Rak gruczołu naczyniówkowego
Diagnostyka i diagnoza
Choroid plexus carcinoma (CPC) to złośliwy guz splotu naczyniówkowego, klasyfikowany jako WHO stopień III, występujący głównie u dzieci poniżej 3 roku życia. Diagnostyka opiera się na szczegółowym badaniu neurologicznym oraz obrazowaniu MRI z kontrastem, które ujawnia nieregularne, kalafiorowate masy najczęściej w komorach bocznych (50%) i IV (40%), z cechami inwazji miąższu, obrzękiem okołoguzowym i przerzutami OUN. CT wykazuje hipodensyjne lub lekko hiperdensyjne zmiany z obszarami torbielowatymi i zwapnieniami. Punkcja lędźwiowa pozwala na ocenę rozsiewu poprzez wykrycie komórek nowotworowych i podwyższonego białka w PMR. Ostateczne rozpoznanie stawia się na podstawie histopatologii, gdzie obecność ≥5 mitoz/10 HPF, pleomorfizm jądrowy, martwica i zatarta struktura brodawkowata są kluczowe. Immunohistochemia wykazuje dodatniość cytokeratyn, wysoki indeks Ki-67 (>5%), zmienną ekspresję wimentyny, S-100 i GFAP oraz często ujemną transtyretynę i EMA. Badania molekularne, zwłaszcza mutacje TP53 (ok. 50% przypadków), mają znaczenie prognostyczne i wskazują na konieczność poradnictwa genetycznego, szczególnie w kontekście zespołu Li-Fraumeni.
- Diagnostyka Choroid Plexus Carcinoma
- Badanie neurologiczne i fizykalne
- Badania obrazowe
- Badanie płynu mózgowo-rdzeniowego
- Badania histopatologiczne
- Badania immunohistochemiczne
- Badania genetyczne i molekularne
- Diagnoza różnicowa CPC
- Techniki zaawansowane w diagnostyce CPC
- Znaczenie wczesnej i dokładnej diagnozy
- Badania kliniczne i nowe metody diagnostyczne
- Rokowanie i monitorowanie
- Wyzwania diagnostyczne i kierunki rozwoju
Diagnostyka Choroid Plexus Carcinoma
Choroid plexus carcinoma (CPC) to rzadki, złośliwy nowotwór wywodzący się z nabłonka splotu naczyniówkowego, występujący głównie u dzieci, szczególnie poniżej 3 roku życia. Według klasyfikacji WHO jest to guz III stopnia złośliwości o agresywnym przebiegu klinicznym. Prawidłowa diagnostyka ma kluczowe znaczenie dla zaplanowania optymalnej strategii terapeutycznej.123
Badanie neurologiczne i fizykalne
Diagnostyka choroid plexus carcinoma rozpoczyna się od dokładnego wywiadu i badania fizykalnego. Szczególnie istotne jest przeprowadzenie kompleksowego badania neurologicznego, które obejmuje ocenę:45
- Wzroku i ruchów gałek ocznych
- Słuchu
- Równowagi i koordynacji
- Odruchów neurologicznych
- Siły mięśniowej
- Funkcji nerwów czaszkowych
Badanie to pomaga określić, która część mózgu może być zajęta przez guz oraz dostarcza wstępnych informacji o lokalizacji i rozległości zmiany nowotworowej. U dzieci często obserwuje się objawy związane ze zwiększonym ciśnieniem śródczaszkowym i wodogłowiem spowodowanym przez guz.89
Badania obrazowe
Badania obrazowe stanowią kluczowy element diagnostyki choroid plexus carcinoma. Powszechnie stosowane techniki to:1011
Rezonans magnetyczny (MRI)
MRI z podaniem środka kontrastowego jest preferowaną metodą obrazowania w diagnostyce CPC. Badanie to dostarcza szczegółowych informacji na temat:1213
- Dokładnej lokalizacji guza (najczęściej w komorach bocznych – 50%, w IV komorze – 40%, w III komorze – 5% lub w wielu komorach – 5%)
- Wielkości i rozległości zmiany
- Charakterystyki guza – CPC ma typowo nieregularne granice i wygląd przypominający kalafiora
- Obecności nacieku do otaczającego miąższu mózgu
- Obecności obrzęku okołoguzowego
- Przerzutów w obrębie ośrodkowego układu nerwowego
Charakterystyczny obraz MRI choroid plexus carcinoma wykazuje heterogenną strukturę, silne wzmocnienie kontrastowe, cechy inwazji do otaczającego miąższu mózgu oraz obrzęk okołoguzowy, co odróżnia go od łagodnych brodawczaków splotu naczyniówkowego. Zaleca się wykonanie badania MRI całego ośrodkowego układu nerwowego przed operacją, aby wykluczyć rozsiew guza.1617
Tomografia komputerowa (CT)
CT jest często pierwszym badaniem wykonywanym, szczególnie u małych dzieci lub w sytuacjach nagłych. W obrazie CT, choroid plexus carcinoma prezentuje się jako:1819
- Hipodensyjna lub lekko hiperdensyjna masa z obszarami torbielowatymi
- Zmiana silnie wzmacniająca się po podaniu środka kontrastowego
- Możliwa obecność zwapnień (globalne zwapnienia w obrębie masy są bardziej charakterystyczne dla postaci złośliwej)
- Widoczne obszary martwicy
- Cechy obrzęku lub naciekania sąsiadującego miąższu mózgu
Badanie płynu mózgowo-rdzeniowego
Nakłucie lędźwiowe (punkcja lędźwiowa) z pobraniem płynu mózgowo-rdzeniowego (PMR) jest ważnym elementem diagnostyki CPC, szczególnie w celu wykrycia rozsiewu nowotworu. W badaniu PMR można zaobserwować:2122
- Podwyższone stężenie białka
- Obecność komórek nowotworowych (w przypadku rozsiewu drogą PMR)
Analiza PMR jest szczególnie istotna w przypadku planowania dalszego leczenia, w tym radioterapii obejmującej oś mózgowo-rdzeniową.2526
Badania histopatologiczne
Ostateczne rozpoznanie choroid plexus carcinoma opiera się na badaniu histopatologicznym materiału pobranego podczas zabiegu chirurgicznego. Według wytycznych WHO, aby postawić diagnozę CPC, powinno być obecnych co najmniej 4 z 5 następujących cech:2728
- Zwiększona aktywność mitotyczna (≥5 mitoz na 10 pól widzenia)
- Zwiększona komórkowość
- Pleomorfizm jądrowy
- Obecność martwicy
- Zatarcie struktury brodawkowatej (przejście do układu litego)
Badanie histopatologiczne jest niezbędne do odróżnienia CPC od innych guzów brodawkowatych, szczególnie u dorosłych, gdzie należy wykluczyć guzy przerzutowe, np. z nerki lub tarczycy.3132
Badania immunohistochemiczne
Badania immunohistochemiczne są pomocne w potwierdzeniu rozpoznania CPC oraz w różnicowaniu z innymi guzami. W diagnostyce wykorzystuje się następujące markery:3334
- Cytokeratyny (zwykle dodatnie)
- Wimentyna (ekspresja zmienna)
- S-100 (ekspresja zmienna)
- Transtyretyna (często ujemna w CPC, w przeciwieństwie do brodawczaków)
- GFAP (kwaśne białko włókienkowe gleju, ekspresja zmienna)
- EMA (antygen błonowy nabłonka, zwykle ujemny)
- Ki-67 (wysoki indeks proliferacyjny)
W diagnostyce różnicowej pomocne jest również określenie profilu ekspresji cytokeratyn 7 i 20, gdyż większość guzów splotu naczyniówkowego wykazuje fenotyp CK7-dodatni/CK20-ujemny, co może pomóc w różnicowaniu z przerzutami raków z innych narządów.3738
Badania genetyczne i molekularne
Badania genetyczne i molekularne odgrywają coraz ważniejszą rolę w diagnostyce i planowaniu leczenia CPC. Szczególnie istotne są:3940
- Badanie mutacji genu TP53 – obecna w około 50% przypadków CPC i związana z gorszym rokowaniem
- Badania w kierunku zespołu Li-Fraumeni (germinalnej mutacji TP53) – szczególnie u dzieci z CPC
- Profilowanie metylacji – może pomóc w wyodrębnieniu różnych podtypów molekularnych
- Badania markerów molekularnych guza – pomocne w planowaniu optymalnego leczenia
Zaleca się wykonanie badań genetycznych u wszystkich dzieci z CPC, a w przypadku wykrycia mutacji germinalnej TP53, wskazane jest poradnictwo genetyczne dla rodziny.4445
Diagnoza różnicowa CPC
Diagnostyka różnicowa choroid plexus carcinoma obejmuje szereg innych guzów ośrodkowego układu nerwowego, szczególnie tych o strukturze brodawkowatej lub lokalizacji wewnątrzkomorowej:4647
- Choroid plexus papilloma (WHO grade I) i atypical choroid plexus papilloma (WHO grade II) – łagodniejsze formy guzów splotu naczyniówkowego
- Brodawczak wyściółczaka (papillary ependymoma)
- Rdzeniak (primitive neuroectodermal tumor)
- Gwiaździak (astrocytoma)
- Zarodczak (germinoma)
- Potworniak (teratoma)
- Oponiaki (meningioma)
- Guzy przerzutowe do splotu naczyniówkowego (szczególnie u dorosłych)
- Atypical teratoid/rhabdoid tumor
Rozróżnienie między CPC a innymi guzami może być trudne wyłącznie na podstawie obrazowania, dlatego ostateczna diagnoza wymaga badania histopatologicznego.5051
Techniki zaawansowane w diagnostyce CPC
W diagnostyce CPC wykorzystuje się również zaawansowane techniki obrazowe, które mogą dostarczyć dodatkowych informacji pomocnych w różnicowaniu i planowaniu leczenia:5253
Obrazowanie perfuzyjne
Techniki obrazowania perfuzyjnego, takie jak arterial spin-labeling (ASL) czy dynamic susceptibility contrast (DSC), mogą być pomocne w przedoperacyjnym różnicowaniu między brodawczakiem a rakiem splotu naczyniówkowego. Badania wykazały, że CPC charakteryzuje się odmiennym wzorcem perfuzji w porównaniu do łagodnych brodawczaków, co może pomóc chirurgowi w dostosowaniu strategii operacyjnej, np. rozważeniu przedoperacyjnej embolizacji.5455
Obrazowanie dyfuzyjne
Badanie dyfuzji (DWI) i pomiar współczynnika dyfuzji (ADC) dostarczają informacji o gęstości komórkowej guza. Niższe wartości ADC są zwykle związane z większą złośliwością i gorszym rokowaniem, co może być pomocne w ocenie agresywności CPC.56
Badania angiograficzne
Angiografia może być pomocna w ocenie unaczynienia guza, szczególnie przed planowaną resekcją. CPC są zwykle silnie unaczynionymi guzami, a informacja o charakterze i źródle unaczynienia może być istotna dla chirurga.57
| Cechy diagnostyczne | Choroid Plexus Papilloma (Grade I) | Atypical Choroid Plexus Papilloma (Grade II) | Choroid Plexus Carcinoma (Grade III) |
|---|---|---|---|
| Struktura | Dobrze zachowana struktura brodawkowata | Częściowo zachowana struktura brodawkowata | Zatarta struktura brodawkowata, obszary lite |
| Aktywność mitotyczna | < 2 mitozy/10 HPF | 2-5 mitoz/10 HPF | ≥ 5 mitoz/10 HPF |
| Martwica | Brak | Może występować | Często obecna |
| Pleomorfizm jądrowy | Minimalny | Umiarkowany | Wyraźny |
| Inwazja do miąższu mózgu | Brak | Rzadko | Często |
| Indeks Ki-67 | < 1% | 1-5% | > 5%, zwykle 5-9% |
| Obrzęk okołoguzowy w MRI | Minimalny lub brak | Umiarkowany | Rozległy |
| Tendencja do rozsiewu | Bardzo rzadko | Rzadko | Często |
| Mutacja TP53 | Rzadko | Czasami | Często (około 50% przypadków) |
Znaczenie wczesnej i dokładnej diagnozy
Wczesna i dokładna diagnoza choroid plexus carcinoma ma kluczowe znaczenie dla wyniku leczenia:5859
- Umożliwia zaplanowanie optymalnej strategii chirurgicznej
- Pozwala na wcześniejsze wdrożenie odpowiedniego leczenia uzupełniającego
