Pineoblastoma
Leczenie

Pineoblastoma to agresywny, wysokozłośliwy (WHO stopień IV) nowotwór wywodzący się z szyszynki, charakteryzujący się szybkim wzrostem i dyspersją przez płyn mózgowo-rdzeniowy. Stanowi 24-50% guzów zarodkowych szyszynki u dzieci i wymaga leczenia wielomodalnego, obejmującego chirurgię, radioterapię oraz chemioterapię. Chirurgiczne usunięcie guza jest często ograniczone ze względu na głębokie położenie i bliskość struktur krytycznych; całkowita resekcja osiągana jest w około 30% przypadków i wiąże się z lepszym rokowaniem. Wodogłowie obturacyjne, często towarzyszące pineoblastoma, wymaga interwencji neurochirurgicznej, takiej jak endoskopowa wentrikulostomia komory trzeciej (ETV) lub założenie zastawki komorowo-otrzewnowej. Radioterapia całego ośrodkowego układu nerwowego (craniospinal irradiation, CSI) z dawką 23,4-36 Gy oraz boostem do 54-59,5 Gy na łoże guza stanowi standard leczenia u dzieci powyżej 3. roku życia, natomiast u młodszych pacjentów stosuje się intensywną chemioterapię w celu opóźnienia napromieniania ze względu na ryzyko neurotoksyczności.

Wprowadzenie do Pineoblastoma

Pineoblastoma to rzadki, agresywny nowotwór złośliwy wywodzący się z szyszynki – małego gruczołu zlokalizowanego głęboko w mózgu, odpowiedzialnego za produkcję melatoniny, hormonu regulującego sen. Jest to nowotwór wysokiego stopnia złośliwości (IV stopień według Światowej Organizacji Zdrowia), cechujący się szybkim wzrostem i zdolnością do rozprzestrzeniania się w obrębie centralnego układu nerwowego, głównie przez płyn mózgowo-rdzeniowy.12 Pineoblastoma stanowi około 24-50% guzów zarodkowych szyszynki u dzieci, chociaż może występować również u dorosłych.3

Wielomodalne podejście do leczenia

Leczenie pineoblastoma wymaga kompleksowego, wielomodułowego podejścia. Ze względu na agresywny charakter guza i jego głębokie umiejscowienie w mózgu, leczenie zwykle obejmuje kombinację chirurgii, radioterapii i chemioterapii. Plan terapeutyczny jest dostosowywany indywidualnie w zależności od wieku pacjenta, rozmiaru i lokalizacji guza, obecności przerzutów oraz ogólnego stanu zdrowia.456

Najlepsze wyniki są osiągane, gdy leczenie jest prowadzone w wyspecjalizowanych ośrodkach onkologicznych, które mają doświadczenie w leczeniu rzadkich nowotworów mózgu i mogą zapewnić multidyscyplinarne podejście przez zespół ekspertów, w tym neurochirurgów, onkologów, radioterapeutów i neuropatologów.78

Leczenie chirurgiczne

Resekcja guza

Leczenie chirurgiczne jest zwykle pierwszym etapem w terapii pineoblastoma. Głównym celem operacji jest usunięcie jak największej części guza, bez uszkodzenia ważnych struktur mózgu. Jednak ze względu na głębokie położenie szyszynki w mózgu i bliskość ważnych struktur naczyniowo-nerwowych, całkowite usunięcie (resekcja totalna) guza jest często trudne do osiągnięcia i występuje tylko w około 30% przypadków.91011

W wielu przypadkach wykonuje się resekcję częściową (subtotalną) lub maksymalnie bezpieczną resekcję, aby zmniejszyć masę guza i złagodzić objawy związane z uciskiem na otaczające struktury. W przypadkach, gdy bezpośrednia resekcja nie jest możliwa, przeprowadza się biopsję w celu uzyskania materiału do badania histopatologicznego, a następnie stosuje się chemioterapię w celu zmniejszenia guza przed ewentualną późniejszą operacją.1213

Całkowita resekcja guza jest związana z lepszym rokowaniem, jednak operacja musi być wykonana z uwzględnieniem zachowania funkcji neurologicznych pacjenta. W przypadku guzów znacząco naciekających krytyczne struktury, bardziej bezpieczne może być wykonanie częściowej resekcji, a następnie zastosowanie terapii uzupełniającej.1415

Leczenie wodogłowia

W wielu przypadkach pineoblastoma prowadzi do wodogłowia obturacyjnego, spowodowanego blokadą przepływu płynu mózgowo-rdzeniowego. W takich sytuacjach konieczne jest wykonanie zabiegu odbarczającego, który może obejmować:

  • Endoskopową wentrikulostomię komory trzeciej (ETV) – zabieg polegający na wytworzeniu małego otworu w komorze trzeciej mózgu, co umożliwia swobodniejszy przepływ płynu mózgowo-rdzeniowego i zapobiega jego gromadzeniu się
  • Założenie zastawki komorowo-otrzewnowej – implantacja małego drenu odprowadzającego nadmiar płynu mózgowo-rdzeniowego z komór mózgu do jamy otrzewnowej

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Zabiegi odbarczające wodogłowie mogą być wykonywane razem z biopsją lub resekcją guza, lub jako oddzielna procedura.1920

Radioterapia

Znaczenie radioterapii

Radioterapia jest kluczowym elementem leczenia pineoblastoma, szczególnie w przypadkach, gdy całkowita resekcja guza nie była możliwa. Jest to metoda wykorzystująca wysokoenergetyczne promieniowanie (promienie X, protony lub inne źródła) do niszczenia komórek nowotworowych.2122

Ze względu na tendencję pineoblastoma do rozprzestrzeniania się wzdłuż dróg płynu mózgowo-rdzeniowego, standardem jest zastosowanie napromieniania całego ośrodkowego układu nerwowego (craniospinal irradiation, CSI). Technika ta obejmuje napromienianie całego mózgu i rdzenia kręgowego, z dodatkowym zwiększeniem dawki (boost) na obszar pierwotnego guza.2324

Dawkowanie i techniki radioterapii

Dawka, lokalizacja i czas radioterapii zależą od wielu czynników, w tym od wieku pacjenta, typu guza i tego, czy rozprzestrzenił się do innych części mózgu lub rdzenia kręgowego. U dzieci powyżej 3. roku życia z nierozsiany pineoblastoma, standardowe podejście obejmuje:

  • Napromienianie całego ośrodkowego układu nerwowego (CSI) dawką 23,4-36 Gy
  • Dodatkowy boost na obszar łoża guza do całkowitej dawki 54-59,5 Gy

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U dzieci poniżej 3. roku życia zwykle unika się radioterapii lub opóźnia jej zastosowanie ze względu na ryzyko poważnych długoterminowych skutków ubocznych wpływających na rozwijający się mózg. W takich przypadkach stosuje się intensywną chemioterapię, aby opóźnić potrzebę radioterapii.2829

Nowoczesne techniki radioterapii, takie jak:

pozwalają na precyzyjne dostarczenie wysokich dawek promieniowania do guza, przy jednoczesnej minimalizacji uszkodzeń zdrowych tkanek.303132

Radiochirurgia stereotaktyczna, koncentrująca wiele wiązek promieniowania na dokładnie określonych punktach, jest czasami stosowana w leczeniu pineoblastoma, który nawrócił po wcześniejszym leczeniu.3334

Skuteczność radioterapii

Badania wykazały, że radioterapia znacząco poprawia przeżycie pacjentów z pineoblastoma. Zastosowanie napromieniania całego ośrodkowego układu nerwowego (CSI) wiąże się z lepszymi wynikami niż napromienianie ograniczone tylko do obszaru guza, szczególnie ze względu na tendencję tego nowotworu do rozprzestrzeniania się w obrębie układu nerwowego.3536

W jednym z badań trzyletnie przeżycie bez progresji choroby (EFS) i całkowite przeżycie (OS) wynosiło 50,7% u pacjentów, którzy otrzymali radioterapię, w porównaniu do znacznie niższych wskaźników u pacjentów, którzy jej nie otrzymali.37

Chemioterapia

Rola chemioterapii

Chemioterapia jest istotnym elementem leczenia pineoblastoma, często stosowanym w połączeniu z chirurgią i radioterapią. Wykorzystuje silne leki do niszczenia komórek nowotworowych lub hamowania ich wzrostu.3839

Chemioterapia może być stosowana w różnych fazach leczenia:

  • Przed operacją (neoadjuwantowa) – w celu zmniejszenia guza i ułatwienia jego późniejszego usunięcia
  • Po operacji (adjuwantowa) – w celu zniszczenia pozostałych komórek nowotworowych
  • Równocześnie z radioterapią – w celu zwiększenia skuteczności leczenia
  • Jako terapia podtrzymująca po zakończeniu intensywnego leczenia
  • U bardzo małych dzieci – w celu opóźnienia potrzeby radioterapii do czasu, gdy dziecko będzie starsze

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Schematy chemioterapii

W leczeniu pineoblastoma stosuje się różne schematy chemioterapii, często zawierające kombinacje kilku leków cytostatycznych. Najczęściej stosowane leki obejmują:

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Dobór konkretnych leków i schematów leczenia zależy od wieku pacjenta, stadium choroby i wcześniejszego leczenia. Często stosuje się chemioterapię wielolekową, wykorzystującą synergistyczne działanie różnych cytostatyków.4748

Chemioterapia wysokodawkowa

W niektórych przypadkach, szczególnie u dzieci poniżej 3. roku życia lub pacjentów z chorobą nawrotową, stosuje się wysokodawkową chemioterapię z autologicznym przeszczepieniem komórek macierzystych (HDCx/AuHCR). Metoda ta polega na podaniu bardzo wysokich dawek chemioterapii, które niszczą komórki nowotworowe, ale również szpik kostny. Wcześniej pobrane komórki macierzyste pacjenta są następnie przeszczepiane, aby odbudować szpik i układ krwiotwórczy.4950

Badania sugerują, że HDCx/AuHCR może pozytywnie wpływać na przeżycie bez progresji choroby (PFS) i całkowite przeżycie (OS) u pacjentów z pineoblastoma, chociaż wysoki odsetek nawrotów po jednym cyklu konsolidacji wskazuje na potrzebę bardziej intensywnych schematów leczenia.5152

