Materiały źródłowe

• #1 Pineoblastoma: Causes, Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24950-pineoblastoma

  Pineoblastoma is a cancerous (malignant) brain tumor in your pineal gland. This type of cancer grows quickly and can spread to tissue and fluid around your brain. […] An overgrowth of pinealocyte cells, which are the cells within your brains pineal gland, causes pineoblastoma. Research suggests that a change to your genetic code (mutation) may influence these cells to function abnormally. A child can inherit genetic changes from their biological parents when the egg and sperm meet during conception (germline mutation). In contrast, these changes can happen randomly after conception (sporadically), without any history of the genetic change in a childs biological family. […] You may be at a higher risk of developing pineoblastoma if you have retinoblastoma. Retinoblastoma is a type of cancer that affects the retina of your eyes. […] In addition, if you have a genetic change to the RB1 and DICER1 genes, youre more likely to develop pineoblastoma. A child can either inherit this genetic change or the change happens randomly in their DNA after conception.

• #2 Pineoblastoma – Symptoms, Diagnosis, and TreatmentSecond Opinion IconGroup 9Second Opinion IconGroup 9Group 49

  https://www.barrowneuro.org/condition/pineoblastoma/

  Pineoblastomas are rare, aggressive brain tumors originating in the pineal gland, a small almond-shaped structure in the brain’s center. […] The exact cause of pineoblastomas is poorly understood, but doctors think they stem from a combination of genetic and cellular factors. […] Pineoblastomas most often occur in children and are thought to relate to the developing brain and the presence of cells more susceptible to mutations or abnormal growth. […] A mutation in the RB1 gene is a widely known risk factor for pineoblastomas in people with retinoblastoma, an eye cancer. […] A less common risk factor is a mutated DICER1 gene—a tumor protection gene that, when altered, can cause cells to grow and divide uncontrollably, leading to tumors. […] Pineoblastomas have no known environmental triggers, like radiation or chemical exposure, that contribute to their growth. Ongoing research continues to explore the exact cellular and molecular mechanisms that lead to the condition. […] Currently, there is no known way to prevent pineoblastomas—they’re extremely rare tumors, and their exact causes are not yet fully understood. No specific environmental factors are linked to pineoblastomas, and lifestyle changes or environmental interventions do not prevent them.

• #3 Clinical Trials for Pineal-Region Tumors | Ivy Brain Tumor Center

  https://www.ivybraintumorcenter.org/brain-tumor-care/brain-tumor-types/pineal-region-tumors/

  Like most brain tumors, the exact cause of pineal-region tumors is not known. […] Pineoblastomas are WHO grade IV tumors that are the most aggressive variety.

• #4 Pineoblastoma | About the Disease | GARD

  https://rarediseases.info.nih.gov/diseases/9369/pineoblastoma

  Pineoblastoma is a type of cancerous (malignant) tumor that grows in a part of the brain known as the pineal gland. […] The cause of Pineoblastoma is unknown, but specific inherited genetic variants in two genes, RB1 and DICER1 can increase the risk for a Pineoblastoma. […] GARD does not currently have information about the cause of this disease.

• #5 SSA – POMS: DI 23022.273 – Pineoblastoma – Childhood – 08/09/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022273

  Pineoblastoma is an aggressive cancerous (malignant) tumor that grows in a part of the brain known as the pineal gland. It occurs mainly in children. […] The cause of pineoblastoma is unknown, but specific inherited variants in two genes, RB1 and DICER1 are associated with increased risk for developing pineoblastoma.