- Wpływa na decyzje dotyczące zastosowania chemioterapii i radioterapii
- Ma znaczenie prognostyczne – całkowita resekcja guza jest najważniejszym czynnikiem rokowniczym
- Umożliwia identyfikację pacjentów z predyspozycją genetyczną (np. zespół Li-Fraumeni), co ma znaczenie dla dalszej opieki i poradnictwa genetycznego
Ze względu na rzadkość występowania CPC, zwłaszcza u dorosłych, zaleca się, aby diagnostyka i leczenie odbywały się w specjalistycznych ośrodkach z doświadczeniem w leczeniu guzów mózgu, z udziałem interdyscyplinarnego zespołu specjalistów.6263
Badania kliniczne i nowe metody diagnostyczne
W związku z rzadkością występowania CPC, prowadzone są badania kliniczne mające na celu rozwój nowych metod diagnostycznych i terapeutycznych:6465
- Badania nad nowymi markerami diagnostycznymi dla guzów splotu naczyniówkowego
- Rozwój zaawansowanych technik obrazowania molekularnego
- Badania nad personalizacją leczenia w oparciu o profil genetyczny i molekularny guza
- Próby kliniczne dotyczące nowych metod leczenia, takich jak chemioterapia dotętnicza
Udział w badaniach klinicznych może być rozważany u pacjentów z nawrotowym lub opornym na standardowe leczenie CPC, szczególnie w przypadku niemożności całkowitej resekcji guza.6869
Rokowanie i monitorowanie
Rokowanie u pacjentów z CPC zależy od wielu czynników, w tym:7071
- Stopnia resekcji guza – całkowita resekcja wiąże się z istotnie lepszym rokowaniem
- Wieku pacjenta w momencie diagnozy
- Obecności rozsiewu w chwili rozpoznania
- Statusu mutacji TP53
- Odpowiedzi na leczenie uzupełniające
Pięcioletni wskaźnik przeżycia dla pacjentów z CPC wynosi około 40-65%, w porównaniu do 80-100% dla pacjentów z łagodnym brodawczakiem splotu naczyniówkowego. Całkowita resekcja guza zwiększa pięcioletnie przeżycie do około 58%, podczas gdy w przypadku resekcji częściowej wynosi ono około 20%.7475
Po zakończeniu leczenia, pacjenci wymagają regularnego monitorowania za pomocą badań obrazowych (MRI mózgu i rdzenia kręgowego) oraz badań neurologicznych, aby wcześnie wykryć ewentualny nawrót lub progresję choroby.7677
Wyzwania diagnostyczne i kierunki rozwoju
Diagnostyka choroid plexus carcinoma nadal stanowi wyzwanie ze względu na:7879
- Rzadkość występowania (stanowi mniej niż 1% wszystkich guzów mózgu)
- Trudności w przedoperacyjnym różnicowaniu między różnymi typami guzów splotu naczyniówkowego
- Brak specyficznych markerów serologicznych
- Ograniczoną liczbę badań klinicznych ze względu na rzadkość występowania
Kierunki rozwoju w diagnostyce CPC obejmują:8283
- Opracowanie zaawansowanych algorytmów diagnostycznych i nomogramów prognostycznych
- Rozwój technik obrazowania molekularnego
- Identyfikację nowych biomarkerów diagnostycznych i prognostycznych
- Szersze zastosowanie analizy genetycznej i molekularnej w diagnostyce i planowaniu leczenia
- Tworzenie międzynarodowych rejestrów i baz danych w celu lepszego zrozumienia tej rzadkiej choroby
Ze względu na rzadkość występowania CPC, szczególnie u dorosłych, ważne jest dzielenie się doświadczeniami i wynikami badań między różnymi ośrodkami, co może przyczynić się do poprawy diagnostyki i leczenia tego rzadkiego nowotworu.8586
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Materiały źródłowe
- #1 SSA – POMS: DI 23022.938 – Choroid Plexus Carcinoma – 08/11/2021https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022938
Choroid Plexus Carcinoma is a rare malignant cancer of the brain. It grows aggressively, invading surrounding tissue and impairing normal brain function. […] Diagnostic testing: Diagnosis of Choroid Plexus Carcinoma is made based on: […] Brain imaging including magnetic resonance imaging (MRI) and computerized tomography (CT) scans; and […] Choroid Plexus Carcinoma tumors appear as a distinct cauliflower-like shape on an MRI. […] Suggested MER for Evaluation: […] Imaging reports such as CT scan or MRI scan; […] Treatment of Choroid Plexus Carcinoma is often surgery followed by chemotherapy radiation therapy or both, which would satisfy listing 113.13 C.
- #2 Choroid plexus carcinoma | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/choroid-plexus-carcinoma?lang=us
Choroid plexus carcinomas are malignant neoplasms arising from the choroid plexus. They are classified as a WHO grade 3 tumor and while there is considerable overlap in imaging characteristics they carry a significantly poorer prognosis than both WHO grade 2 atypical choroid plexus papilloma, and WHO grade 1 choroid plexus papilloma. […] Choroid plexus carcinomas are designated as WHO grade 3 tumors in the 2021 WHO classification of CNS tumors. […] It has been suggested that to make the diagnosis of choroid plexus carcinoma, at least 4 of the following 5 features should be present: increased mitotic rate: 5 per 10 high-power fields, increased cellularity, nuclear pleomorphism, necrosis, blurred papillary structure. […] The immunophenotype of choroid plexus carcinomas is similar to that of choroid plexus papillomas, with both S11 and transthyretin more likely to be negative.
- #3 Choroid plexus carcinoma: A case report and literature reviewhttps://www.oatext.com/Choroid-plexus-carcinoma-A-case-report-and-literature-review.php
Choroid plexus carcinoma is a rare tumor representing less than 1% of all brain tumors. In adult, the incidence is extremely rare making the diagnosis difficult. […] In adult however, due to its extremely rare occurrence, the diagnosis of choroid plexus carcinoma should be made with caution as it more frequently resembles a metastatic papillary tumor such as from kidney and thyroid. […] The clinical presentation, pathology and management are discussed. […] Most of the diagnosis of choroid plexus carcinoma is made in paediatrics age group and rarely reported in adult. If present in adult, it should be interpreted with caution and patient should be investigated thoroughly to exclude metastatic tumors particularly from renal and thyroid. […] In investigating the tumor, both CT scan and MRI is needed, and sometimes angiography will be useful especially if we are suspecting a highly vascularized tumor.
- #4 Choroid plexus carcinoma – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/choroid-plexus-carcinoma/diagnosis-treatment/drc-20578787
Tests and procedures used to diagnose choroid plexus carcinoma include: […] During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor. […] Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery. […] Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling. […] If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors.
- #5 SSA – POMS: DI 23022.938 – Choroid Plexus Carcinoma – 08/11/2021https://secure.ssa.gov/poms.nsf/lnx/0423022938
Choroid Plexus Carcinoma is a rare malignant cancer of the brain. It grows aggressively, invading surrounding tissue and impairing normal brain function. […] Diagnosis of Choroid Plexus Carcinoma is made based on: Physical and neurological examination; Brain imaging including magnetic resonance imaging (MRI) and computerized tomography (CT) scans; and Genetic testing. […] Choroid Plexus Carcinoma tumors appear as a distinct cauliflower-like shape on an MRI. […] Suggested MER for Evaluation: Clinical history and examination that describes the diagnostic features of the impairment; Imaging reports such as CT scan or MRI scan; Operative reports; and Pathology/biopsy reports.
- #6 Choroid Plexus Brain Tumor | Boston Children’s Hospitalhttps://www.childrenshospital.org/conditions/choroid-plexus-brain-tumor
Choroid plexus carcinoma (CPCs) grow more aggressively and are more likely to spread than either APPs or CPPs. CPCs are less common, accounting for 25-35 percent of all choroid plexus tumors. […] The first step in treating your child is forming an accurate and complete diagnosis. Choroid plexus tumors are most commonly diagnosed from imaging studies and biopsy. […] Choroid plexus tumor diagnostic tests may include: A physical exam and complete medical history, A neurological function test of reflexes, muscle strength, eye and mouth movement, coordination, and alertness, Magnetic resonance imaging (MRI), Lumbar puncture (spinal tap). […] Patients with choroid plexus papilloma do extremely well with surgery. Children with choroid plexus carcinoma have a more guarded prognosis and often require aggressive treatment.
- #7 Choroid Plexus Carcinoma Diagnosis And Treatment – Klarity Health Libraryhttps://my.klarity.health/choroid-plexus-carcinoma-diagnosis-and-treatment/
Choroid plexus carcinoma poses a serious challenge in the world of brain cancers. It not only poses difficulty in diagnosis, because of non-specific symptoms, but it primarily affects children who cannot express themselves. With the use of MRI however, we can easily discover tumours, giving hints about the severity and location of the tumour, along with providing guidance to surgeons for treatment. […] This article explores the process of diagnosing choroid plexus carcinoma, along with the possible treatment options currently available. […] Following the reporting of these symptoms, a clinical evaluation is the next step in the diagnosis of CPC. This often includes a complete medical history followed by the physical exam, which can include neurological function tests for reflexes, coordination, alertness, muscle strength, and eye and mouth movement.
- #8 Choroid plexus carcinoma | Beacon Health Systemhttps://www.beaconhealthsystem.org/library/diseases-and-conditions/choroid-plexus-carcinoma?content_id=CON-20308119
Tests and procedures used to diagnose choroid plexus carcinoma include: […] During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor. […] Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery. […] Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling. […] If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors. The management of this cancer is complex. Seek out a medical center that has experience with this cancer and can offer the latest treatment options for your child.