Nowoczesne podejścia terapeutyczne

Badania kliniczne

Ze względu na rzadkość występowania pineoblastoma i ograniczone opcje standardowego leczenia, badania kliniczne mogą stanowić ważną alternatywę dla pacjentów, szczególnie w przypadku nawrotu choroby. Badania kliniczne oferują dostęp do najnowszych opcji terapeutycznych, chociaż potencjalne skutki uboczne mogą nie być w pełni znane.5354

Obecnie badane są nowe strategie leczenia pineoblastoma, w tym:

  • Terapie celowane molekularnie, oparte na specyficznych cechach genetycznych i molekularnych guza
  • Immunoterapia
  • Metronomiczna chemioterapia – podawanie niskich, ale częstych dawek leków przeciwnowotworowych
  • Kombinacje leków ukierunkowanych molekularnie z konwencjonalną chemioterapią

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Terapia protonowa

Terapia protonowa to zaawansowana forma radioterapii, która wykorzystuje wiązki protonów (zamiast tradycyjnych promieni X) do precyzyjnego napromieniania guza. Ta metoda pozwala na lepszą ochronę zdrowych tkanek, co jest szczególnie istotne w przypadku guzów zlokalizowanych w pobliżu krytycznych struktur mózgu.5859

Centrum Terapii Protonowej St. Jude jest pierwszym na świecie ośrodkiem, który stosuje tę terapię wyłącznie u dzieci, co podkreśla jej potencjalną wartość w leczeniu pediatrycznych guzów mózgu, w tym pineoblastoma.60

Badania nad nową techniką terapii protonowej, znaną jako „FLASH” (z ultrawysoką dawką), wykazują mniejszą toksyczność i lepszą ochronę zdrowych tkanek, przy zachowaniu skuteczności w eliminacji komórek nowotworowych.61

Terapie celowane i badania molekularne

Postęp w zrozumieniu biologii molekularnej pineoblastoma doprowadził do identyfikacji czterech głównych podtypów tego nowotworu, różniących się zmianami genetycznymi, grupami wiekowymi i rokowaniem. Ta molekularna klasyfikacja otwiera drogę do bardziej spersonalizowanych podejść terapeutycznych.62

Badania wskazują, że różne podtypy pineoblastoma mogą wykazywać różną wrażliwość na specyficzne inhibitory i prawdopodobnie różnie reagują na standardowe schematy leczenia. Na przykład, podtypy MYC-PB i RB1-PB wykazują wrażliwość na specyficzne inhibitory.63

Badania genetyczne i molekularne, w tym profilowanie metylacji DNA, stają się coraz ważniejszym narzędziem w diagnostyce i klasyfikacji pineoblastoma, co może prowadzić do opracowania bardziej skutecznych, ukierunkowanych terapii.6465

Skuteczność leczenia i rokowanie

Rokowanie w pineoblastoma zależy od wielu czynników, w tym od wieku pacjenta, zakresu resekcji guza, obecności przerzutów i zastosowanego leczenia.6667

Czynniki związane z lepszym rokowaniem obejmują:

  • Wiek powyżej 3-4 lat w momencie diagnozy
  • Całkowitą resekcję guza
  • Brak przerzutów w momencie rozpoznania
  • Zastosowanie wielomodułowego leczenia, obejmującego chirurgię, radioterapię i chemioterapię

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Historycznie, pineoblastoma wiązał się z niekorzystnym rokowaniem, z 5-letnim przeżyciem wynoszącym około 10%. Jednak nowsze dane z 2021 roku wskazują na poprawę wyników, z 5-letnim przeżyciem sięgającym 58-81% i medianą całkowitego przeżycia wynoszącą 4-8 lat.71

U dzieci poniżej 3. roku życia rokowanie jest zwykle gorsze, szczególnie w przypadku choroby przerzutowej. W jednym z badań mediana całkowitego przeżycia dla młodszych dzieci (poniżej 3 lat) wynosiła 0,9 roku, w porównaniu do 7,9 roku dla dzieci starszych (powyżej 3 lat) leczonych chemioterapią i radioterapią.72

Analiza danych z ostatnich lat wskazuje na poprawę wyników leczenia od 2012 roku, co prawdopodobnie wynika częściowo ze zwiększenia odsetka pacjentów poddawanych całkowitej resekcji guza oraz zastosowania wielomodułowych schematów leczenia.73

Leczenie nawrotów

Pomimo agresywnego leczenia początkowego, pineoblastoma może nawracać. W przypadku nawrotu choroby, opcje leczenia mogą obejmować:

  • Powtórną operację, jeśli jest technicznie możliwa
  • Radiochirurgię stereotaktyczną
  • Alternatywne schematy chemioterapii
  • Wysokodawkową chemioterapię z przeszczepieniem komórek macierzystych
  • Udział w badaniach klinicznych testujących nowe terapie

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Ze względu na ograniczone opcje standardowego leczenia nawrotów pineoblastoma, badania kliniczne fazy 0 i I, testujące nowe leki i strategie terapeutyczne, mogą być cenną alternatywą dla pacjentów z nawrotową chorobą.77

Podsumowanie

Leczenie pineoblastoma wymaga kompleksowego, wielomodałowego podejścia, dostosowanego do indywidualnych potrzeb pacjenta. Najlepsze wyniki osiąga się poprzez połączenie maksymalnie bezpiecznej resekcji chirurgicznej, radioterapii całego ośrodkowego układu nerwowego z dodatkowym boostem na obszar guza oraz odpowiednio dobranej chemioterapii.7879

Postęp w technikach neurochirurgicznych, radioterapii i zrozumieniu biologii molekularnej pineoblastoma prowadzi do poprawy wyników leczenia i jakości życia pacjentów z tym rzadkim nowotworem. Dalsze badania nad terapiami celowanymi molekularnie i spersonalizowanym podejściem terapeutycznym, opartym na cechach genetycznych i molekularnych guza, mogą przynieść przełom w leczeniu tej trudnej choroby.8081

