 Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report
https://www.spandidos-publications.com/10.3892/ol.2015.4024
Radiotherapy, and whole abdominal-pelvic radiotherapy in particular in DSRCT, has not been used extensively due to its acute toxicity and low response rate. […] Chemoradiotherapy is significant in the treatment of advanced abdominal DSRCT. As a therapeutic measure chemoradiotherapy may relieve symptoms and improve patient outcome. […] The effectiveness and safety of chemoradiotherapy urgently require improvement prior to use in the treatment of this disease, particularly when patients are unable to tolerate aggressive surgery or the tumors cannot be surgically resected.
#22 Desmoplastic small round cell tumors – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/dsrct/diagnosis-treatment/drc-20449484
Targeted therapy might be recommended if your cancer comes back after treatment. It may also be offered if your cancer has spread to other parts of the body. Your healthcare professional may have your cancer cells tested to see if targeted therapy drugs are likely to work against your cancer. Targeted therapy can be used alone or combined with chemotherapy. […] Ask your healthcare professional for details about your cancer. Ask about your treatment options. If you like, ask about your prognosis. As you learn more about desmoplastic small round cell tumors, you may become more confident in making treatment decisions. […] Your healthcare professional is likely to ask you a number of questions, such as: When did you first notice your signs and symptoms? Are you experiencing pain? Does anything seem to improve your symptoms? What, if anything, appears to worsen your symptoms?
#23 Mayo Clinic Health Library – Desmoplastic small round cell tumors | Swiss Medical Network
https://www.swissmedical.net/en/healtcare-library/con-20248516
Radiation therapy uses powerful beams of energy to kill cancer cells. For desmoplastic small round cell tumors that affect the abdomen, radiation might be an option to kill cancer cells that remain after surgery. […] Targeted drug treatments attack specific chemicals present within cancer cells. By blocking these chemicals, targeted drug treatments can cause cancer cells to die.
#24 Desmoplastic small round cell tumors – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/dsrct/diagnosis-treatment/drc-20449484
Targeted therapy might be recommended if your cancer comes back after treatment. It may also be offered if your cancer has spread to other parts of the body. Your healthcare professional may have your cancer cells tested to see if targeted therapy drugs are likely to work against your cancer. Targeted therapy can be used alone or combined with chemotherapy. […] Ask your healthcare professional for details about your cancer. Ask about your treatment options. If you like, ask about your prognosis. As you learn more about desmoplastic small round cell tumors, you may become more confident in making treatment decisions. […] Your healthcare professional is likely to ask you a number of questions, such as: When did you first notice your signs and symptoms? Are you experiencing pain? Does anything seem to improve your symptoms? What, if anything, appears to worsen your symptoms?
#25 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
https://www.mdpi.com/1718-7729/30/4/299
In the setting of disseminated abdominal disease, surgical resection alone may not provide durable control, as microscopic residual disease appears almost inevitable. The theoretical benefit of adding HIPEC immediately after CRS is to eradicate potential microscopic disease. […] Considering the high risk of relapse, postoperative treatment is critical. After surgery, further consolidative regimens with radiation therapy and systemic therapy have been employed to target microscopic disease. […] Induction chemotherapy and complete cytoreduction are essential cornerstones in the treatment of DSRCT. Although CRS and even debulking surgery have demonstrated survival benefits, they should be restricted to patients who respond to chemotherapy and those without extra-peritoneal metastases.
#26 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
https://www.mdpi.com/1718-7729/30/4/299
Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. […] There is no consensus on the best treatment approach for DSRCT, but several studies have reported improved survival with multimodal treatment that consists of systemic therapy combined with surgery and radiotherapy. Although hyperthermic intraperitoneal chemotherapy (HIPEC) has been employed after cytoreductive surgery (CRS), its benefit remains unclear. The aim of this review is to identify the role of HIPEC after CRS in patients with DSRCT.
#27 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
https://www.mdpi.com/1718-7729/30/4/299
In the setting of disseminated abdominal disease, surgical resection alone may not provide durable control, as microscopic residual disease appears almost inevitable. The theoretical benefit of adding HIPEC immediately after CRS is to eradicate potential microscopic disease. […] Considering the high risk of relapse, postoperative treatment is critical. After surgery, further consolidative regimens with radiation therapy and systemic therapy have been employed to target microscopic disease. […] Induction chemotherapy and complete cytoreduction are essential cornerstones in the treatment of DSRCT. Although CRS and even debulking surgery have demonstrated survival benefits, they should be restricted to patients who respond to chemotherapy and those without extra-peritoneal metastases.