- #9 Tumors of the Choroid Plexus in New Jersey | Centers for Neurosurgery, Spine & Orthopedicshttps://www.cnsomd.com/surgery-conditions/brain-tumors/choroid-pluxus-tumor/
Choroid plexus tumors form in the central nervous system, which includes the brain, cerebellum, and spinal cord. […] The expert medical team at Centers for Neurosurgery, Spine & Orthopedics has experience with the different types of tumors that grow in the choroid plexus. […] Choroid plexus carcinoma is malignant, or cancerous. […] These malignant tumors can affect structures in the brain similarly to Papilloma. However, since they are cancerous, they may also spread throughout the central nervous system via the cerebrospinal fluid. […] Initial examination by the physicians at Centers for Neurosurgery, Spine & Orthopedics will likely include a physical test of balance, vision, hearing, and coordination. […] Imaging of the brain with a magnetic resonance imaging (MRI) will help with identifying any tumors, and their placement within the central nervous system.
- #10 SSA – POMS: DI 23022.938 – Choroid Plexus Carcinoma – 08/11/2021https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022938
Choroid Plexus Carcinoma is a rare malignant cancer of the brain. It grows aggressively, invading surrounding tissue and impairing normal brain function. […] Diagnostic testing: Diagnosis of Choroid Plexus Carcinoma is made based on: […] Brain imaging including magnetic resonance imaging (MRI) and computerized tomography (CT) scans; and […] Choroid Plexus Carcinoma tumors appear as a distinct cauliflower-like shape on an MRI. […] Suggested MER for Evaluation: […] Imaging reports such as CT scan or MRI scan; […] Treatment of Choroid Plexus Carcinoma is often surgery followed by chemotherapy radiation therapy or both, which would satisfy listing 113.13 C.
- #11 Choroid Plexus Tumor Symptoms and Treatment | UPMChttps://www.upmc.com/services/neurosurgery/brain/conditions/brain-tumors/choroid-plexus-tumor
To help diagnose choroid plexus tumors, your doctor will ask you about your symptoms and order imaging tests. […] Your doctor can identify a choroid plexus tumor using imaging studies such as MRI or CT scans. […] People with choroid plexus carcinomas a malignant (cancerous) type of choroid plexus tumor usually require additional treatments, such as radiation therapy and chemotherapy. […] Chemotherapy treatment uses drugs to stop the growth of cancer cells and may be used to treat choroid plexus carcinoma.
- #12 Choroid Plexus Tumors Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York Cityhttps://www.neurosurgery.columbia.edu/patient-care/conditions/choroid-plexus-tumors
Choroid plexus carcinomas, accounting for the remaining 10 to 20 percent of choroid plexus tumors, are malignant and grow more quickly than choroid plexus papillomas. These are Grade III and can invade surrounding brain tissue. […] The key component to diagnosis, as is the case for nearly all brain tumors, is imaging. Magnetic resonance imaging (MRI) scans or computed tomography (CT) scans can be used, although MRI tends to be used more often. Most often the study will be done with contrast enhancement. […] Choroid plexus carcinomas may not be amenable to total removal. So as not to risk harming important, healthy structures, neurosurgeons perform subtotal resection in such situations. Adjuvant treatment with radiotherapy, chemotherapy or both may be needed to treat the residual tumor and prevent tumor recurrence.
- #13 Choroid plexus carcinoma | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/choroid-plexus-carcinoma?lang=us
Choroid plexus carcinomas are markedly enhancing intraventricular tumors, usually arising in the trigone of a lateral ventricle and invading adjacent brain parenchyma. […] The tumors may have CSF seeding, therefore imaging of the entire neural axis is recommended prior to surgery. […] Choroid plexus carcinomas are rapidly growing tumors with a 40% 5-year survival. TP53 mutation, brain invasion and CSF seeding are considered poor prognostic factors. […] Surgical en-bloc resection is the mainstay of treatment and can result in a cure, achieved in as many as 50% of cases, but this result has only been reported in some selected series.
- #14 Choroid plexus tumors, a systematic reviewhttps://epos.myesr.org/poster/esr/ecr2024/C-20424/findings%20and%20procedure%20details
Choroid plexus carcinoma is a malignant aggressive lesion, WHO grade 3, with high vascularity and various dimensions, usually large. TP53 gene mutations are often associated with choroid plexus carcinoma. […] Choroid plexus carcinoma has hypercellular character, with multiple mitoses, cerebral parenchyma invasion and cerebrospinal fluid metastatic seeds. […] Due to increased intracranial pressure, there are several symptoms that may show. The patients usually have symptoms like headache, nausea and vomiting or neurologic defects. […] This neoplasm can show tumoral infiltration in the surrounding structures, peritumoral edema and CSF spread and the most reliable imaging techniques are CT and MRI. Choroid plexus carcinoma is a complex tumor, with some typical characteristics like: lobulated contour, well-defined, large dimensions or mixed content. Tumor density on CT can be intermediate or high, with irregular border. Heterogeneous appearance of the lesion is frequently encountered, with cystic areas, necrotic, calcified or hemorrhagic zones. Choroid plexus carcinoma is strongly iodophil on contrast enhanced CT, with heterogenous pattern due to cysts / necrosis / calcifications. Other associated signs, well seen on CT are hydrocephalus or perilesional edema. On T1WI the signal is isointense or hypointense, mostly heterogeneous. T2WI sequence is optimal to perceive necrosis/cysts (appear hyperintense), calcifications or blood (depending on the age of hemorrhage) or sometimes, flow voids (loss of signal). T2WI and FLAIR sequences highlight periventricular edema, which means invasion in the white matter. Transependymal edema is the consequence of hydrocephalus. ADC values are very important, the lower the value, the worse the prognosis. The tumor enhances inhomogeneous, but intense. […] Choroid plexus papilloma is sometimes very similar with choroid plexus carcinoma on MRI, and only the biopsy can make the difference between them.
- #15 Choroid Plexus Papilloma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findingshttps://emedicine.medscape.com/article/250795-workup
Imaging studies are required in patients presenting with presumed mass effect. For very young children, CT scanning is the procedure of choice. CT scan demonstrates a homogeneously hypodense to slightly hyperdense enhancing mass with cystic areas. This mass may be sizable and may be associated with hydrocephalus. Punctate calcifications, observed in 20% of tumors, are more indicative of a papilloma, whereas global calcification throughout the mass is more indicative of carcinoma. […] Choroid plexus carcinoma is generally associated with edema or invasion into the surrounding parenchyma, which may be observed as an area of enhancement. Areas suggestive of necrosis by imaging studies generally are not a feature of CPP but may be seen in choroid plexus carcinoma. […] For older children and adults, MRI is indicated. The appearance of a CPP on MRI (with and without contrast) is similar to that on CT scan and shows intermediate-to-strong intensity on both T1- and T2-weighted images. The malignant choroid plexus carcinoma appears more heterogeneous than the papilloma and often shows adjacent parenchymal invasion or surrounding edema.
- #16 Choroid Plexus Carcinoma Diagnosis And Treatment – Klarity Health Libraryhttps://my.klarity.health/choroid-plexus-carcinoma-diagnosis-and-treatment/
It should be noted however that Li Fraumeni is quite rare as well. Brain imaging […] An MRI is incredibly helpful not only as a firm and reliable method of diagnosing tumours such as CPC, but it is also useful for future surgeries, as it allows surgeons to adapt their method to the size and location of the tumour. […] Therefore, MRIs are useful in initial diagnosis and pre-surgical planning, but also incredibly useful for checking patients after treatment, as choroid plexus tumours can come back. […] With surgery, you also obtain tissue for analysis to be able to determine the specific tissue type and grade of the CPC, which could not be done through MRIs. […] Although treatment of grade 1 and grade 2 choroid plexus tumours has shown to be successful in fully removing the benign growth, it doesn’t have the same success with CPC because of its tendency to spread to other parts of the CNS.
- #17 Choroid plexus tumours on MRI: similarities and distinctions in different grades | Cancer Imaging | Full Texthttps://cancerimagingjournal.biomedcentral.com/articles/10.1186/s40644-019-0200-1
The therapeutic planning varies for different grades of choroid plexus tumours (CPTs). […] The aim of this study was to define the similarities and distinctions among MRIs for different grades of CPTs, providing more guidance for clinical decisions. […] We reviewed the MRI findings in 35 patients with CPT verified by surgical pathology, including 18 choroid plexus papillomas (CPPs, grade I), 11 atypical choroid plexus papillomas (aCPPs, grade II), and 6 choroid plexus carcinomas (CPCs, grade III). […] The grades of CPTs were statistically associated with tumour longest diameter (r_s=0.68, P0.001), internal morphology (2=10.32, P=0.016), necrosis (Z=2.27, P=0.023), and oedema diameter (r_s=0.72, P0.001). […] CPTs typically appeared as intraventricular papillary or lobulated lesions, often accompanied by hydrocephalus.
- #18 Choroid Plexus Papilloma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findingshttps://emedicine.medscape.com/article/250795-workup
Imaging studies are required in patients presenting with presumed mass effect. For very young children, CT scanning is the procedure of choice. CT scan demonstrates a homogeneously hypodense to slightly hyperdense enhancing mass with cystic areas. This mass may be sizable and may be associated with hydrocephalus. Punctate calcifications, observed in 20% of tumors, are more indicative of a papilloma, whereas global calcification throughout the mass is more indicative of carcinoma. […] Choroid plexus carcinoma is generally associated with edema or invasion into the surrounding parenchyma, which may be observed as an area of enhancement. Areas suggestive of necrosis by imaging studies generally are not a feature of CPP but may be seen in choroid plexus carcinoma. […] For older children and adults, MRI is indicated. The appearance of a CPP on MRI (with and without contrast) is similar to that on CT scan and shows intermediate-to-strong intensity on both T1- and T2-weighted images. The malignant choroid plexus carcinoma appears more heterogeneous than the papilloma and often shows adjacent parenchymal invasion or surrounding edema.
- #19https://link.springer.com/article/10.1007/s42399-025-01831-5
According to the latest WHO classification (5th edition), choroid plexus carcinoma (CPC) is a rare, grade 3 malignancy originating from the choroid plexus, predominantly affecting pediatric patients. […] In adults, CPC is uncommon, and the limited data available have hindered the development of standardized treatment protocols. […] Definitive diagnosis relies on histopathology. Treatment typically involves surgical resection, while adjuvant therapies offer limited benefit to overall survival (OS). […] There is currently no consensus on a standard treatment approach, and outcomes remain unsatisfactory. […] The initial diagnosis is based on computed tomography (CT), which reveals hypodense, heterogeneous lesions, and, in some cases, calcifications. […] The definitive diagnosis depends on histopathological findings and can be primary, mixed, or secondary to CPP.