Dla osiągnięcia optymalnych wyników kluczowe znaczenie ma leczenie w wyspecjalizowanych ośrodkach onkologicznych, posiadających doświadczenie w terapii rzadkich nowotworów mózgu oraz możliwość zapewnienia kompleksowej opieki multidyscyplinarnej.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Pineoblastoma: Causes, Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24950-pineoblastoma
    Pineoblastoma is a cancerous (malignant) brain tumor in your pineal gland. This type of cancer grows quickly and can spread to tissue and fluid around your brain. […] While there isn’t a cure available for pineoblastoma, there are several treatment options. Your healthcare provider can surgically remove the tumor if it hasn’t spread. Pineoblastoma can be challenging to treat, which increases the risk of complications from this type of cancer. It may lead to symptoms that get worse over time and cause a shortened life expectancy. There’s a chance that it may come back after treatment. […] Treatment for pineoblastoma may include: Surgery to remove some or all of the tumor. Your surgeon will let you know what’s possible with surgery and the potential risks. Surgery to treat hydrocephalus. A surgeon may insert a shunt, or a small tube, to drain excess cerebrospinal fluid from building up around your brain. Radiation therapy. A common treatment after surgery, radiation therapy can shrink tumors or destroy cancer cells. Chemotherapy. Chemotherapy is a treatment option in addition to radiation therapy and surgery. Chemotherapy can shrink the tumor before surgery or destroy the remaining cancer cells after surgery. High-dose chemotherapy followed by autologous stem cell transplant. A healthcare provider will collect your own stem cells before receiving certain high-dose chemotherapy. Then you’ll receive an injection of the stem cells back into you after the chemotherapy.
  • #2 What Is Pineoblastoma? | St. Baldrick’s Blog
    https://blog.stbaldricks.org/what-is-pineoblastoma/
    Pineoblastoma is a rare and aggressive type of brain tumor that starts in the pineal gland, a small organ deep in the brain responsible for producing melatonin, the hormone that regulates sleep. […] Pineoblastoma is more aggressive than other types of pineal gland tumors. […] Treatment for pineoblastoma focuses on removing the tumor or reducing its size and managing symptoms. Treatment plans will likely consist of surgery, radiation, and/or chemotherapy. […] Researchers are looking at ways to avoid damage to healthy cells during treatment. […] A highly targeted form of proton therapy, known as “FLASH,” with an ultrahigh dose rate, shows less toxicity and improved healthy tissue sparing, while maintaining effectiveness in eradicating tumor cells. […] Researchers are also working to determine the effectiveness of combined proton therapy with immunotherapy on pineoblastoma.
  • #3 Persistent pineoblastoma: Complete response and >26 years overall survival in a ten-month-old female treated with antineoplastons
    https://www.oatext.com/persistent-pineoblastoma-complete-response-and-26-years-overall-survival-in-a-ten-month-old-female-treated-with-antineoplastons.php
    Pineoblastomas account for 24-50% of pediatric pineal parenchymal tumors. They are aggressive, being classified as grade 4 tumors by the World Health Organization (WHO). Objectives: A young female child with persistent pineoblastoma after maximal surgery is presented to 1) demonstrate efficacy of ANP therapy (Antineoplaston A10 {Atengenal} and Antineoplaston AS2-1 {Astugenal}) in treatment of pineoblastoma and 2) review Protocol BT-12, a Phase II Study of Antineoplastons A10 and AS2-1 in Children with Primitive Neuroectodermal Tumors, which utilized intravenous (IV) ANP therapy. […] We conclude that ANP therapy is an attractive therapeutic option for children with pineoblastoma. Continued study in clinical studies is indicated. […] Treatment consists of surgery, radiation therapy (RT) in children 3 years of age, and chemotherapy. In cases of persistent or recurrent disease, stereotactic radiosurgery is frequently utilized.
  • #4 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Pineoblastoma treatment focuses on safely removing as much of the tumor as possible with surgery, or with a combination of chemotherapy and surgery, followed by radiation and chemotherapy. […] Treatment may include: Surgery to get a sample for diagnosis and to remove part or all of the tumor. In some cases, it is not possible to remove all of the tumor in 1 surgery. In these cases, we either perform a biopsy, use chemotherapy to shrink the tumor, or do both in order to help brain surgeons (neurosurgeons) later fully remove the tumor. […] Radiation is an important part of treatment for pineoblastoma. The dose, location, and timing of radiation will depend on many factors. These include your child’s age, type of tumor, and if it has spread to other parts of the brain or spine. […] Chemotherapy (chemo) is important in the treatment of pineoblastoma. The exact type of chemotherapy and the length of treatment will depend on your child’s age and type of pineoblastoma.
  • #5 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Treatment for pineoblastoma depends on: […] – The type of pineoblastoma […] – Patient age and health when diagnosed […] – If the cancer has spread […] – If the tumor can be surgically removed […] – If the patient is old enough to receive radiotherapy […] – Available treatments […] Treatments may include: […] – Surgery […] – Chemotherapy […] – Radiation therapy for older children […] The goal of surgery is to remove as much of the tumor as possible. Surgery for pineoblastoma can be difficult because of the tumor location and the risk to nearby blood vessels. In some cases, it is not possible to remove all of the tumor in 1 surgery. In such cases, surgeons may decide to: […] – Perform a biopsy […] – Use chemotherapy to shrink the tumor […] – Do both a biopsy and chemotherapy so that surgeons can later fully remove the tumor
  • #6 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49
    https://www.barrowneuro.org/condition/pineoblastoma/
    Surgery is often one of the first steps in treating a pineoblastoma, although it’s most often combined with radiation and chemotherapy. This multimodal approach reduces overall tumor size and helps relieve symptoms, manage residual tumor cells, and reduce the risk of metastasis. […] Due to the pineal gland’s location deep within the brain, a complete removal is challenging and rare. In most cases, a partial resection, or maximal safe resection, is done to reduce the tumor’s size and alleviate pressure. […] Nonsurgical treatments for pineoblastomas are essential because they are highly aggressive and, in most cases, cannot be entirely removed through surgery. The choice and sequence of treatments will depend on your age, overall health, the tumor’s characteristics, and whether or not it has spread.
  • #7 Childhood Primitive Neuroectodermal Tumors (PNETs) and Pineoblastoma | Dana-Farber Cancer Institute
    https://www.dana-farber.org/cancer-care/types/childhood-primitive-neuroectodermal-tumors-pineoblastoma
    At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our team of specialized pediatric neuro-oncologists, neuropathologists, and neurosurgeons have expertise in treating rare brain and spine tumors. Children with PNETs or pineoblastoma receive expert care through our Medulloblastoma Program, part of our comprehensive Childhood Brain Tumor Center. […] Your child’s physician will determine a specific course of treatment based on several factors, including their health status and the tumors characteristics. […] Treatments may include: […] Surgery: We initially use surgery to biopsy the tumor and form a complete diagnosis. Depending on the tumor, we may recommend further surgery to remove as much of the cancer as possible. […] Chemotherapy: Chemotherapy treatment involves medications that interfere with a cancer cells ability to grow or reproduce. Doctors use it to shrink tumors and eliminate remaining cancer cells.
  • #8 Pineoblastoma – Neurosurgery | UCLA Health
    https://www.uclahealth.org/medical-services/neurosurgery/conditions-treated/pineoblastoma
    Pineoblastoma is one of several different types of tumors that arise in the area of the pineal gland, requiring different therapies. The exact diagnosis is critical for choosing the correct therapy. […] A team of experts is needed for optimum therapy. UCLA experts in neurosurgery, neuropathology (microscopic diagnosis), radiation therapy and oncology will work together to provide the best and latest therapies. […] The surgical removal of pineal region tumors ranks among the most difficult neurosurgical operations. Patients typically are referred to major university medical centers, like UCLA, where certain neurosurgeons have specific expertise in this type of surgery. […] Although surgery can be curative in some cases, in other cases it provides a definitive diagnosis and reduces the size of the tumor.
  • #9 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Pineoblastoma treatment focuses on safely removing as much of the tumor as possible with surgery, or with a combination of chemotherapy and surgery, followed by radiation and chemotherapy. […] Treatment may include: Surgery to get a sample for diagnosis and to remove part or all of the tumor. In some cases, it is not possible to remove all of the tumor in 1 surgery. In these cases, we either perform a biopsy, use chemotherapy to shrink the tumor, or do both in order to help brain surgeons (neurosurgeons) later fully remove the tumor. […] Radiation is an important part of treatment for pineoblastoma. The dose, location, and timing of radiation will depend on many factors. These include your child’s age, type of tumor, and if it has spread to other parts of the brain or spine. […] Chemotherapy (chemo) is important in the treatment of pineoblastoma. The exact type of chemotherapy and the length of treatment will depend on your child’s age and type of pineoblastoma.
  • #10 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Pineoblastoma treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended. […] A brain surgeon, also called a neurosurgeon, works to remove as much of the pineoblastoma as possible. Sometimes all of the cancer can’t be removed. This is because pineoblastoma forms near important structures deep inside the brain. More treatments after surgery are usually needed. These treatments target the remaining cells. […] Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams to the brain and spinal cord. Extra radiation is directed at the cancer cells. […] Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove.
  • #11 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49
    https://www.barrowneuro.org/condition/pineoblastoma/
    Surgery is often one of the first steps in treating a pineoblastoma, although it’s most often combined with radiation and chemotherapy. This multimodal approach reduces overall tumor size and helps relieve symptoms, manage residual tumor cells, and reduce the risk of metastasis. […] Due to the pineal gland’s location deep within the brain, a complete removal is challenging and rare. In most cases, a partial resection, or maximal safe resection, is done to reduce the tumor’s size and alleviate pressure. […] Nonsurgical treatments for pineoblastomas are essential because they are highly aggressive and, in most cases, cannot be entirely removed through surgery. The choice and sequence of treatments will depend on your age, overall health, the tumor’s characteristics, and whether or not it has spread.
  • #12 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Pineoblastoma treatment focuses on safely removing as much of the tumor as possible with surgery, or with a combination of chemotherapy and surgery, followed by radiation and chemotherapy. […] Treatment may include: Surgery to get a sample for diagnosis and to remove part or all of the tumor. In some cases, it is not possible to remove all of the tumor in 1 surgery. In these cases, we either perform a biopsy, use chemotherapy to shrink the tumor, or do both in order to help brain surgeons (neurosurgeons) later fully remove the tumor. […] Radiation is an important part of treatment for pineoblastoma. The dose, location, and timing of radiation will depend on many factors. These include your child’s age, type of tumor, and if it has spread to other parts of the brain or spine. […] Chemotherapy (chemo) is important in the treatment of pineoblastoma. The exact type of chemotherapy and the length of treatment will depend on your child’s age and type of pineoblastoma.
  • #13 Pineoblastoma – Wikipedia
    https://en.wikipedia.org/wiki/Pineoblastoma