• #6

  https://braintumourresearch.org/pages/types-of-brain-tumours-pineal-region-tumours?srsltid=AfmBOopqohsPGAotCCRtcBVyYitQo9aYETP6-yI1MzPbfhMCgdoeVvfs

  Pineoblastomas develop from pinealocyte cells and/or their precursors (the stem and progenitor cells from which pinealocytes form). Pinealocytes are the main cells in the pineal gland that produce the hormone melatonin. […] Classified as WHO grade 4, pineoblastomas are fast-growing, malignant tumours that tend to invade nearby tissue within the brain and spine, carried in the cerebrospinal fluid (CFS). […] There is an association between pineoblastomas and hereditary retinoblastoma, a tumour that forms in the eye. Such patients tend to be diagnosed when they are around 1 year old. This combination of a retinoblastoma and pineoblastoma is called trilateral retinoblastoma. […] However, people living with an inherited genetic disorder called bilateral retinoblastoma are more likely to develop a pineal region tumour called pineoblastoma than people without this condition.

• #7 Pineoblastoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pineoblastoma/symptoms-causes/syc-20577723

  Pineoblastoma often isn’t known. This cancer starts as a growth of cells in the pineal gland. Pineoblastoma happens when cells in the pineal gland develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. […] The cancer cells form a growth in the pineal gland. The growth, called a tumor, may press on nearby parts of the brain as it gets bigger. The tumor also may block the flow of fluid in the brain. This causes pressure that builds up inside the brain.

• #8 Mayo Clinic Health Library – Pineoblastoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20310674

  The cause of pineoblastoma often isn’t known. This cancer starts as a growth of cells in the pineal gland. The pineal gland is located in the center of the brain. […] Pineoblastoma happens when cells in the pineal gland develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. […] The cancer cells form a growth in the pineal gland. The growth, called a tumor, may press on nearby parts of the brain as it gets bigger. The tumor also may block the flow of fluid in the brain. This causes pressure that builds up inside the brain.

• #9 Pineoblastoma | Expert Surgeon | Aaron Cohen-Gadol, MD

  https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/overview

  Pineoblastoma is a rare cancerous tumor arising from the pineal gland. […] The exact cause of pineoblastoma is not fully understood. Like many brain tumors, pineoblastomas are believed to result from genetic mutations that lead to abnormal cell growth and tumor formation. These mutations can be sporadic, meaning they occur by chance and are not inherited, or they can be part of a genetic syndrome. […] One genetic condition associated with an increased risk of developing pineoblastoma is hereditary retinoblastoma, which is caused by mutations in the RB1 gene. This condition primarily affects the retina of the eye but can also increase the risk of pineal gland tumors.

• #10 Pineal Region Tumors: Diagnosis and Treatment – NCI

  https://www.cancer.gov/rare-brain-spine-tumor/tumors/pineal-region-tumors

  Pineoblastomas can occur in people with the inherited genetic disorder bilateral retinoblastoma. […] The cause of most pineal region tumors is not known. […] Cancer is a genetic disease that is, it is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells.

• #11 Pineoblastoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pineoblastoma?embed_domain=hackmd.io%2525252F%25252540yIPUAFeCSL2JsU8smR5nJQ%2525252Fbnjhjgjghjghjgh&lang=us

  Pineoblastomas are the most aggressive pineal parenchymal tumor and account for a substantial proportion of such tumors (24-50%). They are typically found in young children, with only a slight female predilection (M:F 0.7:1; similar to other pineal parenchymal tumors), which is in contrast to the male predominance seen in pineal germinomas. […] There is a well-established association with hereditary retinoblastomas. Around 5% of patients with hereditary retinoblastoma (who predominantly have bilateral disease) develop midline (suprasellar or pineal) neuroblastic tumors. Such cases are sometimes referred to as trilateral retinoblastoma. […] Patients with DICER1 syndrome have an increased risk for developing pineoblastomas.