- #20 Choroid plexus tumors, a systematic reviewhttps://epos.myesr.org/poster/esr/ecr2024/C-20424/findings%20and%20procedure%20details
Choroid plexus carcinoma is a malignant aggressive lesion, WHO grade 3, with high vascularity and various dimensions, usually large. TP53 gene mutations are often associated with choroid plexus carcinoma. […] Choroid plexus carcinoma has hypercellular character, with multiple mitoses, cerebral parenchyma invasion and cerebrospinal fluid metastatic seeds. […] Due to increased intracranial pressure, there are several symptoms that may show. The patients usually have symptoms like headache, nausea and vomiting or neurologic defects. […] This neoplasm can show tumoral infiltration in the surrounding structures, peritumoral edema and CSF spread and the most reliable imaging techniques are CT and MRI. Choroid plexus carcinoma is a complex tumor, with some typical characteristics like: lobulated contour, well-defined, large dimensions or mixed content. Tumor density on CT can be intermediate or high, with irregular border. Heterogeneous appearance of the lesion is frequently encountered, with cystic areas, necrotic, calcified or hemorrhagic zones. Choroid plexus carcinoma is strongly iodophil on contrast enhanced CT, with heterogenous pattern due to cysts / necrosis / calcifications. Other associated signs, well seen on CT are hydrocephalus or perilesional edema. On T1WI the signal is isointense or hypointense, mostly heterogeneous. T2WI sequence is optimal to perceive necrosis/cysts (appear hyperintense), calcifications or blood (depending on the age of hemorrhage) or sometimes, flow voids (loss of signal). T2WI and FLAIR sequences highlight periventricular edema, which means invasion in the white matter. Transependymal edema is the consequence of hydrocephalus. ADC values are very important, the lower the value, the worse the prognosis. The tumor enhances inhomogeneous, but intense. […] Choroid plexus papilloma is sometimes very similar with choroid plexus carcinoma on MRI, and only the biopsy can make the difference between them.
- #21 Choroid plexus carcinoma: a report of two cases and review of the literature – PubMedhttps://pubmed.ncbi.nlm.nih.gov/8837074/
Choroid plexus carcinoma is a rare intracranial neoplasm, affecting mainly very young children. The diagnosis of choroid plexus carcinoma is based on histological examination. […] Investigation at diagnosis should include examination of the cerebrospinal fluid and magnetic resonance imaging of the spinal cord. […] Total surgical resection of the tumor should be attempted. Both radiotherapy and chemotherapy are used as adjuvant therapies for primary tumors. No clear difference in effectiveness of these therapies could be found. However craniospinal irradiation seems to be more effective when leptomeningeal seeding is present.
- #22https://www.aurorahealthcare.org/services/neuroscience/brain-skull-base-care/brain-tumor/choroid-plexus-tumor
First, your doctor will conduct a physical exam. Youll talk about your potential choroid plexus tumor symptoms and your doctor may order imaging tests such as an MRI, a CT scan or a lumbar puncture (spinal tap), in which a cerebrospinal fluid (CSF) sample is taken. This helps to show whether cancer cells have spread. […] Your treatment will depend on the type of choroid plexus tumor you have as well as its size and location.
- #23 What Is Choroid Plexus Carcinoma? – Klarity Health Libraryhttps://my.klarity.health/what-is-choroid-plexus-carcinoma/
When you or a loved one receives a choroid plexus carcinoma (CPC) diagnosis, questions and concerns naturally arise. To make informed decisions about treatment and care, it’s vital to understand this exceptionally rare condition. […] Diagnosing CPC is a complex process that typically involves a combination of medical history, physical examinations, and advanced imaging studies. Common diagnostic methods include: […] MRI is a powerful imaging technique that provides detailed brain images, helping identify the presence and location of choroid plexus carcinoma. It reveals the tumour’s size, extent, and characteristics. […] In many cases, a biopsy is essential to confirm the diagnosis of choroid plexus carcinoma. A biopsy involves surgically removing a small sample of the tumour for laboratory analysis. This procedure not only confirms the presence of cancerous cells but also provides information about the tumour’s grade and extent.
- #24 Read-Only Case Details Reviewed: Apr 2008https://www.askjpc.org/vspo/show_page.php?id=K0J4cUIwMjZOOEhYZnozTlVDTlRCUT09
Mitotic index, cell density, and multilayering of papillae are the main significant histologic markers of malignancy for grading CPTs in dogs. […] CPC usually has a higher CSF protein concentration than CPP. […] Choroid plexus carcinoma (grade III; CPC): Piling up of epithelium, invasion of adjacent neuropil, desmoplasia, and/or anaplastic features (nuclear atypia, loss of papillary pattern with transition to cellular sheets, increased mitotic rate (5 per 10 HPF), necrosis). […] All CPT grades may metastasize throughout the ventricular system or subarachnoid space with implantation in the ependymal or meninges respectively; even histologically benign CPP can behave as aggressive carcinomas with widespread metastasis. […] Cytology: Papillomas and carcinomas may only be differentiated via histopathological examination as they have very similar cytological appearance.
- #25 Choroid plexus carcinoma: a report of two cases and review of the literature – PubMedhttps://pubmed.ncbi.nlm.nih.gov/8837074/
Choroid plexus carcinoma is a rare intracranial neoplasm, affecting mainly very young children. The diagnosis of choroid plexus carcinoma is based on histological examination. […] Investigation at diagnosis should include examination of the cerebrospinal fluid and magnetic resonance imaging of the spinal cord. […] Total surgical resection of the tumor should be attempted. Both radiotherapy and chemotherapy are used as adjuvant therapies for primary tumors. No clear difference in effectiveness of these therapies could be found. However craniospinal irradiation seems to be more effective when leptomeningeal seeding is present.
- #26 Choroid plexus tumours – Brief informationhttps://www.gpoh.de/kinderkrebsinfo/content/diseases/brain_tumours/choroid_plexus_tumours/brief_information/index_eng.html
Once the diagnosis of a choroid plexus tumour has been confirmed by histology, additional tests are required to assess the extent of the disease within the central nervous system (CNS). […] In order to secure the validity of the results, having the MRI results as well as the cerebrospinal fluid reviewed by a second expert (so-called referee expert) is recommended. […] Further tests prior to treatment include examination of the heart function by electrocardiography (ECG) and/or echocardiography (echo).
- #27 Choroid plexus carcinoma | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/choroid-plexus-carcinoma?lang=us
Choroid plexus carcinomas are malignant neoplasms arising from the choroid plexus. They are classified as a WHO grade 3 tumor and while there is considerable overlap in imaging characteristics they carry a significantly poorer prognosis than both WHO grade 2 atypical choroid plexus papilloma, and WHO grade 1 choroid plexus papilloma. […] Choroid plexus carcinomas are designated as WHO grade 3 tumors in the 2021 WHO classification of CNS tumors. […] It has been suggested that to make the diagnosis of choroid plexus carcinoma, at least 4 of the following 5 features should be present: increased mitotic rate: 5 per 10 high-power fields, increased cellularity, nuclear pleomorphism, necrosis, blurred papillary structure. […] The immunophenotype of choroid plexus carcinomas is similar to that of choroid plexus papillomas, with both S11 and transthyretin more likely to be negative.
- #28 Pathology Outlines – Choroid plexus tumors (papilloma, atypical papilloma, carcinoma)https://www.pathologyoutlines.com/topic/cnstumorchoroidplexuspapillomas.html
Choroid plexus tumors are rare neoplasms derived from the choroid plexus epithelium. They occur within the ventricle system of the brain. […] Diagnostically, transthyretin (TTR), KIR7.1, cytokeratin and Ki67 immunohistochemistry are most helpful. […] Clinical features, imaging (CT or preferably MRI). Biopsy with histologic examination for definitive diagnosis. […] Choroid plexus carcinoma (CPC, WHO grade 3): Frankly malignant. High cellularity, hyperchromatic nuclei and nuclear pleomorphism. Blurring of papillary pattern and solid arrangement. Frequent mitoses (> 5/10 high power fields), necrosis, with or without brain invasion. […] Recent studies suggest 3 distinct molecular entities based on methylation profiling, patient age and tumor location. […] TP53 sequencing and testing for germ line TP53 mutation is advisable in children with choroid plexus carcinoma.
- #29 Choroid Plexus Carcinomahttps://www.webpathology.com/images/neuropath/non-glial-tumors/choroid-plexus-tumors/33299
Choroid plexus carcinomas show papillary architecture in better differentiated foci (as shown in this image). Poorly-differentiated areas are generally solid and composed of highly atypical cells arranged in sheets. The hallmarks of malignancy include at least 4 of the following features: nuclear pleomorphism, 5 mitoses/10 HFP, increased cellularity, necrosis, solid architecture, and invasion of brain parenchyma. […] Most cases of choroid plexus carcinoma are seen in children and they usually present before 3 yrs. of age.
- #30 Read-Only Case Details Reviewed: Apr 2008https://www.askjpc.org/vspo/show_page.php?id=K0J4cUIwMjZOOEhYZnozTlVDTlRCUT09
Mitotic index, cell density, and multilayering of papillae are the main significant histologic markers of malignancy for grading CPTs in dogs. […] CPC usually has a higher CSF protein concentration than CPP. […] Choroid plexus carcinoma (grade III; CPC): Piling up of epithelium, invasion of adjacent neuropil, desmoplasia, and/or anaplastic features (nuclear atypia, loss of papillary pattern with transition to cellular sheets, increased mitotic rate (5 per 10 HPF), necrosis). […] All CPT grades may metastasize throughout the ventricular system or subarachnoid space with implantation in the ependymal or meninges respectively; even histologically benign CPP can behave as aggressive carcinomas with widespread metastasis. […] Cytology: Papillomas and carcinomas may only be differentiated via histopathological examination as they have very similar cytological appearance.
- #31 Choroid plexus carcinoma: A case report and literature reviewhttps://www.oatext.com/Choroid-plexus-carcinoma-A-case-report-and-literature-review.php
Choroid plexus carcinoma is a rare tumor representing less than 1% of all brain tumors. In adult, the incidence is extremely rare making the diagnosis difficult. […] In adult however, due to its extremely rare occurrence, the diagnosis of choroid plexus carcinoma should be made with caution as it more frequently resembles a metastatic papillary tumor such as from kidney and thyroid. […] The clinical presentation, pathology and management are discussed. […] Most of the diagnosis of choroid plexus carcinoma is made in paediatrics age group and rarely reported in adult. If present in adult, it should be interpreted with caution and patient should be investigated thoroughly to exclude metastatic tumors particularly from renal and thyroid. […] In investigating the tumor, both CT scan and MRI is needed, and sometimes angiography will be useful especially if we are suspecting a highly vascularized tumor.
- #32 Choroid Plexus Papilloma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findingshttps://emedicine.medscape.com/article/250795-workup
Although intraventricular lesions are readily identified on imaging studies, tumor biopsy is still warranted. Biopsy may facilitate differentiation of a papilloma from an aggressive carcinoma that may require a different surgical approach. […] Choroid plexus carcinoma, WHO grade III, is characterized by frank signs of malignancy such as nuclear pleomorphism and hyperchromasia, increased mitotic activity (usually more than 5 mitoses per 10 high-power fields or 2.5 mitoses/mm2), increased cellular density, poorly structured sheets of tumor cells that obscure the papillary pattern, and tumor necrosis. […] The histologic differential diagnosis includes choroid plexus hyperplasia, papillary ependymoma, metastatic carcinoma (in older patients), and rare entities such as medulloepithelioma or germ cell tumor, in which a papillary pattern may predominate.