    Initial treatment for pineoblastoma often includes a shunting procedure to redirect accumulated cerebrospinal fluid secondary to obstructive hydrocephalus. This shunt can help manage increased intracranial pressure and relieve some symptoms. Surgery to remove the tumor is associated with better outcomes, however, this is not always possible due to the proximity of the pineal gland to neurovascular structures. Complete tumor resection is only seen in about 30% of cases. Following surgery, radiation therapy to the brain and spinal cord can increase survival. However, radiation can only safely be used in patients over 3 years old due to the risk of significant neurological impairment. Chemotherapy treatment can also be used, either before or after surgery; its optimal use is still under investigation. […] Complete gross tumor resection is associated with improved prognosis, but is difficult and rare to achieve. Radiation therapy after surgery is also linked to improved survival.
  • #14 Post-Surgical Prognosis of Patients with Pineoblastoma: A Systematic Review and Individual Patient Data Analysis with Trends over Time
    https://www.mdpi.com/2072-6694/15/13/3374
    This study demonstrates that EOR less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and tumor presentation in children less than three years old are associated with poorer prognosis. […] Pineoblastoma remains a severe rare disease, but survival outcomes are improving. […] Our analysis also demonstrated an improvement in outcomes since 2012. This improvement is likely driven at least in part by an increase in the proportion of pineoblastoma patients receiving GTR, which was also independently associated with time to mortality. […] These data stress the importance of GTR followed by adjuvant RT in maximizing OS.
  • #15 Complete regression of adult pineoblastoma following radiotherapy: A case report and review of the literature
    https://www.spandidos-publications.com/10.3892/ol.2015.3574?text=fulltext
    In the present case, the tumor was not amenable to GTR due to its proximity to the midbrain and thalamus, and because both internal cerebral veins and the great cerebral vein were enclosed within the tumor. Considering quality of life, a minimally invasive strategy of partial resection followed by radiotherapy was adopted, which contributed to a favorable response with complete tumor regression and an excellent neurological outcome. […] The function of postoperative therapy remains undefined in PB. […] In the present case, the tumor volume was great and its location was close to critical structures; therefore, partial resection followed by radiotherapy was employed in order to avoid nervous injury. […] In conclusion, PB is rare in adults and, although the appropriate treatment strategy for PB has yet to be determined, the current case successfully demonstrates that aggressive surgery must be avoided in patients whose tumors show significant involvement of critical structures, taking into account surgical complications, and that function-preserving resection followed by postoperative radiotherapy may be the optimal treatment strategy.
  • #16 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Some patients with pineoblastoma may have surgery to help reduce the side effects from hydrocephalus. A surgeon will make a small opening in the third ventricle in the brain. This opening helps cerebrospinal fluid flow more freely to keep it from building up and causing harm. In other cases, doctors may place a shunt, a small tube that drains cerebrospinal fluid from the brain. The shunt may be temporary or permanent. […] Radiation therapy is often given after pineoblastoma surgery. Energy from the radiation can damage or destroy rapidly growing cancer cells. Doctors can also use radiation to shrink the tumor. The dose and amount of radiation therapy depend on: […] – Your child’s age […] – The type of tumor […] – If the disease has spread […] Radiation is not used in young children because it increases the risk of long-term side effects. In patients older than 3, radiation is usually given to the whole nervous system (craniospinal irradiation). A higher dose (boost) is given at the primary tumor site.
  • #17 Pineal Tumors Treatment & Management: Approach Considerations, Treatment Options, Surgical Therapy
    https://emedicine.medscape.com/article/249945-treatment
    Treatment of pineal region tumors is complex. Various strategies exist for managing associated signs and symptoms, such as hydrocephalus or local mass effect, and achieving cytoreduction/oncologic control. The choice of strategies depends on the clinical presentation, the presence of hydrocephalus, results of cerebrospinal fluid (CSF) and serum tumor marker studies, the presence of metastatic disease, and the patient’s age and general medical status. […] Treatment typically begins with endoscopic third ventriculostomy (ETV) for relief of the obstruction, followed by inspection of the CSF for dissemination, sampling of the CSF, and biopsy of the tumor. The tumor may be resected endoscopically if indicated. […] For patients with positive tumor markers, treatment depends on tumor type. Treatment may include a combination of radiotherapy, chemotherapy, and resection. Most patients cannot be confidently diagnosed with tumor markers and therefore histological diagnosis via tissue biopsy is required. Surgical resection may be indicated depending on tumor type.
  • #18 Mayo Clinic Health Library – Pineoblastoma | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20310674
    Pineoblastoma treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended. […] A pineoblastoma may grow big enough to block the flow of cerebrospinal fluid. This puts pressure on the brain. An operation can redirect the fluid through a drain or tube. This helps relieve the pressure. This procedure may be done at the same time as a biopsy or surgery to remove the pineoblastoma. […] A brain surgeon, also called a neurosurgeon, works to remove as much of the pineoblastoma as possible. Sometimes all of the cancer can’t be removed. This is because pineoblastoma forms near important structures deep inside the brain. More treatments after surgery are usually needed. These treatments target the remaining cells. […] Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams to the brain and spinal cord. Extra radiation is directed at the cancer cells.
  • #19 Pineoblastoma: Causes, Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24950-pineoblastoma
    Pineoblastoma is a cancerous (malignant) brain tumor in your pineal gland. This type of cancer grows quickly and can spread to tissue and fluid around your brain. […] While there isn’t a cure available for pineoblastoma, there are several treatment options. Your healthcare provider can surgically remove the tumor if it hasn’t spread. Pineoblastoma can be challenging to treat, which increases the risk of complications from this type of cancer. It may lead to symptoms that get worse over time and cause a shortened life expectancy. There’s a chance that it may come back after treatment. […] Treatment for pineoblastoma may include: Surgery to remove some or all of the tumor. Your surgeon will let you know what’s possible with surgery and the potential risks. Surgery to treat hydrocephalus. A surgeon may insert a shunt, or a small tube, to drain excess cerebrospinal fluid from building up around your brain. Radiation therapy. A common treatment after surgery, radiation therapy can shrink tumors or destroy cancer cells. Chemotherapy. Chemotherapy is a treatment option in addition to radiation therapy and surgery. Chemotherapy can shrink the tumor before surgery or destroy the remaining cancer cells after surgery. High-dose chemotherapy followed by autologous stem cell transplant. A healthcare provider will collect your own stem cells before receiving certain high-dose chemotherapy. Then you’ll receive an injection of the stem cells back into you after the chemotherapy.
  • #20 Pineoblastoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube
    https://www.augustahealth.com/disease/pineoblastoma/
    Pineoblastoma can be very difficult to treat. It can spread within the brain and the fluid (cerebrospinal fluid) around the brain, but it rarely spreads beyond the central nervous system. Treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments may also be recommended. […] Pineoblastoma treatment options include: […] Surgery to relieve fluid buildup in the brain. A pineoblastoma may grow to block the flow of cerebrospinal fluid, which can cause a buildup of fluid that puts pressure on the brain (hydrocephalus). An operation to create a way for the fluid to flow out of the brain may be recommended. Sometimes this procedure can be combined with a biopsy or surgery to remove the tumor. […] Surgery to remove the pineoblastoma. The brain surgeon (neurosurgeon) will work to remove the pineoblastoma with the goal of removing as much of the tumor as possible. But it’s often impossible to remove the tumor entirely because pineoblastoma forms near critical structures deep within the brain. Most children with pineoblastoma receive additional treatments after surgery to target the remaining cells.
  • #21 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Pineoblastoma treatment focuses on safely removing as much of the tumor as possible with surgery, or with a combination of chemotherapy and surgery, followed by radiation and chemotherapy. […] Treatment may include: Surgery to get a sample for diagnosis and to remove part or all of the tumor. In some cases, it is not possible to remove all of the tumor in 1 surgery. In these cases, we either perform a biopsy, use chemotherapy to shrink the tumor, or do both in order to help brain surgeons (neurosurgeons) later fully remove the tumor. […] Radiation is an important part of treatment for pineoblastoma. The dose, location, and timing of radiation will depend on many factors. These include your child’s age, type of tumor, and if it has spread to other parts of the brain or spine. […] Chemotherapy (chemo) is important in the treatment of pineoblastoma. The exact type of chemotherapy and the length of treatment will depend on your child’s age and type of pineoblastoma.
  • #22 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Pineoblastoma treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended. […] A brain surgeon, also called a neurosurgeon, works to remove as much of the pineoblastoma as possible. Sometimes all of the cancer can’t be removed. This is because pineoblastoma forms near important structures deep inside the brain. More treatments after surgery are usually needed. These treatments target the remaining cells. […] Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams to the brain and spinal cord. Extra radiation is directed at the cancer cells. […] Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove.
  • #23 Pineoblastoma | The Brain Tumour Charity
    https://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/pineoblastoma/
    Pineoblastoma treatment can include one or more of the following: Surgery, Radiotherapy, Chemotherapy, Steroids. […] Surgery is an essential part of brain tumour treatment in general. It can aim to reduce the size of the tumour or remove it completely. […] However, due to the position of pineoblastomas deep within the brain, surgery may not always be a viable option. There could be a risk of damaging critical areas of the brain. […] Even if doctors deem that surgery is possible for a pineoblastoma, in most occasions it does not result in complete resection. This is because pineoblastomas, which are grade 4 tumours, may have already invaded other, difficult to reach parts of the brain or spine. […] Radiotherapy is the gold standard of treatment for pineoblastomas. Depending on factors such as whether cancerous cells have spread to other parts of the brain, your MDT will decide the type of radiotherapy you will have and at which dose. […] Pineoblastomas may also be treated with chemotherapy.
  • #24 Pineoblastoma in Adults: A Rare Case Successfully Treated with Multimodal Approach Including Craniospinal Irradiation Using Helical Tomotherapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6830851/
    Pineoblastomas (PBs) are rare and aggressive malignancies of the pineal gland. […] For their clinical behaviour and embryonal histology, PBs are often grouped together with medulloblastomas in clinical trials. […] He then received adjuvant craniospinal radiotherapy with a boost to the tumour bed followed by consolidation chemotherapy. […] Surgery followed by craniospinal irradiation and consolidation chemotherapy can be a safe and effective treatment option in adult PBs. […] The treatment of PB mainly derives from the paediatric practice and chemotherapy and craniospinal irradiation (CSI) are often advocated, for their propensity to disseminate along the cerebrospinal fluid (CSF). […] The treatment strategy agreed during multidisciplinary tumor board was to give adjuvant radiotherapy followed by consolidation chemotherapy.