#28 Gianna’s Rare Desmoplastic Small Round Cell Tumors (DSRCT) Story
https://thepatientstory.com/patient-stories/rare/desmoplastic-small-round-cell-tumor-dsrct/gianna-c/
Gianna, a graphic designer from Philadelphia, PA, shares her journey after being diagnosed with desmoplastic small round cell tumor (DSRCT), a rare sarcoma, in 2024. Her treatment plan at Fox Chase Cancer Center involved chemotherapy over 7 rounds, translating to 14 cycles. Giannaâs routine includes hospital stays for chemotherapy, interspersed with rest periods at home. Due to her chemotherapy, she consequently experiences side effects like a runny nose, bone pain, and hair loss. […] Beyond dealing with the physical challenges brought about by desmoplastic small round cell tumor (DSRCT), Gianna also emphasizes the importance of mental health. Walking outdoors and speaking with a therapist contribute significantly to her well-being. […] Gianna advocates maintaining hope and staying positive. She likewise stresses the value of focusing on activities that bring joy and taking life one day at a time. […] In her message, Gianna urges those listening to cherish every moment and engage in activities they love. She further underscores the significance of maintaining hope and living in the present, asserting that cancer does not dictate oneâs ability to enjoy life.
#29 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
When your child is hurting, our pediatric pain and palliative care team can help. Through medication, integrative medicine and other proven methods, we can increase your child’s comfort and quality of life during and after treatment. […] Our registered dietitians and exercise physiologists work with your child’s medical team to create a nutrition and exercise plan that can help your child feel better and stay stronger and possibly reduce the future risk of cancer or complications.
#30 Gianna’s Rare Desmoplastic Small Round Cell Tumors (DSRCT) Story
https://thepatientstory.com/patient-stories/rare/desmoplastic-small-round-cell-tumor-dsrct/gianna-c/
Gianna, a graphic designer from Philadelphia, PA, shares her journey after being diagnosed with desmoplastic small round cell tumor (DSRCT), a rare sarcoma, in 2024. Her treatment plan at Fox Chase Cancer Center involved chemotherapy over 7 rounds, translating to 14 cycles. Giannaâs routine includes hospital stays for chemotherapy, interspersed with rest periods at home. Due to her chemotherapy, she consequently experiences side effects like a runny nose, bone pain, and hair loss. […] Beyond dealing with the physical challenges brought about by desmoplastic small round cell tumor (DSRCT), Gianna also emphasizes the importance of mental health. Walking outdoors and speaking with a therapist contribute significantly to her well-being. […] Gianna advocates maintaining hope and staying positive. She likewise stresses the value of focusing on activities that bring joy and taking life one day at a time. […] In her message, Gianna urges those listening to cherish every moment and engage in activities they love. She further underscores the significance of maintaining hope and living in the present, asserting that cancer does not dictate oneâs ability to enjoy life.
#31 Hamishâs Rare Desmoplastic Small Round Cell Tumors Story
https://thepatientstory.com/patient-stories/rare/desmoplastic-small-round-cell-tumor-dsrct/hamish-s/
Hamish credits the support of healthcare professionals, particularly oncology nurses, his family, and friends, for helping him navigate the emotional and physical toll of cancer. […] He emphasizes the importance of finding joy, staying connected, and seeking hope even in uncertainty. […] Through his story, Hamish aims to inspire others to radically embrace hope, build support networks, and find strength in both community and perspective.
#32 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#33 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#34 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#35 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#36 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#37 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
After your child is treated for DSRCT, we will provide follow-up care to check for recurrence and to deal with any side effects of the disease or its treatment. Your child may need rehabilitation, including physical and occupational therapy to regain strength and function and to help with daily living skills. […] Your child’s life as a cancer survivor will include regular visits with our pediatric cancer team to ensure the best possible outcomes and to help prevent or treat any complications following treatment. We’ll work with your child’s primary care physician and other health providers to monitor growth and development, and offer education and counseling about nutrition, exercise and health living so you can take the best care of your child. […] Our pediatric integrative medicine team offers evidence-based therapies focused on your child’s physical and emotional comfort and well-being. Programs such as art and music therapy, movement therapy and massage therapy can help your child release tension and overcome cancer-related challenges.
#38 Desmoplastic Small Round Cell Tumors Support | Baptist Health Miami Cancer Institute
https://baptisthealth.net/services/cancer-care/miami-cancer-institute/our-approach/pediatric-cancers/desmoplastic-small-round-cell-tumor/living-beyond-desmoplastic-small-round-cell-tumor
When your child is hurting, our pediatric pain and palliative care team can help. Through medication, integrative medicine and other proven methods, we can increase your child’s comfort and quality of life during and after treatment. […] Our registered dietitians and exercise physiologists work with your child’s medical team to create a nutrition and exercise plan that can help your child feel better and stay stronger and possibly reduce the future risk of cancer or complications.
#39 Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report
https://www.spandidos-publications.com/10.3892/ol.2015.4024
Desmoplastic small round cell tumor (DSRCT) is a rare tumor that mainly affects adolescents, and typically involves the abdominal and pelvic peritoneum. […] DSRCT is a rare malignancy requiring multidisciplinary treatment, including surgery, chemotherapy and radiotherapy. […] The results of the present study confirm that radiotherapy has a significant role in the treatment of advanced abdominal DSRCT and may contribute to durable remission. […] The standard treatment protocol for DSRCT has not been well established. […] The treatment of DSRCT remains clinically challenging and lacks a standard treatment strategy. Potential therapeutic strategies include, debulking surgery, radiotherapy and chemotherapy. […] To date, there is no standard chemotherapeutic regimen for the treatment of DSRCT.