- #33https://link.springer.com/article/10.1007/s42399-025-01831-5
Immunohistochemically, CPC is typically positive for broad-spectrum cytokeratins and variably for vimentin, S100, transthyretin, and glial fibrillary acid protein (GFAP), while it is usually negative for epithelial membrane antigen (EMA). […] Treatment modalities for CPC typically include surgery, RT, and CTx. […] Surgery is often the first-line treatment to alleviate mass effect and CSF obstruction. […] This comprehensive analysis, combining descriptive data with Cox regression, indicates that the extent of surgical resection is the only significant predictor of survival in this study. […] Our findings reinforce the importance of gross total resection (GTR) as the primary prognostic factor for OS in patients with CPC.
- #34 Pathology Outlines – Choroid plexus tumors (papilloma, atypical papilloma, carcinoma)https://www.pathologyoutlines.com/topic/cnstumorchoroidplexuspapillomas.html
Choroid plexus tumors are rare neoplasms derived from the choroid plexus epithelium. They occur within the ventricle system of the brain. […] Diagnostically, transthyretin (TTR), KIR7.1, cytokeratin and Ki67 immunohistochemistry are most helpful. […] Clinical features, imaging (CT or preferably MRI). Biopsy with histologic examination for definitive diagnosis. […] Choroid plexus carcinoma (CPC, WHO grade 3): Frankly malignant. High cellularity, hyperchromatic nuclei and nuclear pleomorphism. Blurring of papillary pattern and solid arrangement. Frequent mitoses (> 5/10 high power fields), necrosis, with or without brain invasion. […] Recent studies suggest 3 distinct molecular entities based on methylation profiling, patient age and tumor location. […] TP53 sequencing and testing for germ line TP53 mutation is advisable in children with choroid plexus carcinoma.
- #35 Choroid plexus carcinoma – Libre Pathologyhttps://librepathology.org/wiki/Choroid_plexus_carcinoma
Mitoses. […] Necrosis. […] +/-Brain invasion. […] DDx: Choroid plexus papilloma. […] Atypical plexus papilloma – has features intermediate between choroid plexus papilloma and choroid plexus carcinoma. […] Atypical teratoid/rhabdoid tumour. […] Features: Cytokeratins +ve. […] EMA usu. -ve. […] Ki-67 high. Useful to diff. from benign counterpart. […] INI1 +ve.
- #36 Cytokeratin 7 and 20 Expression in Choroid Plexus Tumors: Utility in Differentiating These Neoplasms from Metastatic Carcinomas | Modern Pathologyhttps://www.nature.com/articles/3880111
Tumors derived from choroid plexus epithelium are uncommon and may exhibit a wide variety of histologic patterns. They often are difficult to distinguish from metastatic carcinomas. […] Recent reports have demonstrated that evaluation of coordinate expression of cytokeratin (CK) 7 and CK20 aids in distinguishing primary from metastatic lesions in a number of anatomic sites and that tumors that commonly are metastatic to the brain retain their CK7/CK20 immunophenotype in this location. […] Our results indicate that the majority of choroid plexus tumors have a CK7-positive/CK20-negative immunophenotype. This finding may be useful in differentiating these lesions from metastatic carcinomas that have differing CK7/CK20 profiles. […] The majority of choroid plexus tumors in this study (74%), including all atypical papillomas, possessed a CK7-positive/CK20-negative immunophenotype.
- #37 Cytokeratin 7 and 20 Expression in Choroid Plexus Tumors: Utility in Differentiating These Neoplasms from Metastatic Carcinomas | Modern Pathologyhttps://www.nature.com/articles/3880111
Tumors derived from choroid plexus epithelium are uncommon and may exhibit a wide variety of histologic patterns. They often are difficult to distinguish from metastatic carcinomas. […] Recent reports have demonstrated that evaluation of coordinate expression of cytokeratin (CK) 7 and CK20 aids in distinguishing primary from metastatic lesions in a number of anatomic sites and that tumors that commonly are metastatic to the brain retain their CK7/CK20 immunophenotype in this location. […] Our results indicate that the majority of choroid plexus tumors have a CK7-positive/CK20-negative immunophenotype. This finding may be useful in differentiating these lesions from metastatic carcinomas that have differing CK7/CK20 profiles. […] The majority of choroid plexus tumors in this study (74%), including all atypical papillomas, possessed a CK7-positive/CK20-negative immunophenotype.
- #38 Cytokeratin 7 and 20 Expression in Choroid Plexus Tumors: Utility in Differentiating These Neoplasms from Metastatic Carcinomas | Modern Pathologyhttps://www.nature.com/articles/3880111
This finding may be useful in distinguishing these neoplasms from metastatic carcinomas that have differing CK7/CK20 profiles. Our findings also confirm the utility of GFAP, transthyretin, and Ber EP4 in this differential diagnosis. Used in combination, these markers should differentiate choroid plexus tumors from metastatic lesions in most cases.
- #39 Choroid plexus carcinoma – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/choroid-plexus-carcinoma/diagnosis-treatment/drc-20578787
Tests and procedures used to diagnose choroid plexus carcinoma include: […] During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor. […] Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery. […] Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling. […] If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors.
- #40 Choroid Plexus Tumor Treatment | St. Jude Care & Treatmenthttps://www.stjude.org/care-treatment/treatment/childhood-cancer/brain-tumors/choroid-plexus-tumor.html
Molecular testing must occur on your tumor before you can get a correct diagnosis. […] Health care teams use genetic testing to look for genes that can give them more information about the tumor. These genes are called molecular markers. The markers help to plan the best treatment for the tumor type.
- #41 Choroid Plexus Tumor: Diagnosis and Treatment – NCIhttps://www.cancer.gov/rare-brain-spine-tumor/tumors/choroid-plexus-tumors
To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue. […] Primary CNS tumors are graded based on a tumor tissue analysis performed by a neuropathologist. […] Genetic changes, notably mutation of the TP53 gene, have been linked to the formation of grade 3 choroid plexus carcinomas in about 50 percent of cases. […] The first treatment for choroid plexus tumors is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and remove as much tumor as possible without causing more symptoms.
- #42 Choroid Plexus – American Brain Tumor Association | Learn Morehttps://www.abta.org/tumor_types/choroid-plexus/
Choroid plexus carcinomas are generally treated with post-operative chemotherapy. […] TP53 mutations are common in choroid plexus carcinoma and are associated with a worse prognosis.
- #43 LFS Childhood Cancer Awareness: Choroid Plexus Carcinoma | Living LFS: Li-Fraumeni Syndromehttps://livinglfs.org/lfs-childhood-cancer-awareness-choroid-plexus-carcinoma/
Choroid Plexus Brain Tumors are highly associated with Li-Fraumeni Syndrome. […] Choroid plexus tumors are very rare, they make up less than 1% of all brain tumors. Yet, greater than 85% of choroid plexus tumors occur in children under the age of 5. […] In the early 1990’s, clinicians were beginning to see a link between kids with Choroid Plexus Carcinoma and LFS. Yet, it wasn’t until the 2000s that there was enough data to tell. The problem with Choroid Plexus tumors – they are very rare. Li-Fraumeni Syndrome is also very rare. In order for clinicians and researchers to draw conclusions, they needed data. Unfortunately, with rare cancers, that data can be tough to get. […] Choroid Plexus Carcinoma generally occurs in children under the age of 2 and the survival prognosis is poor, especially in tumors that have acquired many mutations. If the child is diagnosed with a TP53 mutation, yet their parents do not have the mutation, they are considered „de novo”- a new mutation. These mutations can happen in the parent’s germ cells or at some point during pregnancy.
- #44 Choroid plexus carcinoma | Beacon Health Systemhttps://www.beaconhealthsystem.org/library/diseases-and-conditions/choroid-plexus-carcinoma?content_id=CON-20308119
Tests and procedures used to diagnose choroid plexus carcinoma include: […] During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor. […] Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery. […] Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling. […] If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors. The management of this cancer is complex. Seek out a medical center that has experience with this cancer and can offer the latest treatment options for your child.
- #45 Pathology Outlines – Choroid plexus tumors (papilloma, atypical papilloma, carcinoma)https://www.pathologyoutlines.com/topic/cnstumorchoroidplexuspapillomas.html
Choroid plexus tumors are rare neoplasms derived from the choroid plexus epithelium. They occur within the ventricle system of the brain. […] Diagnostically, transthyretin (TTR), KIR7.1, cytokeratin and Ki67 immunohistochemistry are most helpful. […] Clinical features, imaging (CT or preferably MRI). Biopsy with histologic examination for definitive diagnosis. […] Choroid plexus carcinoma (CPC, WHO grade 3): Frankly malignant. High cellularity, hyperchromatic nuclei and nuclear pleomorphism. Blurring of papillary pattern and solid arrangement. Frequent mitoses (> 5/10 high power fields), necrosis, with or without brain invasion. […] Recent studies suggest 3 distinct molecular entities based on methylation profiling, patient age and tumor location. […] TP53 sequencing and testing for germ line TP53 mutation is advisable in children with choroid plexus carcinoma.
- #46 :: BTRT :: Brain Tumor Research and Treatmenthttps://btrt.org/DOIx.php?id=10.14791/btrt.2019.7.e23
The first case in this report presented with 4th ventricular CPC and a tectal mass at 40 years of age. […] The second case presented here was diagnosed at age 49, and the location of the tumor was more consistent with extraventricular than lateral ventricle. […] Differential diagnosis based on imaging characteristics are difficult in extraventricular cases of CPC as well as CPP, choroid plexus cyst, ependymoma, primitive neuroectodermal tumor, astrocytoma, germinoma, teratoma, meningioma, metastasis to the choroid plexus, and xanthogranuloma. […] CPC is associated with poor prognosis, and the 5-year survival rate for patients with CPCs is approximately 40%. […] Numerous authors have emphasized the importance of gross total removal of CPC as part of the therapeutic strategy. […] The extent of tumor removal is associated with significantly superior overall survival (OS); whereas, the effect of adjuvant chemotherapy or radiotherapy on OS remains controversial.
- #47 Choroid Plexus Papilloma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findingshttps://emedicine.medscape.com/article/250795-workup
Although intraventricular lesions are readily identified on imaging studies, tumor biopsy is still warranted. Biopsy may facilitate differentiation of a papilloma from an aggressive carcinoma that may require a different surgical approach. […] Choroid plexus carcinoma, WHO grade III, is characterized by frank signs of malignancy such as nuclear pleomorphism and hyperchromasia, increased mitotic activity (usually more than 5 mitoses per 10 high-power fields or 2.5 mitoses/mm2), increased cellular density, poorly structured sheets of tumor cells that obscure the papillary pattern, and tumor necrosis. […] The histologic differential diagnosis includes choroid plexus hyperplasia, papillary ependymoma, metastatic carcinoma (in older patients), and rare entities such as medulloepithelioma or germ cell tumor, in which a papillary pattern may predominate.