• #12 Pathology Outlines – Pineoblastoma

  https://www.pathologyoutlines.com/topic/cnstumorpineoblastoma.html

  Can be associated with DICER1 tumor predisposition syndrome (Acta Neuropathol 2021;141:771) […] RB1 altered subgroup can be seen in association with germline RB1 mutations (Acta Neuropathol 2020;139:243) […] Rare cases in patients with familial adenomatous polyposis (Dis Colon Rectum 2005;48:2343)

• #13 Research Advances the Genetic Understanding of Pineoblastoma, a Rare Brain Cancer | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/news/new-research-advances-genetic-understanding-pineoblastoma-rare-brain

  Pineoblastoma arises in the pineal gland. […] Sometimes pineoblastoma runs in families that have a certain inherited mutation. These inherited mutations lead to errors in one of the proteins that control small molecules called microRNAs. […] Another surprising finding was that, similar to what is seen in familial pineoblastoma, dysregulation of microRNAs appears to play a major role in the development of pineoblastoma that comes up sporadically. […] Further studies will be needed to assess the role of ARRB2 and the other genes we found to be recurrently mutated in pineoblastoma, Dr. Karajannis concludes.

• #14 A rare case of pineoblastoma – Case report – IJPO

  https://www.ijpo.co.in/html-article/22228

  Pineoblastoma is a very rare and aggressive malignant tumor of pineal gland. […] Pineoblastomas originate from pinealocytes and /or their precursors. […] Germline mutations of RB1 (retinoblastoma) and DICER 1 genes predispose to Pineoblastoma. […] In nonfamilial pineoblastoma, dysregulation of microRNA occurs because of mutated gene called DROSHA. […] Pineoblastoma should be differentiated from Germinoma, Papillary tumor of pineal region, pineocytoma and metastatic carcinomas. […] Initial treatment for Pineoblastoma often includes a shunting procedure for obstructive hydrocephalus and removal of tumor by surgery following radiation therapy to brain and spinal cord. […] Pineoblastoma is a very rare malignant tumor of pineal gland which should be differentiated from other pineal gland region tumors by histopathology and immunohistochemistry.

• #15 Recent Advances in Pineoblastoma Research: Molecular Classification, Modelling and Targetable Vulnerabilities

  https://www.mdpi.com/2072-6694/17/5/720

  In this context, the parallel between retinoblastoma and pineoblastoma is noteworthy. Both are driven by germline mutations and loss of heterozygosity (i.e., complete loss) of RB1. However, while a small group of retinoblastomas with intact, phosphorylatable pRB, is driven by MYCN amplifications, a major subset of pineoblastomas is induced by amplifications or stabilization of cMYC. Notably, at least in mice, cMYC and MYCN are genetically interchangeable. Thus, the reason for this tumor-specific preference for cMYC vs. MYCN amplification may be related to the effect of nearby genes that are co-amplified in each oncogenic event, or differences in mechanisms of stabilization and regulation of the respective onco-proteins. […] Germline mutations in DICER1, which cleaves pre-miRNA to form mature miRNA, also predispose individuals to PB. Such germline mutations, Dicer syndrome, increase the risk of tumors of the kidney, thyroid, ovary, cervix, testicle, brain, eye and lining of the lung without loss of heterozygosity (LOH). LOH of DICER1, leading to complete loss of this tumor suppressor, is associated with PB. In an early study, 4 DICER1 mutations were observed in 18 PB patients. In addition, homozygous deletion of DROSHA, a miRNA-processing enzyme upstream of DICER1, and duplication of PDE4DIP, phosphodiesterase 4D-interacting protein, have been identified in sporadic cases of PB.

• #16 Pineoblastoma – Wikipedia

  https://en.wikipedia.org/wiki/Pineoblastoma

  Pineoblastoma is a malignant tumor of the pineal gland. […] The exact cause of pineoblastoma is unknown. MicroRNA dysregulation has been found to be associated with many cases of pineoblastoma, specifically, mutations in DICER1 and DROSHA genes. […] Pineoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma. […] Additionally, various mutations or deletions in chromosomes 1, 9, 13, 16 and 22 have been associated with pineoblastoma incidence.