#40
https://link.springer.com/article/10.1007/s00432-025-06102-3
Finally, the patients most sustained response was on enfortumab-vedotin, a novel anti-nectin-4 targeted antibody-drug conjugate highlighting a potential crossover between therapies for urothelial carcinoma and DSRCT. […] DSRCT is an ultra-rare sarcoma with limited treatment options and poor prognosis. Such a case of long sustained response and clinical benefit with EV is encouraging and merits exploration. Nectin-4 expression should be examined in DSRCT and other sarcomas as a potential prognostic and predictive biomarker.
#41
https://link.springer.com/article/10.1007/s00432-025-06102-3
Finally, the patients most sustained response was on enfortumab-vedotin, a novel anti-nectin-4 targeted antibody-drug conjugate highlighting a potential crossover between therapies for urothelial carcinoma and DSRCT. […] DSRCT is an ultra-rare sarcoma with limited treatment options and poor prognosis. Such a case of long sustained response and clinical benefit with EV is encouraging and merits exploration. Nectin-4 expression should be examined in DSRCT and other sarcomas as a potential prognostic and predictive biomarker.
#42 Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report
https://www.spandidos-publications.com/10.3892/ol.2015.4024
Desmoplastic small round cell tumor (DSRCT) is a rare tumor that mainly affects adolescents, and typically involves the abdominal and pelvic peritoneum. […] DSRCT is a rare malignancy requiring multidisciplinary treatment, including surgery, chemotherapy and radiotherapy. […] The results of the present study confirm that radiotherapy has a significant role in the treatment of advanced abdominal DSRCT and may contribute to durable remission. […] The standard treatment protocol for DSRCT has not been well established. […] The treatment of DSRCT remains clinically challenging and lacks a standard treatment strategy. Potential therapeutic strategies include, debulking surgery, radiotherapy and chemotherapy. […] To date, there is no standard chemotherapeutic regimen for the treatment of DSRCT.
#43 A Case of Intra-Abdominal Desmoplastic Small Round Cell Tumor in a Young Girl Patient
https://clinmedjournals.org/articles/ogcr/obstetrics-and-gynaecology-cases-reviews-ogcr-5-128.php?jid=ogcr
The main aim of this case report was to present the method of diagnosis, management, and prognosis of a young adolescent diagnosed with a desmoplastic small round cell tumor (DSRCT). […] Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm and a multimodal treatment with surgery, chemotherapy and radiotherapy is used for these rare cases. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis. […] The prognosis remains bleak and evolution is usually done to death, despite a heavy therapeutic care including surgery, high-dose chemotherapy and sometimes radiotherapy.
#44 Desmoplastic Small Round Cell Tumor Treatments for Your Child | MSK Kids | Memorial Sloan Kettering Cancer Center
https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts/treatment
Chemotherapy and surgery are common treatments for desmoplastic small round cell tumor (DSRCT) in children. MSK Kids offers all treatments for DSRCT. […] We will talk with you about the best treatment plan for your child. We will answer all your questions, so you will know what to expect during your child’s treatment. […] MSK Kids cancer surgeons in New York City have improved DSRCT treatment. They have developed new ways to remove these tumors. They work as part of a very experienced team that includes medical and radiation oncologists, nurses, and others. […] Our surgeons do more desmoplastic small round cell tumors operations in a year than many others do in a career. Research suggests that patients at hospitals that do a high volume of a complex surgery have better treatment results.
#45 What are Desmoplastic Small Round Cell Tumors (DSRCT)? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center
https://www.mdanderson.org/cancer-types/desmoplastic-small-round-cell-tumors.html
Desmoplastic small round cell tumors (DSRCT) are a type of soft-tissue sarcoma. DSRCT causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. […] DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment. […] A team of highly focused renowned physicians, including surgeons, oncologists and radiologists, as well as a specialized support staff of nurses, physicians assistants and others follow your child from diagnosis through treatment. […] Childrens Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and familys experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child. […] DSRCT is treated in our Children’s Cancer Hospital.
#46 A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy
https://www.spandidos-publications.com/10.3892/ol.2011.457
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that predominantly affects young males. No standard therapy is currently available for patients with DSRCT and the prognosis remains extremely poor. […] This is a long-term survival case of metastatic DSRCT treated with multimodal therapy including 15 courses of multiagent chemotherapy, radiation therapy for the hepatic portal region using 42.5 Gy, and four instances of therapeutic endoscopy. […] DSRCT is an aggressive but chemo-sensitive disease, and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for long-term survival. […] In conclusion, DSRCT is an aggressive but chemosensitive disease and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for the long-term survival of these patients.