- #48 Choroid plexus carcinoma in an adult – Journal of Neurosciences in Rural Practicehttps://ruralneuropractice.com/choroid-plexus-carcinoma-in-an-adult/
Choroid plexus carcinoma is a very rare tumor in adults. […] A 24-year-old male presented with a right temporal intraventricular tumor with a cystic component also extending up to the cortex. […] The patient went through a complete search for a possible primary keeping in mind the differential diagnosis of metastatic carcinoma that is more common in adults but there was no evidence of any other tumor. Finally a diagnosis of choroid plexus carcinoma was rendered. […] Choroid plexus carcinoma is an aggressive tumor that must be distinguished from choroid plexus papilloma and distinction between these entities can sometimes be difficult. The main distinguishing factors are presence of necrosis, mitotic activity, and growth pattern. […] Choroid plexus carcinoma is a highly aggressive malignant tumor WHO grade-III that usually presents with CSF obstruction commonly in the lateral ventricles (50%) followed by IV ventricle (40%), third ventricle (5%), and multiple ventricles (5%).
- #49 Choroid plexus carcinoma in an adult – Journal of Neurosciences in Rural Practicehttps://ruralneuropractice.com/choroid-plexus-carcinoma-in-an-adult/
The differential diagnosis includes choroid plexus papilloma, villous hypertrophy of choroid plexus, papillary variant of ependymoma or meningioma and metastatic papillary neoplasms. […] Based on the clinical and histopathological findings we diagnosed our case as a classical choroid plexus carcinoma except for the fact that this patient was an adult which is extremely rare. […] The tumor must be differentiated from metastatic carcinoma which is especially important in case of choroid plexus carcinoma in adults as it is an extremely rare tumor in adults. […] On immunohistochemical staining, it was found that the tumor was positive for S-100, cytokeratin and GFAP. […] This combination also favors the diagnosis of choroid plexus carcinoma as also suggested in reviews. […] An adult patient presented with a right temporal intraventricular tumor with a cystic component also extending up to the cortex. Histological examination revealed features of a choroid plexus carcinoma. […] Finally a diagnosis of choroid plexus carcinoma was rendered although it a rare entity among adults.
- #50 Choroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling | American Journal of Neuroradiologyhttps://www.ajnr.org/content/36/9/1786
Pediatric choroid plexus papillomas and carcinomas are highly vascularized neoplasms, which are difficult to distinguish with conventional imaging. […] We aimed to analyze the diagnostic accuracy of PWI, by using both pseudocontinuous arterial spin-labeling and DSC-PWI. […] Arterial spin-labeling is a promising technique to differentiate choroid plexus carcinomas and papillomas. […] Conventional imaging alone does not allow differentiating these 2 entities, and a pathologic examination is always needed. […] A preoperative diagnosis could be very useful, even if surgery is performed for all cases. […] Therefore, advanced MR imaging techniques such as perfusion-weighted imaging could provide additional information on tumor vascularization that may help in the differential diagnosis.
- #51 Choroid plexus tumors: problems in diagnosis and management in: Journal of Neurosurgery Volume 66 Issue 6 (1987) Journalshttps://thejns.org/view/journals/j-neurosurg/66/6/article-p800.xml
Choroid plexus tumors are uncommon neoplasms of the central nervous system. […] Many of these tumors are associated with severe hydrocephalus at the time of diagnosis, and the perioperative management of this hydrocephalus remains a matter of some debate. […] The similarity between papillary ependymomas and choroid plexus papillomas has sometimes caused difficulty in pathological diagnosis. Choroid plexus carcinomas, of which there were two in this series, also present a diagnostic challenge. […] Differential diagnosis affects the further treatment and prognosis for the patient.
- #52 Choroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling | American Journal of Neuroradiologyhttps://www.ajnr.org/content/36/9/1786
Pediatric choroid plexus papillomas and carcinomas are highly vascularized neoplasms, which are difficult to distinguish with conventional imaging. […] We aimed to analyze the diagnostic accuracy of PWI, by using both pseudocontinuous arterial spin-labeling and DSC-PWI. […] Arterial spin-labeling is a promising technique to differentiate choroid plexus carcinomas and papillomas. […] Conventional imaging alone does not allow differentiating these 2 entities, and a pathologic examination is always needed. […] A preoperative diagnosis could be very useful, even if surgery is performed for all cases. […] Therefore, advanced MR imaging techniques such as perfusion-weighted imaging could provide additional information on tumor vascularization that may help in the differential diagnosis.
- #53 Preoperative imaging of Pediatric choroid plexus tumors : how radiologists assess the diagnosis and predict malignancy ?https://epos.myesr.org/poster/esr/ecr2020/C-08609
To highlight the accuracy of cross sectional imaging (CT and MRI) in assessing the diagnosis of pediatric choroid plexus tumors, in predicting their malignancy, and therefore guiding the optimal therapy. […] Imaging can detect tumors suspected to be high-grade when they are heterogenous, they invade the neighboring parenchyma and are associated with peritumoral edema and/or mass effect, which may, however, also be present in a benign papilloma.
- #54 Choroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling | American Journal of Neuroradiologyhttps://www.ajnr.org/content/36/9/1786
Pediatric choroid plexus papillomas and carcinomas are highly vascularized neoplasms, which are difficult to distinguish with conventional imaging. […] We aimed to analyze the diagnostic accuracy of PWI, by using both pseudocontinuous arterial spin-labeling and DSC-PWI. […] Arterial spin-labeling is a promising technique to differentiate choroid plexus carcinomas and papillomas. […] Conventional imaging alone does not allow differentiating these 2 entities, and a pathologic examination is always needed. […] A preoperative diagnosis could be very useful, even if surgery is performed for all cases. […] Therefore, advanced MR imaging techniques such as perfusion-weighted imaging could provide additional information on tumor vascularization that may help in the differential diagnosis.
- #55 Choroid Plexus Neoplasms: Toward a Distinction between Carcinoma and Papilloma Using Arterial Spin-Labeling | American Journal of Neuroradiologyhttps://www.ajnr.org/content/36/9/1786
The objective of our study was to analyze the perfusion pattern of pediatric choroid plexus papilloma and carcinoma with perfusion weighted-imaging, by using ASL or DSC-PWI. […] ASL perfusion MR imaging can discriminate preoperatively between choroid plexus carcinomas and papillomas; this finding helps the surgeon to adapt his or her operative strategy (possible preoperative embolization).
- #56 Choroid plexus tumors, a systematic reviewhttps://epos.myesr.org/poster/esr/ecr2024/C-20424/findings%20and%20procedure%20details
Choroid plexus carcinoma is a malignant aggressive lesion, WHO grade 3, with high vascularity and various dimensions, usually large. TP53 gene mutations are often associated with choroid plexus carcinoma. […] Choroid plexus carcinoma has hypercellular character, with multiple mitoses, cerebral parenchyma invasion and cerebrospinal fluid metastatic seeds. […] Due to increased intracranial pressure, there are several symptoms that may show. The patients usually have symptoms like headache, nausea and vomiting or neurologic defects. […] This neoplasm can show tumoral infiltration in the surrounding structures, peritumoral edema and CSF spread and the most reliable imaging techniques are CT and MRI. Choroid plexus carcinoma is a complex tumor, with some typical characteristics like: lobulated contour, well-defined, large dimensions or mixed content. Tumor density on CT can be intermediate or high, with irregular border. Heterogeneous appearance of the lesion is frequently encountered, with cystic areas, necrotic, calcified or hemorrhagic zones. Choroid plexus carcinoma is strongly iodophil on contrast enhanced CT, with heterogenous pattern due to cysts / necrosis / calcifications. Other associated signs, well seen on CT are hydrocephalus or perilesional edema. On T1WI the signal is isointense or hypointense, mostly heterogeneous. T2WI sequence is optimal to perceive necrosis/cysts (appear hyperintense), calcifications or blood (depending on the age of hemorrhage) or sometimes, flow voids (loss of signal). T2WI and FLAIR sequences highlight periventricular edema, which means invasion in the white matter. Transependymal edema is the consequence of hydrocephalus. ADC values are very important, the lower the value, the worse the prognosis. The tumor enhances inhomogeneous, but intense. […] Choroid plexus papilloma is sometimes very similar with choroid plexus carcinoma on MRI, and only the biopsy can make the difference between them.
- #57 Choroid plexus carcinoma: A case report and literature reviewhttps://www.oatext.com/Choroid-plexus-carcinoma-A-case-report-and-literature-review.php
Choroid plexus carcinoma is a rare tumor representing less than 1% of all brain tumors. In adult, the incidence is extremely rare making the diagnosis difficult. […] In adult however, due to its extremely rare occurrence, the diagnosis of choroid plexus carcinoma should be made with caution as it more frequently resembles a metastatic papillary tumor such as from kidney and thyroid. […] The clinical presentation, pathology and management are discussed. […] Most of the diagnosis of choroid plexus carcinoma is made in paediatrics age group and rarely reported in adult. If present in adult, it should be interpreted with caution and patient should be investigated thoroughly to exclude metastatic tumors particularly from renal and thyroid. […] In investigating the tumor, both CT scan and MRI is needed, and sometimes angiography will be useful especially if we are suspecting a highly vascularized tumor.
- #58 Diagnosing and Treating a Choroid Plexus Tumor | Neurological Surgeryhttps://neurosurgery.weillcornell.org/condition/choroid-plexus-tumors/diagnosing-and-treating-choroid-plexus-tumor
When symptoms of a brain tumor are present, a doctor will often order a magnetic resonance imaging (MRI) study. Choroid plexus tumors are usually easy to identify on an MRI scan, with typically irregular borders and a cauliflower-like appearance. An MRI scan may reveal multiple tumors in different regions of the brain. Swelling and hydrocephalus are commonly seen along with choroid plexus tumors. […] A biopsy is typically performed on the removed tumor to confirm the grade and type of tumor. […] Due to the complexity and rarity of choroid plexus tumors, patients should be evaluated and treated at major medical centers with interdisciplinary teams who have experience with this tumor. The teams at smaller hospitals may never have seen a choroid plexus tumor before, as there are only about 125 new diagnoses each year.
- #59 Choroid plexus tumor: Symptoms, treatment, and outlookhttps://www.medicalnewstoday.com/articles/choroid-plexus-tumor
People may also be able to take part in clinical trials for other types of treatment, such as targeted therapies or immunotherapy. […] The prognosis for choroid plexus tumors varies based on the tumors type and grade. […] It is important to note these statistics do not predict survival in an individuals case. Additionally, this data comes from a span of nearly 20 years. As cancer treatments are always improving, these figures may not accurately represent survival rates today. […] Early intervention and a comprehensive, multidisciplinary approach are crucial for managing symptoms and improving outcomes.
- #60 Choroid Plexus Tumors | UCSF Brain Tumor Centerhttps://braintumorcenter.ucsf.edu/condition/choroid-plexus-tumors
Choroid plexus carcinoma is a malignant (cancerous) lesion that is able to spread to other parts of the brain via the cerebrospinal fluid. […] Children with choroid plexus carcinoma may be screened to help guide future treatment. Parents of children with choroid plexus carcinoma may also benefit from genetic counseling. […] Patients with choroid plexus carcinomas also have improved outcomes when the tumor is completely removed, but are more successfully treated with a combination of therapies. […] Aggressive chemotherapy, which may be provided as high-dose chemotherapy with stem cell transplant, is often recommended for patients with choroid plexus carcinomas. […] For patients with choroid plexus carcinoma, long-term outcomes depend on a variety of factors, including the size and location of the tumor, whether it spread (or metastasized) to other brain regions, and whether it was completely removed during surgery. […] However, patients with choroid plexus carcinoma often require more aggressive treatments, such as chemotherapy and/or radiation after surgery.