• #17 An imbalance between proliferation and differentiation underlies the development of microRNA-defective pineoblastoma

  https://genesdev.cshlp.org/content/early/2025/04/16/gad.352485.124

  Mutations in the microRNA processing genes DROSHA and DICER1 drive several cancers that resemble embryonic progenitors. […] To understand how microRNAs regulate tumorigenesis, we ablated Drosha or Dicer1 in the developing pineal gland to emulate the pathogenesis of pineoblastoma, a brain tumor that resembles undifferentiated precursors of the pineal gland. […] Pineal tumors driven by loss of Drosha or Dicer1 mimic tumors driven by Rb1 loss, as they exhibit upregulation of S-phase genes and homeobox transcription factors that regulate pineal development. […] Thus, we demonstrate that tumors driven by loss of microRNA processing may be therapeutically targeted by inhibiting downstream drivers of proliferation.

• #18 DNA methylation profiling from cerebrospinal fluid as a diagnostic tool for pineoblastoma | Acta Neuropathologica Communications | Full Text

  https://actaneurocomms.biomedcentral.com/articles/10.1186/s40478-025-01960-x

  Pineoblastoma is a rare and aggressive malignancy that often affects pediatric populations. […] Due to the rarity of these tumors, the current knowledge of the biology and the molecular features of PBs is limited thus impacting accurate diagnosis and treatment. […] DNA Methylation profiling of PB has allowed for the definition of the molecular identity of these tumors helping to discriminate them from other embryonal CNS tumors and to better clarify intertumoral heterogeneity among PB. […] Indeed based on molecular features, pineoblastomas have been divided into four molecular subtypes with distinct age of onset and prognosis: pineoblastoma, microRNA processing-altered 1; pineoblastoma, microRNA processing-altered 2; pineoblastoma, RB1-altered (pineal retinoblastoma); and pineoblastoma, MYC- and/or FOXR2-activated.

• #19 Pineoblastoma: Causes, Symptoms, & Treatment

  https://www.medicoverhospitals.in/diseases/pineoblastoma/

  The exact causes of pineoblastoma remain largely unknown. However, researchers have identified several potential risk factors: […] Certain genetic mutations, such as those affecting the RB1 gene, have been linked to an increased risk of developing pineoblastoma. […] While the exact causes of pineoblastoma are not fully understood, several risk factors have been identified: […] Conditions such as Li-Fraumeni syndrome and hereditary retinoblastoma increase the likelihood of developing pineoblastoma. […] Prior exposure to radiation, particularly at a young age, may elevate the risk of developing brain tumors, including pineoblastoma. […] Pineoblastoma is a rare tumor often associated with genetic mutations, including those linked to familial retinoblastoma.

• #20 Pineoblastoma – Causes, Symptoms, Diagnosis, and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/pineoblastoma

  Pineoblastoma is a rare and aggressive type of brain tumor that primarily affects the pineal gland, a small organ located deep within the brain. […] While the exact cause of pineoblastoma remains unclear, some studies suggest that environmental factors may play a role. Exposure to certain chemicals or radiation during critical periods of brain development could potentially increase the risk of developing this tumor. […] Genetic predispositions may also contribute to the development of pineoblastoma. Certain hereditary conditions, such as retinoblastoma, have been associated with an increased risk of developing pineoblastoma. […] Currently, there is limited evidence to suggest that lifestyle or dietary factors directly contribute to the development of pineoblastoma. […] Several risk factors have been identified that may increase the likelihood of developing pineoblastoma: Age: Pineoblastoma primarily affects children and young adults, with the majority of cases diagnosed in individuals under the age of 20.

• #21 Pineal Tumors: Symptoms, Diagnoses, and Treatment

  https://www.webmd.com/brain/what-to-know-about-pineal-tumors

  We still dont know what causes pineal tumors. But those with an inherited genetic disorder called bilateral retinoblastoma a rare form of cancer affecting the eyes may be at higher risk of developing pineal region tumors. […] Pineoblastoma is classified as WHO grade 4, a fast-growing, malignant tumor likely to invade and spread to nearby tissue in the brain and spine. Pineoblastomas are aggressive and the most difficult to treat. They occur slightly more frequently in women and are most often diagnosed in people under 20.