  • #25 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Some patients with pineoblastoma may have surgery to help reduce the side effects from hydrocephalus. A surgeon will make a small opening in the third ventricle in the brain. This opening helps cerebrospinal fluid flow more freely to keep it from building up and causing harm. In other cases, doctors may place a shunt, a small tube that drains cerebrospinal fluid from the brain. The shunt may be temporary or permanent. […] Radiation therapy is often given after pineoblastoma surgery. Energy from the radiation can damage or destroy rapidly growing cancer cells. Doctors can also use radiation to shrink the tumor. The dose and amount of radiation therapy depend on: […] – Your child’s age […] – The type of tumor […] – If the disease has spread […] Radiation is not used in young children because it increases the risk of long-term side effects. In patients older than 3, radiation is usually given to the whole nervous system (craniospinal irradiation). A higher dose (boost) is given at the primary tumor site.
  • #26 Pineoblastoma | PPT
    https://www.slideshare.net/slideshow/pineoblastoma/46322327
    56) RT Dose for Non Germinomas British Oncology Society 2011 Non-metastatic disease (negative CSF-cytology, negative spinal MRI) 24Gy /15# @1.6 to WVRT Primary tumor bed additional boost to 54Gy Metastatic disease (positive CSF-cytology and / or positive spinal MRI) No Of # Dose/# Total dose (Gy) Duration (weeks) Brain 20 1.5 30 4 Spinal cord 20 1.5 30 4 Boost CNS +15 1.6 24 +3 Boost SC +10 1.6 16 +2 Total 35 54 To CNS 46 To S. mets 30 to CSI 7.
  • #27 Pineoblastoma in Adults: A Rare Case Successfully Treated with Multimodal Approach Including Craniospinal Irradiation Using Helical Tomotherapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6830851/
    The patient underwent a CT scan for radiotherapy (RT) planning purposes and was started on external beam RT using tomotherapy. […] All the treatment was performed using 6 MV photons with helical tomotherapy. […] The role of chemotherapy is unclear for adult patients and mainly derives from that of medulloblastomas. […] Therefore, it is reasonable to treat SPNETs like high risk medulloblastomas with CSI followed by chemotherapy. […] This case is an aggressive malignancy of the central nervous system which presented in an uncommon age group of the fifth decade. […] Despite the scarcity of literature evidence, the current case successfully demonstrates that aggressive surgery, whenever is technically feasible, followed by adjuvant RT and consolidation CHT may be the optimal strategy.
  • #28 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Some patients with pineoblastoma may have surgery to help reduce the side effects from hydrocephalus. A surgeon will make a small opening in the third ventricle in the brain. This opening helps cerebrospinal fluid flow more freely to keep it from building up and causing harm. In other cases, doctors may place a shunt, a small tube that drains cerebrospinal fluid from the brain. The shunt may be temporary or permanent. […] Radiation therapy is often given after pineoblastoma surgery. Energy from the radiation can damage or destroy rapidly growing cancer cells. Doctors can also use radiation to shrink the tumor. The dose and amount of radiation therapy depend on: […] – Your child’s age […] – The type of tumor […] – If the disease has spread […] Radiation is not used in young children because it increases the risk of long-term side effects. In patients older than 3, radiation is usually given to the whole nervous system (craniospinal irradiation). A higher dose (boost) is given at the primary tumor site.
  • #29 SSA – POMS: DI 23022.273 – Pineoblastoma – Childhood – 08/09/2023
    https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022273
    The first treatment for pineal region tumors is surgery to remove or reduce the size of the tumor and to obtain tissue to determine the tumor type. […] Treatments after surgery may include radiation therapy, chemotherapy, and/or clinical trials. Very young children are treated with a combination of chemotherapy medications to delay radiation therapy until the child is older. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available.
  • #30 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Our brain tumor clinical trials have led to the clinical use of better therapies in children such as lower-dose therapies, targeted therapy, and proton therapy. These treatments kill cancer cells while sparing healthy cells. They may lessen side effects that affect brain function after treatment for some types of brain tumors. […] The St. Jude Proton Therapy Center is the first center in the world that treats only children with this therapy.
  • #31 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #32 Pineoblastoma – Neurosurgery | UCLA Health
    https://www.uclahealth.org/medical-services/neurosurgery/conditions-treated/pineoblastoma
    Whenever possible, UCLA neurosurgeons use minimally invasive surgical techniques. […] UCLA neurosurgeons have extensive expertise in the minimally invasive, endoscopic treatment of hydrocephalus. Typically, this can be done by an endoscopic third ventriculostomy. In other cases, a shunt may be necessary. […] More than 70 percent of tumors are highly sensitive to radiation therapy. […] In many cases, stereotactic radiosurgery can provide excellent control of pineal tumors. UCLA uses the advanced Novalis shaped-beam stereotactic radiosurgery system, which is as or more effective than Gamma Knife. […] The choice is dependent on the type of tumor and the patient. Many tumor types need both radiation and chemotherapy.
  • #33 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #34 Mayo Clinic Health Library – Pineoblastoma | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20310674
    Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove. […] Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #35 Prognostic factors and outcome of pineoblastoma: 10 years single-center experience | Journal of the Egyptian National Cancer Institute | Full Text
    https://jenci.springeropen.com/articles/10.1186/s43046-021-00083-3
    HDCTH in pineoblastoma is increasingly used with or without the application of focal or craniospinal (CSI) irradiation. […] The extent of resection did not show a significant impact on the outcome in both age groups. […] Receiving adjuvant chemotherapy had a significant positive impact on survival, with 3-year EFS and OS of 49% for the chemotherapy group compared to 0% for both in the non-chemotherapy group. […] Radiotherapy administration significantly improved the outcome, with 3-year EFS and OS of 50.7%. […] The field of RTH had a significant impact on survival. CSI was associated with better outcome compared to focal irradiation. […] The survival of patients with objective responses (CR and PR) was significantly better than those with MR, SD, and PD, which raises the importance of intensifying induction by CTH/RTH to achieve a better outcome. […] A more aggressive approach using high-dose chemotherapy or CSI is required to improve infantile pineoblastomas dismal outcome.
  • #36 Pineoblastoma | PPT
    https://www.slideshare.net/slideshow/pineoblastoma/46322327
    28) RADIOTHERAPY Objective: To treat microscopic cancer cells Residual tumor with the goal of reducing its size or stopping its progression Prevent or treat spread through CSF Covering entire subarachnoid space. […] 30) German HIT-SKK87, HIT 91, and HIT-SKK92 (1987-1992, 1992- 1997) Subset of 11 patients with PB. If 3 years, surgery + chemo with RT deferred until 3 years or progression (n=5). If 3 years, surgery + chemo + CSI (35.2/22 + 20/10 boost) +/- maintenance chemo (n=6) Hinkes BG, J Neurooncol. 2007 Older children (3): 5/6 alive with median OS/PFS 7.9 years after chemo and RT. All had M0 disease Younger children (3): 0/5 alive with median OS 0.9 years and PFS 0.6 years. All had M1 disease and/or postop residual disease. Response to chemo lower, only 1/5 received RT. […] 31) Role of RT: All older children received it, benefited (PR-CR or stayed in CR). One younger child received who, after progressing on chemo, and showed PR to it Conclusion: Combined chemo and RT feasible and effective if 3 years. More intensified regimens necessary for 3 years Subsequent HIT trial for young children with supratentorial PNET investigates short dose-intense induction, followed by high-dose chemo and CSI.
  • #37 Prognostic factors and outcome of pineoblastoma: 10 years single-center experience | Journal of the Egyptian National Cancer Institute | Full Text
    https://jenci.springeropen.com/articles/10.1186/s43046-021-00083-3
    HDCTH in pineoblastoma is increasingly used with or without the application of focal or craniospinal (CSI) irradiation. […] The extent of resection did not show a significant impact on the outcome in both age groups. […] Receiving adjuvant chemotherapy had a significant positive impact on survival, with 3-year EFS and OS of 49% for the chemotherapy group compared to 0% for both in the non-chemotherapy group. […] Radiotherapy administration significantly improved the outcome, with 3-year EFS and OS of 50.7%. […] The field of RTH had a significant impact on survival. CSI was associated with better outcome compared to focal irradiation. […] The survival of patients with objective responses (CR and PR) was significantly better than those with MR, SD, and PD, which raises the importance of intensifying induction by CTH/RTH to achieve a better outcome. […] A more aggressive approach using high-dose chemotherapy or CSI is required to improve infantile pineoblastomas dismal outcome.
  • #38 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Pineoblastoma treatment focuses on safely removing as much of the tumor as possible with surgery, or with a combination of chemotherapy and surgery, followed by radiation and chemotherapy. […] Treatment may include: Surgery to get a sample for diagnosis and to remove part or all of the tumor. In some cases, it is not possible to remove all of the tumor in 1 surgery. In these cases, we either perform a biopsy, use chemotherapy to shrink the tumor, or do both in order to help brain surgeons (neurosurgeons) later fully remove the tumor. […] Radiation is an important part of treatment for pineoblastoma. The dose, location, and timing of radiation will depend on many factors. These include your child’s age, type of tumor, and if it has spread to other parts of the brain or spine. […] Chemotherapy (chemo) is important in the treatment of pineoblastoma. The exact type of chemotherapy and the length of treatment will depend on your child’s age and type of pineoblastoma.
  • #39 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49
    https://www.barrowneuro.org/condition/pineoblastoma/
    Radiation therapy is often used after surgery to reduce the risk of recurrence, especially for high-grade tumors. By relying on precisely aimed beams of radiation to destroy or shrink tumors in the body, radiation therapy damages the DNA of the tumor cells, which then lose their ability to reproduce and eventually die. […] Chemotherapy is a standard treatment for pineal tumors and can be used as a primary treatment or in combination with surgery and radiation. Neuro-oncologists often treat pineoblastomas with combination chemotherapy, which relies on multiple drugs that work together to destroy tumor cells more effectively. […] Due to pineoblastomas’ aggressive nature and high reoccurrence rate, long-term monitoring with consistent imaging studies is crucial—even after a successful initial treatment. […] While pineoblastomas are challenging to treat, some patients do achieve long-term remission, particularly with aggressive, multimodal treatment.
  • #40 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Chemotherapy is often used along with radiation therapy and surgery: […] – Before surgery, doctors sometimes use chemotherapy to shrink the tumor so it is easier to remove. […] – After surgery, chemotherapy can destroy cancer cells left behind. […] – In very young children, chemotherapy may be used to slow the growth of cancer until the child is old enough to get radiation safely. […] Several chemotherapy medicines may be used at the same time to treat pineoblastoma. Common treatments are vincristine, cyclophosphamide and cisplatin. Your child’s care team may use other types of chemotherapy. […] Successful removal of the tumor and treatment with radiation therapy are linked to better outcomes for children with pineoblastoma.