- #61 LFS Childhood Cancer Awareness: Choroid Plexus Carcinoma | Living LFS: Li-Fraumeni Syndromehttps://livinglfs.org/lfs-childhood-cancer-awareness-choroid-plexus-carcinoma/
Kids and people with LFS are very sensitive to ionizing radiation, especially to the brain. Risks and benefits should be discussed with a professional team knowledgeable in Li-Fraumeni Syndrome. Recent studies show that radiation therapy for Choroid Plexus Tumors in LFS could negatively affect survival. Treatment options for Choroid Plexus Tumors include surgery and chemotherapy. […] Knowing LFS status can affect screening and treatment decisions. Many don’t know at the time of Choroid Plexus Carcinoma diagnosis that there is a TP53 mutation. Often genetic counseling and testing is recommended during treatment. Testing for hereditary conditions like LFS is a personal and even controversial decision. […] There is HOPE. Choroid Plexus Carcinoma is nasty and aggressive. Like LFS, it is not a death sentence. There is no „one size fits all” for screening, treatment, or diagnosis. Being aware of the symptoms, trusting your gut and knowing LFS status can make a difference in survival.
- #62 Choroid plexus carcinoma | Beacon Health Systemhttps://www.beaconhealthsystem.org/library/diseases-and-conditions/choroid-plexus-carcinoma?content_id=CON-20308119
Tests and procedures used to diagnose choroid plexus carcinoma include: […] During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor. […] Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery. […] Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling. […] If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors. The management of this cancer is complex. Seek out a medical center that has experience with this cancer and can offer the latest treatment options for your child.
- #63 Diagnosing and Treating a Choroid Plexus Tumor | Neurological Surgeryhttps://neurosurgery.weillcornell.org/condition/choroid-plexus-tumors/diagnosing-and-treating-choroid-plexus-tumor
When symptoms of a brain tumor are present, a doctor will often order a magnetic resonance imaging (MRI) study. Choroid plexus tumors are usually easy to identify on an MRI scan, with typically irregular borders and a cauliflower-like appearance. An MRI scan may reveal multiple tumors in different regions of the brain. Swelling and hydrocephalus are commonly seen along with choroid plexus tumors. […] A biopsy is typically performed on the removed tumor to confirm the grade and type of tumor. […] Due to the complexity and rarity of choroid plexus tumors, patients should be evaluated and treated at major medical centers with interdisciplinary teams who have experience with this tumor. The teams at smaller hospitals may never have seen a choroid plexus tumor before, as there are only about 125 new diagnoses each year.
- #64 First Patient Treated in Choroid Plexus Clinical Trial | Neurological Surgeryhttps://neurosurgery.weillcornell.org/in-the-news/first-patient-treated-choroid-plexus-clinical-trial
A new clinical trial is now underway at Weill Cornell Medicine, testing intra-arterial chemotherapy for choroid plexus carcinoma. […] It is hoped that this same combination will be a safe and feasible treatment for patients with newly diagnosed, residual, or recurrent atypical choroid plexus papilloma and choroid plexus carcinoma. […] The primary outcome goal for this Phase I study is establishing the safety of the procedure, and patients will be monitored for a year for any adverse effects. […] Intra-arterial chemotherapy (IAC) is an advanced procedure administered by interventional neurosurgeons or neuroradiologists, who specialize in image-guided treatments for brain and spine conditions. […] Choroid plexus carcinoma has excellent survival rates in patients who undergo complete tumor resection, but it is significantly less in those who only have partial resections. […] With this new trial, he now offers new hope to children and families facing the diagnosis of choroid plexus tumors.
- #65 Choroid plexus tumor: Symptoms, treatment, and outlookhttps://www.medicalnewstoday.com/articles/choroid-plexus-tumor
People may also be able to take part in clinical trials for other types of treatment, such as targeted therapies or immunotherapy. […] The prognosis for choroid plexus tumors varies based on the tumors type and grade. […] It is important to note these statistics do not predict survival in an individuals case. Additionally, this data comes from a span of nearly 20 years. As cancer treatments are always improving, these figures may not accurately represent survival rates today. […] Early intervention and a comprehensive, multidisciplinary approach are crucial for managing symptoms and improving outcomes.
- #66 Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach | Health & Environmental Research Online (HERO) | US EPAhttps://hero.epa.gov/hero/index.cfm/reference/details/reference_id/8584770
Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach […] To identify specific markers for the diagnosis of choroid plexus tumors, gene expression profiles of choroid plexus epithelial cells (n = 8) and ependymal cells (n = 6) microdissected from human autopsy brains as well as choroid plexus papilloma tissue were investigated using DNA microarrays. […] Protein expression of genes overexpressed in choroid plexus was evaluated in normal choroid plexus, choroid plexus papilloma, choroid plexus carcinoma, other primary brain tumors, and cerebral metastases. […] Our data suggest that antibodies directed against Kir7.1 and stanniocalcin-1 might serve as sensitive and specific diagnostic markers for choroid plexus tumors.
- #67 Intra-Arterial (IA) Chemotherapy for Newly Diagnosed, Residual, or Recurrent Atypical Choroid Plexus Papilloma (ACPP) and Choroid Plexus Carcinoma (CPC) Prior to Second-Look Surgery | Joint Clinical Trials Officehttps://jcto.weill.cornell.edu/open_clinical_trials/intra-arterial-ia-chemotherapy-for-newly-diagnosed-residual-or-recurrent-atypical-choroid-plexus-papilloma-acpp-and-choroid-plexus-carcinoma-cpc-prior-to-second-look-surgery
Atypical choroid plexus papilloma (ACPP) and choroid plexus carcinoma (CPC) are both primary brain tumors that begin near the brain tissue. Choroid plexus carcinoma is fast-growing high-grade tumor, whereas atypical choroid plexus papilloma is an intermediate-grade tumor. […] The purpose of this study is to better understand if a single dose of inter-arterial (IA) chemotherapy is safe in patients with atypical choroid plexus papilloma or choroid plexus carcinoma. […] Participants must have a confirmed diagnosis of ACPP or CPP that is either newly diagnosed, residual, or recurrent.
- #68 Choroid plexus tumours | British Journal of Cancerhttps://www.nature.com/articles/6600609
Treatment of choroid plexus tumours should start with radical surgical resection. This should be followed by adjuvant treatment in case of choroid plexus-carcinoma, and a wait and see approach in choroid plexus-papilloma. […] The definitive diagnosis of a choroid plexus-tumour can only be made histologically. […] Surgery is therefore required for the diagnosis. Tumour resection is also a powerful therapeutical step. […] In CPC patients adjuvant treatment should follow surgery. […] We confirmed significant improved survival for patients with CPC who received irradiation in both subgroups (a) with residual tumour and (b) after gross total resection. […] Recurrence of CPC has to be judged incurable with conventional treatment, and is therefore an indication for experimental treatment. […] In contrast to CPC, there is no published evidence yet that adjuvant treatment has any impact on survival of CPP patients.
- #69 September Share – Madison – Choroid Plexus Carcinoma – The Gold Hope Projecthttps://goldhopeproject.com/september-share-madison-choroid-plexus-carcinoma/
Choroid Plexus Carcinoma a rare form of brain cancer. We are currently on a clinical trial and its her last hope for a cure. […] Maddies diagnosis is brain cancer with hydrocephalus and also cancer in her spinal fluid with a spot on her spine. Its called choroid plexus. […] We went and got a second opinion from a dr in Boston that specialized and studied choroid plexus carcinoma. […] That tumor was sent out to have a tumor marker test done to see what would be the best drugs to help her. […] She started cycle 25 of her treatment on August 5, 2015 she has been on this treatment for 24 months of this clinical trial not knowing if she will beat this. […] She gets spinal taps, echos, EKG and MRIs of the brain and spine every 3 months. […] She has been through 3 brain surgeries, 2 tumor removals, shunt placement. Broviac and port placement, too many MRIs, echo, ekgs sedations and spinal taps to count. Chemo since she was 4 months old and she is still getting treatment now and she is almost 5.
- #70 Choroid plexus tumours | British Journal of Cancerhttps://www.nature.com/articles/6600609
Choroid plexus tumours are rare epithelial brain tumours and limited information is available regarding their biology and the best treatment. […] A thorough review of the medical literature (19661998) revealed 566 well-documented choroid plexus tumours. […] Histology was the most important prognostic factor, as one, five, and 10-year projected survival rates were 90, 81, and 77% in choroid plexus-papilloma (n=353) compared to only 71, 41, and 35% in choroid plexus-carcinoma respectively (P0.0005). […] Surgery was prognostically relevant for both choroid plexus-papilloma (P=0.0005) and choroid plexus-carcinoma (P=0.0001). […] Radiotherapy was associated with significantly better survival in choroid plexus-carcinomas. […] Relapse after primary treatment was a poor prognostic factor in choroid plexus-carcinoma patients but not in choroid plexus-papilloma patients.
- #71 Choroid Plexus Tumors in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/choroid-plexus-tumor.html
Choroid plexus carcinoma (CPC) is a cancerous form of choroid plexus tumor. It grows faster. It is more likely to spread through the cerebrospinal fluid to other tissues. About 10-20% of choroid plexus tumors are CPC. […] Choroid plexus carcinoma (CPC) is more aggressive. But the chance of cure is about 50-70%. […] Choroid plexus carcinoma is a more aggressive cancer. Most of these patients will receive many types of treatments. […] Choroid plexus carcinoma generally is cancerous, typically Grade III, more aggressive, and can grow rapidly. […] Children with choroid plexus carcinoma usually need chemotherapy and radiation along with surgery. […] Radiation therapy is sometimes used in addition to other treatments. The type of radiation therapy used depends on the location of the tumor and if it has spread. The child’s age is an important consideration in the use of radiation, too.
- #72https://link.springer.com/article/10.1007/s00701-019-03832-5
Choroid plexus tumors are rare entities. Resection is the mainstay of treatment in grade I and grade II tumors and adjuvant treatment is usually reserved for the less frequent choroid plexus carcinoma (CPC). […] Adjuvant therapy was performed in 19.4% of patients, mainly diagnosed with CPC. […] We could confirm that GTR is crucial for treatment of choroid plexus tumors. […] The infiltrative growth of aCPP resembles the appearance of CPC, resulting in poor OS and PFS as well in aCPP cases. […] GTR is the most important prognostic factor in CPTs. […] In our cohort, GTR was strongly associated with higher survival rates and a decreased risk of tumor recurrence. […] The significantly higher 5-year OS of CPC patients (100%) than their 5-year PFS (33.3%) may be explained by the efficacy of the adjuvant therapy as reported elsewhere.