• #22 Childhood Medulloblastoma & Other CNS Embryonal Tumors Treatment – NCI

  https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq

  Medulloblastoma and other central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. […] Pineoblastoma forms in cells of the pineal gland. […] Certain genetic conditions increase the risk of childhood medulloblastoma. […] Pineoblastoma is linked with inherited changes in the retinoblastoma (RB1) gene. A child with the inherited form of retinoblastoma (cancer that forms in the tissues of the retina) has an increased risk of pineoblastoma. […] Childhood medulloblastoma is caused by certain changes to the way brain cells function, especially how they grow and divide into new cells. Often, the exact cause of the cell changes is unknown. […] The risk for medulloblastoma is increased in people who have any of the following inherited diseases: Turcot syndrome, Rubinstein-Taybi syndrome, Nevoid basal cell carcinoma (Gorlin) syndrome, Li-Fraumeni syndrome, Fanconi anemia. […] Genetic counseling may be done for children with medulloblastoma or pineoblastoma.

• #23 Pineoblastoma – Brief information

  https://www.gpoh.de/kinderkrebsinfo/content/diseases/brain_tumours/pohpatinfomedullo120080109/literatur/index_eng.html

  A pineoblastoma is caused by a malignant transformation of nerve tissue cells. The reasons for tumour development have not been completely found out yet. It is well-known, though, that radiotherapy of the brain, for example as received by children with certain forms of leukaemia or with eye cancer (retinoblastoma), leads to an increased risk of developing a CNS tumour later in life. […] In addition, it has been shown that pineoblastoma are sometimes associated with certain genetic and chromosomal abnormalities in the tumour cells. The resulting impairments of cell development and cell communication may be contributing factors promoting the transformation of a healthy into a cancer cell. However, since pineoblastomas are rare, only a few molecular abnormalities that might be responsible for causing the disease have been identified yet. […] Rarely, pineoblastoma can be associated with hereditary retinoblastoma and thus genetic alterations of the retinoblastoma gene (so-called trilateral retinoblastoma).

• #24 Pineoblastoma | The Brain Tumour Charity

  https://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/pineoblastoma/

  Pineoblastoma is a primary brain tumour, meaning that it starts in the brain as opposed to spreading there. […] It originates from embryonal cells which were left behind from the time the person was a developing fetus. Healthy embryonal cells go on to differentiate and play a part in the growth of body parts such as the pineal gland and retina. In the case of pineoblastomas these cells have divided incorrectly causing an abnormal growth or tumour. […] Pineoblastomas are rare brain tumours. But, unfortunately, they are difficult to treat in the people who do develop them.

• #25 Pineoblastoma Symptoms | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD

  https://www.aaroncohen-gadol.com/en/patients/pineoblastoma/types/symptoms

  The exact cause of pineoblastoma remains unknown, but researchers believe a combination of genetic and environmental factors may contribute to its development. Understanding possible risk factors can help provide some insight. […] Certain genetic changes are associated with an increased risk of pineoblastoma. For example, children with hereditary retinoblastoma, a rare eye cancer, have a higher chance of developing pineoblastoma. This connection suggests that some people may have a genetic predisposition that makes them more vulnerable to this type of brain tumor. […] Although less understood, some studies have explored potential environmental risk factors, like exposure to radiation. However, there is no conclusive evidence linking specific environmental exposures to the development of pineoblastomas.

• #26 Prognostic factors and outcome of pineoblastoma: 10 years single-center experience | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-021-00083-3

  The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. […] The survival of patients with metastatic disease did not differ between age groups. However, age had a significant impact on the outcome of M0 disease, with 3-year EFS and OS of 65.3% and 74%, respectively, in the older group compared to 0% for both rates in younger patients. […] A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastomas dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. […] The impact of age on outcome is mostly due to different molecular subgroups that affected the response to treatment and metastases occurrence.