  • #41 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Pineoblastoma treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments also may be recommended. […] A brain surgeon, also called a neurosurgeon, works to remove as much of the pineoblastoma as possible. Sometimes all of the cancer can’t be removed. This is because pineoblastoma forms near important structures deep inside the brain. More treatments after surgery are usually needed. These treatments target the remaining cells. […] Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams to the brain and spinal cord. Extra radiation is directed at the cancer cells. […] Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove.
  • #42 Mayo Clinic Health Library – Pineoblastoma | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20310674
    Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove. […] Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #43 Pineoblastoma in Children and Teens – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/cancers/pineoblastoma.html
    Chemotherapy is often used along with radiation therapy and surgery: […] – Before surgery, doctors sometimes use chemotherapy to shrink the tumor so it is easier to remove. […] – After surgery, chemotherapy can destroy cancer cells left behind. […] – In very young children, chemotherapy may be used to slow the growth of cancer until the child is old enough to get radiation safely. […] Several chemotherapy medicines may be used at the same time to treat pineoblastoma. Common treatments are vincristine, cyclophosphamide and cisplatin. Your child’s care team may use other types of chemotherapy. […] Successful removal of the tumor and treatment with radiation therapy are linked to better outcomes for children with pineoblastoma.
  • #44
    https://scholars.duke.edu/individual/pub724146
    Treatment of patients with pineoblastoma with high dose cyclophosphamide. […] The outcome for patients with pineoblastoma has historically been very poor, with most patients dying of disseminated disease despite irradiation. […] Here we report the activity and toxicity of cyclophosphamide administered as a single agent at a dose schedule of 2 g/m2/day for 2 successive days at monthly intervals for a maximum of four courses. […] Amongst the six newly diagnosed patients, there were three patients demonstrating partial responses, and three had stable disease throughout the cyclophosphamide treatment period. […] All six patients are alive and disease free after further therapy. […] The major toxicity of high dose cyclophosphamide was hematopoietic, with one patient requiring a dose reduction after three courses due to prolonged thrombocytopenia. […] This study demonstrates the activity of high dose cyclophosphamide in the treatment of pineoblastoma and may serve as basis for the design of future studies of this tumor.
  • #45 Pineoblastoma – Brief information
    https://www.gpoh.de/kinderkrebsinfo/content/diseases/brain_tumours/pohpatinfomedullo120080109/literatur/index_eng.html
    Pineoblastoma Treatment, therapy […] Treatment of children and adolescents with pineoblastoma should take place in a children’s hospital with a paediatric oncology program. Only in such a childhood cancer centre, highly experienced and qualified staff (doctors, nurses and many more) is guaranteed, since they are specialized and focus on the diagnostics and treatment of children and teenagers with cancer according to the most advanced treatment concepts. The doctors in these centres collaborate closely with each other. Together, they treat their patients according to treatment plans (protocols) that are continuously optimised. The goal of the treatment is to achieve higher cure rates while avoiding side effects as much as possible. […] Current treatment concepts involve neurosurgical tumour removal, chemotherapy and, depending on the patients age, radiotherapy. In selected patients, high-dose chemotherapy followed by autologous stem cell transplantation may also be an option. […] The first step in treating a pineoblastoma is surgery. Goal of surgery is gross (microsurgical) total tumour removal. This means that at the end of the surgical procedure, no tumour tissue can be identified through the surgical microscope. Frequently, however, total tumour resection cannot be achieved due to the localization of these tumours. […] Since pineoblastomas tend to infiltrate adjacent tissue and, furthermore, often spread into other parts of the central nervous system via the cerebrovascular fluid (CSF), treating the tumour locally only is not sufficient. Therefore, surgery is followed by additional non-surgical treatment, comprising radiotherapy and/or chemotherapy. […] Chemotherapy uses drugs (so-called cytostatic agents or cytostatics) that can kill fast-dividing cells, such as cancer cells, or inhibit their growth, respectively. In order to eliminate as many of the cancer cells as possible, a combination of several cytostatics is usually applied. Frequently-used agents are, for example, carboplatin, etoposide, methotrexate, vincristine, cyclophosphamide, lomustine, cisplatin and/or temozolomide. […] Radiotherapy is done using energy-rich, electromagnetic radiation, given through the skin to the tumour region. Radiation causes DNA damage in tumour cells, thereby leading to cell death. Modern techniques, such as intensity-modulated radiotherapy (IMRT), help minimise radiation damages in healthy tissue. Instead of conventional radiotherapy with photons, particle-radiation with protons (proton therapy) can be an option in suitable cases as well. This type of radiotherapy allows to reduce the effects of radiation in healthy tissue even better and is, therefore, gaining an increasing importance in the treatment of children and teenagers with solid tumours. […] After maximal possible tumour removal, patients with non-metastasised pineoblastoma, who are older than four years of age, will receive radiation of the complete central nervous system (craniospinal radiotherapy), followed by an additional boost to the tumour site. Radiotherapy is followed by a so-called maintenance chemotherapy, which includes multiple cytostatic agents. In cases of metastasis, treatment will be intensified, for example by giving higher doses of radiotherapy combined with a preceding chemotherapy (induction chemotherapy). […] In children under four years of age, radiotherapy should be avoided or delayed in order to minimize the risk of serious late effects. Instead of radiotherapy after surgery, patients will receive chemotherapy with multiple agents. In some patients, radiotherapy may be an option later on. Some patients are also eligible for high-dose chemotherapy followed by autologous stem cell transplantation to increase the chances of survival.
  • #46 (PDF) Treatment of a Pineoblastoma in a 51-year-old patient
    https://www.academia.edu/55268413/Treatment_of_a_Pineoblastoma_in_a_51_year_old_patient
    The management of pinealoblastoma is multimodality approach, surgery followed with radiation and chemotherapy. […] The treatment for adults with pineoblastoma consists of surgery followed by craniospinal radiotherapy with or without addition of chemotherapy. […] A proper multidisciplinary management of pineoblastomas, which associates gross total microsurgical resection of the lesion and an adjuvant therapy determined by our neurooncology team based on accurate craniospinal adjuvant radiotherapy with boost of radiation on the tumoral bed, and when needed, an adequate but aggressive medulloblastoma-like chemotherapy, may improve the overall survival of these malignant lesions. […] Pineoblastoma patients, chemotherapy protocols are based on various alkylating or platinum-based agents, vincristine, etoposide, cyclophosphamide and are used in association with radiotherapy.
  • #47 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49
    https://www.barrowneuro.org/condition/pineoblastoma/
    Radiation therapy is often used after surgery to reduce the risk of recurrence, especially for high-grade tumors. By relying on precisely aimed beams of radiation to destroy or shrink tumors in the body, radiation therapy damages the DNA of the tumor cells, which then lose their ability to reproduce and eventually die. […] Chemotherapy is a standard treatment for pineal tumors and can be used as a primary treatment or in combination with surgery and radiation. Neuro-oncologists often treat pineoblastomas with combination chemotherapy, which relies on multiple drugs that work together to destroy tumor cells more effectively. […] Due to pineoblastomas’ aggressive nature and high reoccurrence rate, long-term monitoring with consistent imaging studies is crucial—even after a successful initial treatment. […] While pineoblastomas are challenging to treat, some patients do achieve long-term remission, particularly with aggressive, multimodal treatment.
  • #48 A Case Report of Adult Pineoblastoma Occurring in a Pregnant Woman | Anticancer Research
    https://ar.iiarjournals.org/content/39/5/2627
    For adult patients, only limited data are available with inconclusive results. Considering the biological similarity of medulloblastomas and pineoblastomas, for our patient adjuvant chemotherapy was proposed with CDDP plus CCNU plus vincristine. […] The advanced gestational stage of our patient facilitated the management of this rare tumor, since adjuvant therapies were safely performed after delivery. The integration of radiotherapy and chemotherapy after maximal safe resection revealed excellent outcomes.
  • #49 Pineoblastoma: Causes, Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24950-pineoblastoma
    Pineoblastoma is a cancerous (malignant) brain tumor in your pineal gland. This type of cancer grows quickly and can spread to tissue and fluid around your brain. […] While there isn’t a cure available for pineoblastoma, there are several treatment options. Your healthcare provider can surgically remove the tumor if it hasn’t spread. Pineoblastoma can be challenging to treat, which increases the risk of complications from this type of cancer. It may lead to symptoms that get worse over time and cause a shortened life expectancy. There’s a chance that it may come back after treatment. […] Treatment for pineoblastoma may include: Surgery to remove some or all of the tumor. Your surgeon will let you know what’s possible with surgery and the potential risks. Surgery to treat hydrocephalus. A surgeon may insert a shunt, or a small tube, to drain excess cerebrospinal fluid from building up around your brain. Radiation therapy. A common treatment after surgery, radiation therapy can shrink tumors or destroy cancer cells. Chemotherapy. Chemotherapy is a treatment option in addition to radiation therapy and surgery. Chemotherapy can shrink the tumor before surgery or destroy the remaining cancer cells after surgery. High-dose chemotherapy followed by autologous stem cell transplant. A healthcare provider will collect your own stem cells before receiving certain high-dose chemotherapy. Then you’ll receive an injection of the stem cells back into you after the chemotherapy.
  • #50 Pineoblastoma in Children Less Than Six Years of Age: The Head Start I, II and III Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8428681/
    Given the well-known and significant side-effects of brain irradiation on young children, the Head Start (HS) protocols have utilized an irradiation-sparing strategy. […] The HS protocols utilize high-dose induction chemotherapy followed by marrow-ablative chemotherapy and autologous hematopoietic cell rescue (HDCx/AuHCR). […] Radiotherapy was reserved for children over 6 years of age at diagnosis or those with residual tumor prior to consolidation. […] The consolidation chemotherapy was identical in all HS trials which included carboplatin, thiotepa and etoposide followed by a single AuHCR. […] Patients were eligible to receive radiotherapy based on their age at diagnosis as well as treatment response. […] The CSI dose ranged between 1836 Gy with a boost to the primary site of 4554 Gy.
  • #51 Pineoblastoma in Children Less Than Six Years of Age: The Head Start I, II and III Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8428681/
    In our cohort, only 3 patients received CSI before tumor progression, which limited our ability to draw any conclusions in regards to radiotherapy. […] However, CSI was associated with statistically significant improved OS, but not PFS. […] Among our patients, HDCx/AuHCR was associated with a positive impact on both PFS and OS. […] The high progression rate post-HDCx/AuHCR (80%) in our cohort indicates that one cycle of consolidation was not sufficient to provide any long-term survival. […] Therefore, treatment modalities including HDCx/AuHCR may provide a tool to delay the administration of CSI or focal irradiation. […] Future trials need to explore shorter and more intensive chemotherapy regimens to address the early chemotherapy failure rates noticed among young patients. […] Moreover, the high rate of progression post-consolidation HDCx/AuHCR may indicate the need to investigate post-consolidation maintenance therapies or intensification of consolidation with multiple cycles of HDCx/AuHCR.