- #73 Choroid Plexus Brain Tumor | Boston Children’s Hospitalhttps://www.childrenshospital.org/conditions/choroid-plexus-brain-tumor
The five-year survival rate for children with CPP is 80 to 100 percent following complete surgical removal of the tumor and about 70 percent with partial removal. CPC requires additional postoperative treatment, with a survival rate of about 60 to 65 percent. […] Children with choroid plexus tumors are treated through the Brain Tumor Center at Dana-Farber/Boston Childrenâs Cancer and Blood Disorders Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.
- #74 Choroid Plexus Brain Tumor | Boston Children’s Hospitalhttps://www.childrenshospital.org/conditions/choroid-plexus-brain-tumor
The five-year survival rate for children with CPP is 80 to 100 percent following complete surgical removal of the tumor and about 70 percent with partial removal. CPC requires additional postoperative treatment, with a survival rate of about 60 to 65 percent. […] Children with choroid plexus tumors are treated through the Brain Tumor Center at Dana-Farber/Boston Childrenâs Cancer and Blood Disorders Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.
- #75 Choroid plexus tumor – Wikipediahttps://en.wikipedia.org/wiki/Choroid_plexus_tumor
Choroid plexus carcinoma (WHO grade 3) is one of the classifications of choroid plexus tumors. […] Chemotherapy confers a definite survival benefit in choroid plexus carcinoma and maybe beneficial in selected cases of atypical choroid plexus papillomas. […] Following total or partial resection, the 5-year survival rates for choroid plexus carcinoma patients are, respectively, 58 percent and 20 percent.
- #76 September Share – Madison – Choroid Plexus Carcinoma – The Gold Hope Projecthttps://goldhopeproject.com/september-share-madison-choroid-plexus-carcinoma/
Choroid Plexus Carcinoma a rare form of brain cancer. We are currently on a clinical trial and its her last hope for a cure. […] Maddies diagnosis is brain cancer with hydrocephalus and also cancer in her spinal fluid with a spot on her spine. Its called choroid plexus. […] We went and got a second opinion from a dr in Boston that specialized and studied choroid plexus carcinoma. […] That tumor was sent out to have a tumor marker test done to see what would be the best drugs to help her. […] She started cycle 25 of her treatment on August 5, 2015 she has been on this treatment for 24 months of this clinical trial not knowing if she will beat this. […] She gets spinal taps, echos, EKG and MRIs of the brain and spine every 3 months. […] She has been through 3 brain surgeries, 2 tumor removals, shunt placement. Broviac and port placement, too many MRIs, echo, ekgs sedations and spinal taps to count. Chemo since she was 4 months old and she is still getting treatment now and she is almost 5.
- #77 Choroid Plexus Carcinoma Diagnosis And Treatment – Klarity Health Libraryhttps://my.klarity.health/choroid-plexus-carcinoma-diagnosis-and-treatment/
It should be noted however that Li Fraumeni is quite rare as well. Brain imaging […] An MRI is incredibly helpful not only as a firm and reliable method of diagnosing tumours such as CPC, but it is also useful for future surgeries, as it allows surgeons to adapt their method to the size and location of the tumour. […] Therefore, MRIs are useful in initial diagnosis and pre-surgical planning, but also incredibly useful for checking patients after treatment, as choroid plexus tumours can come back. […] With surgery, you also obtain tissue for analysis to be able to determine the specific tissue type and grade of the CPC, which could not be done through MRIs. […] Although treatment of grade 1 and grade 2 choroid plexus tumours has shown to be successful in fully removing the benign growth, it doesn’t have the same success with CPC because of its tendency to spread to other parts of the CNS.
- #78https://link.springer.com/article/10.1007/s42399-025-01831-5
According to the latest WHO classification (5th edition), choroid plexus carcinoma (CPC) is a rare, grade 3 malignancy originating from the choroid plexus, predominantly affecting pediatric patients. […] In adults, CPC is uncommon, and the limited data available have hindered the development of standardized treatment protocols. […] Definitive diagnosis relies on histopathology. Treatment typically involves surgical resection, while adjuvant therapies offer limited benefit to overall survival (OS). […] There is currently no consensus on a standard treatment approach, and outcomes remain unsatisfactory. […] The initial diagnosis is based on computed tomography (CT), which reveals hypodense, heterogeneous lesions, and, in some cases, calcifications. […] The definitive diagnosis depends on histopathological findings and can be primary, mixed, or secondary to CPP.
- #79 Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and  review of the literature | Journal of Medical Case Reports | Full Texthttps://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-02887-2
It is well known that maximal surgical resection in CPC majorly influences the overall survival and progression-free survival. […] A few studies have reported that radiation therapy leads to better clinical outcome in patients with CPC. […] However, the radiation field of CPC is a topic of debate. […] Even in our case, radiation therapy was found to be effective for CPC, but it is contemplated that further consideration of the optimal radiation dose and treatment field will be necessary. […] Although the best chemotherapy regimen for CPC is still unknown, a report revealed long-term outcomes while applying temozolomide (TMZ) in CPC patient with MGMT methylation, while another report showed no effect of TMZ in the absence of MGMT methylation. […] As it is difficult to perform randomized controlled trials on rare carcinomas such as CPC, diverse experiences of CPC in different institutions should be reported and shared. […] In particular, additional research on optimal radiation field and dose and suitable chemotherapy regimen is needed.
- #80 Choroid plexus carcinoma: A case report and literature reviewhttps://www.oatext.com/Choroid-plexus-carcinoma-A-case-report-and-literature-review.php
Choroid plexus carcinoma is a rare tumor representing less than 1% of all brain tumors. In adult, the incidence is extremely rare making the diagnosis difficult. […] In adult however, due to its extremely rare occurrence, the diagnosis of choroid plexus carcinoma should be made with caution as it more frequently resembles a metastatic papillary tumor such as from kidney and thyroid. […] The clinical presentation, pathology and management are discussed. […] Most of the diagnosis of choroid plexus carcinoma is made in paediatrics age group and rarely reported in adult. If present in adult, it should be interpreted with caution and patient should be investigated thoroughly to exclude metastatic tumors particularly from renal and thyroid. […] In investigating the tumor, both CT scan and MRI is needed, and sometimes angiography will be useful especially if we are suspecting a highly vascularized tumor.
- #81 :: BTRT :: Brain Tumor Research and Treatmenthttps://btrt.org/DOIx.php?id=10.14791/btrt.2019.7.e23
Current CPC treatment is based on data from pediatric patients who are heavily treated with chemotherapy, and the role of chemotherapy has not been established in adult CPCs. […] In the cases presented here, CPC patients were treated by surgical resection and adjuvant chemotherapy and/or radiotherapy. […] It is evident that further studies are necessary to establish standardized radiation and chemotherapy strategies in adult CPC patients.
- #82 Prognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysishttps://www.mdpi.com/2072-6694/16/3/610
Given the rarity of choroid plexus tumors (CPTs), there is a need for large cohort analyses to provide a better understanding of prognostication and optimal diagnosis. […] This study provides a better understanding of prognostication and optimal treatment, especially the role that neoadjuvant therapies play. […] Although gross total resection for choroid plexus papillomas (CPPs) is associated with long-term survival, there is a scarcity of prospective data concerning the role and sequence of neoadjuvant therapy in treating aCPP and CPC. […] The nomogram for CPC considers types of treatments received. It demonstrates acceptable accuracy in estimating survival probability at 5-year and 10-year intervals, with a C-index of 0.608 (95% CI of 0.446 to 0.77). […] This is the largest study on CPT to date and highlights the optimal treatment strategies for these rare tumors. […] Furthermore, OS is equivalent for CPC with GTR and STR plus adjuvant therapy.
- #83 Choroid plexus tumours on MRI: similarities and distinctions in different grades | Cancer Imaging | Full Texthttps://cancerimagingjournal.biomedcentral.com/articles/10.1186/s40644-019-0200-1
Larger tumour, irregular or fuzzy internal morphology, presentation of necrosis and wide-ranging peritumoural oedema might increase the likelihood of malignancy. […] Although surgical resection has remained the standard of care for each grade of CPTs, pretreatment imaging diagnosis is essential for surgical planning and therapeutic decisions. […] Our study indicated that tumour longest diameter, papillary appearances, necrosis and peritumoural oedema diameter were relevant to the pathological grade based on the MRI findings from 35 CPTs. […] The combination of tumour longest diameter and oedema diameter was helpful in predicting the grade of CPTs by MRI. […] These findings shall help us to improve the pretreatment diagnosis and provide more guidance for clinical decisions.
- #84 Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and  review of the literature | Journal of Medical Case Reports | Full Texthttps://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-02887-2
It is well known that maximal surgical resection in CPC majorly influences the overall survival and progression-free survival. […] A few studies have reported that radiation therapy leads to better clinical outcome in patients with CPC. […] However, the radiation field of CPC is a topic of debate. […] Even in our case, radiation therapy was found to be effective for CPC, but it is contemplated that further consideration of the optimal radiation dose and treatment field will be necessary. […] Although the best chemotherapy regimen for CPC is still unknown, a report revealed long-term outcomes while applying temozolomide (TMZ) in CPC patient with MGMT methylation, while another report showed no effect of TMZ in the absence of MGMT methylation. […] As it is difficult to perform randomized controlled trials on rare carcinomas such as CPC, diverse experiences of CPC in different institutions should be reported and shared. […] In particular, additional research on optimal radiation field and dose and suitable chemotherapy regimen is needed.
- #85 Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and  review of the literature | Journal of Medical Case Reports | Full Texthttps://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-02887-2
It is well known that maximal surgical resection in CPC majorly influences the overall survival and progression-free survival. […] A few studies have reported that radiation therapy leads to better clinical outcome in patients with CPC. […] However, the radiation field of CPC is a topic of debate. […] Even in our case, radiation therapy was found to be effective for CPC, but it is contemplated that further consideration of the optimal radiation dose and treatment field will be necessary. […] Although the best chemotherapy regimen for CPC is still unknown, a report revealed long-term outcomes while applying temozolomide (TMZ) in CPC patient with MGMT methylation, while another report showed no effect of TMZ in the absence of MGMT methylation. […] As it is difficult to perform randomized controlled trials on rare carcinomas such as CPC, diverse experiences of CPC in different institutions should be reported and shared. […] In particular, additional research on optimal radiation field and dose and suitable chemotherapy regimen is needed.
- #86 KoreaMed Synapsehttps://synapse.koreamed.org/articles/1121901
CPC is associated with poor prognosis, and the 5-year survival rate for patients with CPCs is approximately 40%. […] Numerous authors have emphasized the importance of gross total removal of CPC as part of the therapeutic strategy. […] The extent of tumor removal is associated with significantly superior overall survival (OS); whereas, the effect of adjuvant chemotherapy or radiotherapy on OS remains controversial. […] Current CPC treatment is based on data from pediatric patients who are heavily treated with chemotherapy, and the role of chemotherapy has not been established in adult CPCs. […] In case 1, the patient was treated with craniospinal irradiation after craniotomy for spinal metastasis. […] The treatment response was poor, and the patient expired within a year. […] On the other hand, in case 2, the operation was performed with gross total resection of the lesion, and postoperative spinal images and CSF studies showed no evidence of CSF metastasis. […] It is evident that further studies are necessary to establish standardized radiation and chemotherapy strategies in adult CPC patients.