• #27 Prognostic factors and outcome of pineoblastoma: 10 years single-center experience | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-021-00083-3

  The survival discrepancy between our study and the COG 99701 may be attributed to our centers lack of chemotherapy dose intensity policy. […] There is great need to classify pineoblastoma molecularly based on copy number, whole exome sequencing analysis to label patients with poor prognostic molecular groups (MYC, RB) who need intensified therapy, and those with excellent survival who harbored post-transcriptional regulators endonucleases mutations as DROSHA, DGCR8, and DICER1 with survival up to 100%.

• #28 Treatment outcomes for pediatric pineoblastoma: a single institute experience in Taiwan – Kang – Therapeutic Radiology and Oncology

  https://tro.amegroups.org/article/view/4422/html

  The pineoblastomas are a rare malignant tumor, with an estimated incidence of 0.1% of all intracranial tumors, making the development of treatment consensus difficult. […] Pineoblastoma is a WHO grade IV tumor of the CNS that features aggressive growth and early distant metastasis. […] Several factors have been examined to predict the prognosis of pineoblastoma patients, including age, sex, distant metastasis at diagnosis, hydrocephalus at diagnosis, residual tumor after operation, and initial treatment modalities. Age at diagnosis is an important prognostic factor. […] Residual disease after operation is also an important prognostic factor for patients with pineoblastomas. […] Distant metastasis at diagnosis is considered a poor prognostic factor, and has been discussed in prior studies.

• #29 :: BTRT :: Brain Tumor Research and Treatment

  https://btrt.org/DOIx.php?id=10.14791/btrt.2023.0033

  This study underscores the prognostic significance of WHO grades in PPT. It is necessary to provide specific treatment according to tumor grade. Grade 3 PPTID showed a poor prognosis. Potential LMS and malignant transformations necessitate aggressive multimodal treatment and close-interval screening. […] Three patients experienced malignant transformations. […] The present study indicates that the prognosis of PPT obviously varies depending on the histopathological grade. In particular, grade 3 PPTID revealed a distinct clinical course compared to grade 2 tumors. Cases of rare malignant transformations were also observed. […] Malignant transformation of PPT has been reported in several studies. The progression-free period ranged from 3 to 10 years. Most of the cases were transformed from PC to PPTID. We reported two cases from PPTID to PB.

• #30 Modeling germline mutations in pineoblastoma uncovers lysosome disruption-based therapy | Nature Communications

  https://www.nature.com/articles/s41467-020-15585-2

  Pineoblastoma is a rare pediatric cancer induced by germline mutations in the tumor suppressors RB1 or DICER1. […] About 5% of patients with bilateral retinoblastoma (RB), an ocular tumor of infancy, develop PB, termed trilateral RB, directly implicating the tumor suppressor RB1 in the aetiology of this disease. […] Germline mutation in DICER1 also predisposes to PB, whereas DROSHA loss and PDE4DIP duplication are found in sporadic cases. […] These results demonstrate the utility of the WAP-Cre transgene for modeling diverse types of PBs, and suggesting similar cell of origin for RB1- and DICER1-deficient PB. […] Thus, our results model the two most common germline mutations, RB1 and DICER1, that predispose children to PB, and uncover a new therapeutic avenue for this devastating disease.

• #31 Modeling germline mutations in pineoblastoma uncovers lysosome disruption-based therapy | Nature Communications

  https://www.nature.com/articles/s41467-020-15585-2

  In addition to RB1 loss, germline mutation in DICER1, a ribonuclease involved in microRNA processing and DNA repair, also predisposes children to PB. […] Although the latency of WAP-Cre:Dicer1flox/flox:p53flox/flox mice was significantly longer than that of WAP-Cre:Rbflox/flox:p53flox/flox mice, they developed full blown PB with characteristic pleomorphic nuclei, scarce cytoplasm and rosette structures. […] Together, these results demonstrate that mouse and human PBs with different oncogenic drivers exhibit elevated sensitivity to NOR/lysosome disruption, indicating the potential benefit of this drug for diverse types of PB.

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