  • #52
    https://link.springer.com/article/10.1007/s00401-019-02106-9
    Pineoblastoma is a rare embryonal tumor of childhood that is conventionally treated with high-dose craniospinal irradiation (CSI). […] The SJMB03 protocol comprised risk-adapted CSI (average-risk=23.4 Gy, high-risk=36 Gy) with radiation boost to the primary site and adjuvant chemotherapy. The SJYC07 protocol consisted of induction chemotherapy, consolidation with focal radiation (intermediate-risk) or chemotherapy (high-risk), and metronomic maintenance therapy. […] Our findings suggest superior outcome amongst older children with average-risk pineoblastoma treated with reduced-dose CSI. The identification of biologically and clinically distinct pineoblastoma subgroups warrants consideration of future molecularly-driven treatment protocols for this rare pediatric brain tumor entity.
  • #53 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #54 Pineoblastoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube
    https://www.augustahealth.com/disease/pineoblastoma/
    Radiosurgery. Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells. Radiosurgery is sometimes used to treat pineoblastoma that recurs. […] Clinical trials. Clinical trials are studies of new treatments. These studies give you a chance to try the latest treatment options, but the risk of side effects may not be known. Ask your doctor whether your child might be eligible to participate in a clinical trial.
  • #55 Clinical Trial: NCT02596828 – My Cancer Genome
    https://www.mycancergenome.org/content/clinical_trials/NCT02596828/
    Prospective Pilot Trial to Assess a Multimodal Molecular Targeted Therapy in Children, Adolescent and Young Adults With Relapsed or Refractory High-grade Pineoblastoma […] Children, adolescents and young adults with relapsed or treatment refractory pineoblastoma (rPB) represent a group of patients with dismal prognosis for whom a recommended standard salvage therapy is currently not available. […] The multimodal metronomic approach combining molecular targeted drugs (rapamycin and dasatinib) with conventional chemotherapy (irinotecan and temozolomide) will be investigated in a randomized fashion as new treatment strategy for patients with rPB. The intention is to assess the therapeutic benefit of molecular targeted drugs for the treatment of rPB. […] It is assumed that this combination of molecular targeted drugs with a tolerable conventional chemotherapy consisting of irinotecan and temozolomide can substantially improve the outcome of this patient population.
  • #56 Pineoblastoma Treatment | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
    https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/types/treatment
    After surgery, radiation therapy is often used to target any remaining cancer cells that couldn’t be removed, especially for tumors in challenging locations. […] Chemotherapy is often combined with surgery and radiation therapy to treat pineoblastoma. It uses powerful drugs to kill cancer cells and prevent their growth. […] For rare and challenging cancers like pineoblastoma, ongoing research is essential to expand treatment options. Clinical trials may offer access to new or experimental treatments that look promising in early studies but are not yet widely available. […] Given the aggressive nature of pineoblastoma, treatment focuses not only on the tumor but also on managing symptoms and maintaining quality of life. Palliative care offers essential support in helping patients manage pain, treatment side effects, and emotional challenges.
  • #57 Modeling germline mutations in pineoblastoma uncovers lysosome disruption-based therapy | Nature Communications
    https://www.nature.com/articles/s41467-020-15585-2
    Our in silico drug prediction analysis identified multiple tricyclic, antidepressant drugs such as NOR, which ranked at the top, as potential therapeutics for Rb/p53-deleted and Rb/p53-mutated PBs as well as for Dicer/p53- and Rb/Dicer1/p53-deficient lesions. NOR inhibited growth of primary Rb/p53- and Rb/Dicer1/p53-deficient PB cells as well as a human PB cell line. […] Lastly, we demonstrated that NOR synergizes with gemcitabine to effectively suppress PBs in our preclinical model, suggesting a new therapeutic approach for this rare, yet lethal pediatric cancer.
  • #58 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Our brain tumor clinical trials have led to the clinical use of better therapies in children such as lower-dose therapies, targeted therapy, and proton therapy. These treatments kill cancer cells while sparing healthy cells. They may lessen side effects that affect brain function after treatment for some types of brain tumors. […] The St. Jude Proton Therapy Center is the first center in the world that treats only children with this therapy.
  • #59 What Is Pineoblastoma? | St. Baldrick’s Blog
    https://blog.stbaldricks.org/what-is-pineoblastoma/
    Pineoblastoma is a rare and aggressive type of brain tumor that starts in the pineal gland, a small organ deep in the brain responsible for producing melatonin, the hormone that regulates sleep. […] Pineoblastoma is more aggressive than other types of pineal gland tumors. […] Treatment for pineoblastoma focuses on removing the tumor or reducing its size and managing symptoms. Treatment plans will likely consist of surgery, radiation, and/or chemotherapy. […] Researchers are looking at ways to avoid damage to healthy cells during treatment. […] A highly targeted form of proton therapy, known as “FLASH,” with an ultrahigh dose rate, shows less toxicity and improved healthy tissue sparing, while maintaining effectiveness in eradicating tumor cells. […] Researchers are also working to determine the effectiveness of combined proton therapy with immunotherapy on pineoblastoma.
  • #60 Pineoblastoma Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/pineoblastoma.html
    Our brain tumor clinical trials have led to the clinical use of better therapies in children such as lower-dose therapies, targeted therapy, and proton therapy. These treatments kill cancer cells while sparing healthy cells. They may lessen side effects that affect brain function after treatment for some types of brain tumors. […] The St. Jude Proton Therapy Center is the first center in the world that treats only children with this therapy.
  • #61 What Is Pineoblastoma? | St. Baldrick’s Blog
    https://blog.stbaldricks.org/what-is-pineoblastoma/
    Pineoblastoma is a rare and aggressive type of brain tumor that starts in the pineal gland, a small organ deep in the brain responsible for producing melatonin, the hormone that regulates sleep. […] Pineoblastoma is more aggressive than other types of pineal gland tumors. […] Treatment for pineoblastoma focuses on removing the tumor or reducing its size and managing symptoms. Treatment plans will likely consist of surgery, radiation, and/or chemotherapy. […] Researchers are looking at ways to avoid damage to healthy cells during treatment. […] A highly targeted form of proton therapy, known as “FLASH,” with an ultrahigh dose rate, shows less toxicity and improved healthy tissue sparing, while maintaining effectiveness in eradicating tumor cells. […] Researchers are also working to determine the effectiveness of combined proton therapy with immunotherapy on pineoblastoma.
  • #62 Recent Advances in Pineoblastoma Research: Molecular Classification, Modelling and Targetable Vulnerabilities
    https://www.mdpi.com/2072-6694/17/5/720
    Pineoblastoma is a difficult-to-treat pediatric brain cancer that has traditionally been viewed and treated as a single disease. However, over the past five years, pineoblastoma has been recognized as comprising four major subtypes driven by unique oncogenic alterations, affecting different age groups and leading to distinct clinical outcomes. […] Standard treatment, including surgical resection, radiation, and systemic chemotherapy, improves survival but compromises neurocognitive function. A better understanding of the disease and the generation of preclinical models may enable re-evaluation of previous clinical trials, development of precision therapeutic strategies and improve patient outcome. […] Current treatment options for PB include maximal resection of tumor bulk, radiotherapy and chemotherapy with drugs such as cisplatin, vincristine, and cyclophosphamide.
  • #63 Recent Advances in Pineoblastoma Research: Molecular Classification, Modelling and Targetable Vulnerabilities
    https://www.mdpi.com/2072-6694/17/5/720
    While gross total resection is ideal, it is clinically challenging due to the location of the pineal gland and its surrounding vascular structures. […] Chemotherapy has been brought into question as studies suggest it is only marginally more effective than radiotherapy and surgery. […] Thus, there is an urgent need to develop new therapies for PB that can improve patient outcomes while minimizing off-target cytotoxic effects. […] In summary, current therapy and past or ongoing clinical trials treat all PBs as a single disease, potentially overlooking opportunities to detect subtype-specific responses. For instance, MYC-PBs and RB1-PBs are sensitive to specific inhibitors, and these patients likely respond differently to treatment regimens.
  • #64 DNA methylation profiling from cerebrospinal fluid as a diagnostic tool for pineoblastoma | Acta Neuropathologica Communications | Full Text
    https://actaneurocomms.biomedcentral.com/articles/10.1186/s40478-025-01960-x
    In the most recent years, genome-wide DNA methylation profiling has been recognized as a suitable or rather essential diagnostic tool auxiliary to conventional histopathology for many tumor types, especially CNS tumors. In addition, liquid biopsy based on the analysis of tumor DNA in body fluids, has emerged as a promising approach in molecular profiling, early detection, and response to treatments in CNS tumors. […] DNA Methylation profiling of PB has allowed for the definition of the molecular identity of these tumors helping to discriminate them from other embryonal CNS tumors and to better clarify intertumoral heterogeneity among PB. […] Overall the pathological features were in keeping with the diagnosis of PB. Moreover, DNA methylation profiling confirmed the diagnosis of pineoblastoma, subtype miRNA processing altered 1, subclass A, matching the results on CSF. […] This study underscores the potential of CSF-based DNA methylation profiling as a valuable diagnostic tool, particularly in cases where tissue samples are unavailable, while highlighting the need to account for the specific nuances of liquid biopsy in the diagnostic process.
  • #65 Research Advances the Genetic Understanding of Pineoblastoma, a Rare Brain Cancer | Memorial Sloan Kettering Cancer Center
    https://www.mskcc.org/news/new-research-advances-genetic-understanding-pineoblastoma-rare-brain
    Experts say the findings about this type of tumor will lead to more accurate diagnoses and, potentially, to better treatments. […] The findings also point the way toward developing better therapies for the disease. […] Now this research in the lab is starting to bear fruit and help us better diagnose and treat patients, especially those with rare tumors like pineoblastoma. […] These distinct changes make pineoblastoma in children more aggressive. […] The new study found that in nonfamilial pineoblastoma, dysregulation of microRNAs occurs because of a different mutated gene, called DROSHA. […] Further studies will be needed to assess the role of ARRB2 and the other genes we found to be recurrently mutated in pineoblastoma, Dr. Karajannis concludes. But our findings open up new avenues of research toward novel therapies that exploit the abnormal processing of microRNA that we’ve observed.
  • #66 Pineoblastoma Treatment | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
    https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/types/treatment
    Successful treatment depends on factors including the tumor’s size and location, whether it has spread, and the patient’s age and overall health. Early detection and a comprehensive treatment plan can improve the chances of a positive outcome. […] Treatment for pineoblastoma typically involves a combination of surgery, radiation therapy, and chemotherapy. Each approach has a unique role in removing or controlling the tumor. […] Early diagnosis and a comprehensive treatment strategy are key to improving the pineoblastoma prognosis and increasing pineoblastoma life expectancy.
  • #67 Pineoblastoma Overview | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
    https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/types/overview
    Chemotherapy uses drugs to kill cancer cells or stop them from dividing. This can be particularly useful if the tumor has spread or if the tumor is not responding to surgery and radiation alone. […] Ongoing research into new treatments for pineoblastoma may offer additional options, particularly for those whose tumors do not respond well to standard therapies. Participating in clinical trials can provide patients with access to experimental therapies, including targeted drugs, immunotherapies, and newer radiation techniques, that are still being studied for their effectiveness. […] Prognosis varies widely based on factors such as the patients age, the extent of the tumor, and how well it responds to treatment. Generally, pineoblastomas are known to be aggressive tumors making outcomes difficult to predict. Survival rates tend to be lower for younger children, particularly under four years old, compared to adults due to differences in treatment response and the sensitivity of developing brains to aggressive therapies.
  • #68 Post-Surgical Prognosis of Patients with Pineoblastoma: A Systematic Review and Individual Patient Data Analysis with Trends over Time
    https://www.mdpi.com/2072-6694/15/13/3374
    This study demonstrates that EOR less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and tumor presentation in children less than three years old are associated with poorer prognosis. […] Pineoblastoma remains a severe rare disease, but survival outcomes are improving. […] Our analysis also demonstrated an improvement in outcomes since 2012. This improvement is likely driven at least in part by an increase in the proportion of pineoblastoma patients receiving GTR, which was also independently associated with time to mortality. […] These data stress the importance of GTR followed by adjuvant RT in maximizing OS.
  • #69 Prognostic factors and outcome of pineoblastoma: 10 years single-center experience | Journal of the Egyptian National Cancer Institute | Full Text
    https://jenci.springeropen.com/articles/10.1186/s43046-021-00083-3
    HDCTH in pineoblastoma is increasingly used with or without the application of focal or craniospinal (CSI) irradiation. […] The extent of resection did not show a significant impact on the outcome in both age groups. […] Receiving adjuvant chemotherapy had a significant positive impact on survival, with 3-year EFS and OS of 49% for the chemotherapy group compared to 0% for both in the non-chemotherapy group. […] Radiotherapy administration significantly improved the outcome, with 3-year EFS and OS of 50.7%. […] The field of RTH had a significant impact on survival. CSI was associated with better outcome compared to focal irradiation. […] The survival of patients with objective responses (CR and PR) was significantly better than those with MR, SD, and PD, which raises the importance of intensifying induction by CTH/RTH to achieve a better outcome. […] A more aggressive approach using high-dose chemotherapy or CSI is required to improve infantile pineoblastomas dismal outcome.
  • #70 Treatment outcomes for pediatric pineoblastoma: a single institute experience in Taiwan – Kang – Therapeutic Radiology and Oncology
    https://tro.amegroups.org/article/view/4422/html
    Due to its aggressive behavior, pineoblastomas are usually treated in a manner similar to high-risk medulloblastomas. Operation is the first choice of treatment, and maximal safe surgical resection is expected, although technically difficult due to the deep location of the pineal gland. Adjuvant radiotherapy with CSI and a local boost are usually given, and often combined with systemic chemotherapy to control or prevent distant metastasis. […] In summary, pediatric patients with pineoblastomas have a poor prognosis. In our study, survival was associated with age at diagnosis. Radiotherapy plus chemotherapy can provide long-term survival, and patients with distant metastasis at diagnosis may also achieve long-term survival with proper treatment.
  • #71 Pineoblastoma | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/pineoblastoma?lang=us
    Treatment is usually a combination of surgery, chemotherapy and radiation. […] Despite treatment, the prognosis has historically been poor, with a 5-year survival as low as 10%. In contrast, in 2021 a 5-year survival of 58-81% has been reported with median overall survival times of 4-8 years. […] The most important factors predicting a favorable outcome are early detection and treatment with at least chemotherapy, preferably a high dose regimen with stem cell rescue.
  • #72 Pineoblastoma | PPT
    https://www.slideshare.net/slideshow/pineoblastoma/46322327
    28) RADIOTHERAPY Objective: To treat microscopic cancer cells Residual tumor with the goal of reducing its size or stopping its progression Prevent or treat spread through CSF Covering entire subarachnoid space. […] 30) German HIT-SKK87, HIT 91, and HIT-SKK92 (1987-1992, 1992- 1997) Subset of 11 patients with PB. If 3 years, surgery + chemo with RT deferred until 3 years or progression (n=5). If 3 years, surgery + chemo + CSI (35.2/22 + 20/10 boost) +/- maintenance chemo (n=6) Hinkes BG, J Neurooncol. 2007 Older children (3): 5/6 alive with median OS/PFS 7.9 years after chemo and RT. All had M0 disease Younger children (3): 0/5 alive with median OS 0.9 years and PFS 0.6 years. All had M1 disease and/or postop residual disease. Response to chemo lower, only 1/5 received RT. […] 31) Role of RT: All older children received it, benefited (PR-CR or stayed in CR). One younger child received who, after progressing on chemo, and showed PR to it Conclusion: Combined chemo and RT feasible and effective if 3 years. More intensified regimens necessary for 3 years Subsequent HIT trial for young children with supratentorial PNET investigates short dose-intense induction, followed by high-dose chemo and CSI.
  • #73 Post-Surgical Prognosis of Patients with Pineoblastoma: A Systematic Review and Individual Patient Data Analysis with Trends over Time
    https://www.mdpi.com/2072-6694/15/13/3374
    This study demonstrates that EOR less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and tumor presentation in children less than three years old are associated with poorer prognosis. […] Pineoblastoma remains a severe rare disease, but survival outcomes are improving. […] Our analysis also demonstrated an improvement in outcomes since 2012. This improvement is likely driven at least in part by an increase in the proportion of pineoblastoma patients receiving GTR, which was also independently associated with time to mortality. […] These data stress the importance of GTR followed by adjuvant RT in maximizing OS.
  • #74 Pineoblastoma – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pineoblastoma/diagnosis-treatment/drc-20577743
    Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #75 Clinical Trials for Pineal-Region Tumors | Ivy Brain Tumor Center
    https://www.ivybraintumorcenter.org/brain-tumor-care/brain-tumor-types/pineal-region-tumors/
    Pineoblastomas are WHO grade IV tumors that are the most aggressive variety. […] Clinical trials for pineal-region tumors are uncommon, but since there are no proven therapies beyond surgery and radiotherapy, developing new drugs for these patients is critical. […] If you have a pineal-region tumor and have undergone MR imaging with evidence of tumor recurrence, you may be a candidate for one of our Phase 0 clinical trials. Our Phase 0 studies provide you with information you can use right now to make informed treatment decisions. This knowledge allows you to have a voice in your care and places some control back into your hands.
  • #76 Mayo Clinic Health Library – Pineoblastoma | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20310674
    Chemotherapy treats cancer with strong medicines. For pineoblastoma, chemotherapy is usually used after surgery or radiation therapy. Sometimes it’s used at the same time as radiation therapy. For larger pineoblastomas, chemotherapy may be used before surgery. This might shrink the cancer and make it easier to remove. […] Stereotactic radiosurgery focuses multiple beams of radiation on exact points to kill cancer cells. Radiosurgery is sometimes used to treat pineoblastoma that comes back after treatment. […] Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options. Side effects from these treatments may not be known. Ask your child’s healthcare professional whether your child might be able to participate in a clinical trial.
  • #77 Clinical Trials for Pineal-Region Tumors | Ivy Brain Tumor Center
    https://www.ivybraintumorcenter.org/brain-tumor-care/brain-tumor-types/pineal-region-tumors/
    Pineoblastomas are WHO grade IV tumors that are the most aggressive variety. […] Clinical trials for pineal-region tumors are uncommon, but since there are no proven therapies beyond surgery and radiotherapy, developing new drugs for these patients is critical. […] If you have a pineal-region tumor and have undergone MR imaging with evidence of tumor recurrence, you may be a candidate for one of our Phase 0 clinical trials. Our Phase 0 studies provide you with information you can use right now to make informed treatment decisions. This knowledge allows you to have a voice in your care and places some control back into your hands.
  • #78 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49
    https://www.barrowneuro.org/condition/pineoblastoma/
    Radiation therapy is often used after surgery to reduce the risk of recurrence, especially for high-grade tumors. By relying on precisely aimed beams of radiation to destroy or shrink tumors in the body, radiation therapy damages the DNA of the tumor cells, which then lose their ability to reproduce and eventually die. […] Chemotherapy is a standard treatment for pineal tumors and can be used as a primary treatment or in combination with surgery and radiation. Neuro-oncologists often treat pineoblastomas with combination chemotherapy, which relies on multiple drugs that work together to destroy tumor cells more effectively. […] Due to pineoblastomas’ aggressive nature and high reoccurrence rate, long-term monitoring with consistent imaging studies is crucial—even after a successful initial treatment. […] While pineoblastomas are challenging to treat, some patients do achieve long-term remission, particularly with aggressive, multimodal treatment.
  • #79 Pineoblastoma in Adults: A Rare Case Successfully Treated with Multimodal Approach Including Craniospinal Irradiation Using Helical Tomotherapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6830851/
    Pineoblastomas (PBs) are rare and aggressive malignancies of the pineal gland. […] For their clinical behaviour and embryonal histology, PBs are often grouped together with medulloblastomas in clinical trials. […] He then received adjuvant craniospinal radiotherapy with a boost to the tumour bed followed by consolidation chemotherapy. […] Surgery followed by craniospinal irradiation and consolidation chemotherapy can be a safe and effective treatment option in adult PBs. […] The treatment of PB mainly derives from the paediatric practice and chemotherapy and craniospinal irradiation (CSI) are often advocated, for their propensity to disseminate along the cerebrospinal fluid (CSF). […] The treatment strategy agreed during multidisciplinary tumor board was to give adjuvant radiotherapy followed by consolidation chemotherapy.
  • #80 Pineoblastoma Overview | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
    https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/types/overview
    Emerging therapies and improved surgical techniques have led to more favorable outcomes for many patients, and ongoing research continues to test new ways to improve survival rates and life expectancy. Advances in our understanding of cell biology have allowed for the development of personalized treatment approaches, helping clinicians tailor therapies to the specific characteristics of a patients tumor.
  • #81 Recent Advances in Pineoblastoma Research: Molecular Classification, Modelling and Targetable Vulnerabilities
    https://www.mdpi.com/2072-6694/17/5/720
    While gross total resection is ideal, it is clinically challenging due to the location of the pineal gland and its surrounding vascular structures. […] Chemotherapy has been brought into question as studies suggest it is only marginally more effective than radiotherapy and surgery. […] Thus, there is an urgent need to develop new therapies for PB that can improve patient outcomes while minimizing off-target cytotoxic effects. […] In summary, current therapy and past or ongoing clinical trials treat all PBs as a single disease, potentially overlooking opportunities to detect subtype-specific responses. For instance, MYC-PBs and RB1-PBs are sensitive to specific inhibitors, and these patients likely respond differently to treatment regimens.