Publications by authors named "Ralph Ermoian"

Introduction: Proton beam therapy (PT) offers a targeted approach to treating pediatric tumors by minimizing radiation exposure to surrounding healthy tissue. However, the specific toxicity profile associated with it remains underexplored. This study aims to investigate the incidence and risk factors of acute and late treatment-related toxicities following PT in pediatric patients with pelvic tumors.

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Background: Effective therapy for medulloblastoma at the time of relapse is limited. The objective of this study is to review outcomes from the Seattle Children's Hospital (SCH) institutional standard therapy for relapsed medulloblastoma, modified from the published ACNS0821 regimen.

Methods: Retrospective review of patients treated for relapsed medulloblastoma from 2012-2024 treated with modified ACNS0821 therapy, including combination bevacizumab, irinotecan, and temozolomide, referred to as ".

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Purpose: Embryonal tumor with multilayered rosettes (ETMR) is a rare pediatric CNS embryonal tumor with poor survival. The Pediatric Proton/Photon Consortium Registry (PPCR) was queried for outcomes data from prospectively consenting pediatric patients with ETMR treated with proton radiation therapy (RT).

Methods: 20 patients (2013-2021) at 9 institutions had ETMR; 2 with prior RT were excluded from statistical analyses (PPCR ETMR, N = 18).

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Purpose: Medulloblastoma is one of the most common malignant brain tumors in children, and 75% of patients achieve long-term survival. There are limited studies reporting acute toxicity for pediatric patients receiving proton therapy for craniospinal irradiation (CSI) in medulloblastoma, and these are limited by their retrospective nature, modest cohort sizes, and lack of a comparator group.

Materials And Methods: We analyzed data from the Pediatric Proton/Photon Consortium Registry.

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The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Pediatric Central Nervous System Cancers provide multidisciplinary diagnostic workup, staging, and treatment recommendations for diffuse high-grade gliomas and medulloblastomas in children and adolescents. This article summarizes the studies and panel discussion that serve as the rationale for comprehensive care recommendations included in the NCCN Guidelines for Pediatric Central Nervous System Cancers.

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Background: Treatment of pediatric craniopharyngioma requires a multidisciplinary approach to counsel patients and families on the spectrum of treatment options, including biopsy, radiation, and/or resection. Gross-total resection can avoid radiation and its long-term comorbidities. In very young patients, this is of particular importance but is especially challenging because of anatomical considerations.

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As the peak incidence of isocitrate dehydrogenase (IDH)-mutant gliomas is amongst young adults, there is a need to balance tumor control with long term side effects of therapy. Following initial clinical presentation and acquisition of contrasted diagnostic imaging, tissue diagnosis is essential in suspected diffuse glioma. Depending on the location and extent of disease, maximal surgical resection is preferred both for histologic diagnosis and initial therapy.

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Central nervous system (CNS) embryonal tumors represent a diverse group of neoplasms and have a peak incidence in early childhood. These tumors can be located anywhere within the CNS, and presenting symptoms typically represent tumor location. These tumors display distinctive findings on neuroimaging and are staged using magnetic resonance imaging of the brain and spine as well as evaluation of cerebrospinal fluid.

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Purpose: Increasing concern that brainstem toxicity incidence after proton radiation therapy might be higher than with photons led to a 2014 University of Florida (UF) landmark paper identifying its risk factors and proposing more conservative dose constraints. We evaluated how practice patterns changed among the Pediatric Proton/Photon Consortium Registry (PPCR).

Material And Methods: This prospective multicenter cohort study gathered data from patients under the age of 22 years enrolled on the PPCR, treated between 2002 and 2019 for primary posterior fossa brain tumors.

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Purpose: Pediatric acute myeloid leukemia (AML) often involves extramedullary sites, which can be resistant to standard induction chemotherapy. Consolidative radiation therapy can be used in select cases to improve local control rates and help bridge patients to curative stem cell transplants. However, there is no previously published data to support the use of proton radiotherapy (PT) in this setting.

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Background And Objective: The use of radiotherapy (RT) in the palliative and emergent settings for pediatric cancers is an under-utilized resource. Our objective was to provide an evidence-based review of the data to increase awareness of the benefit for this population along with providing guidance on pediatric specific treatment considerations for palliative care physicians, pediatric oncologists, and radiation oncologists.

Methods: A narrative review was performed querying PubMed, MEDLINE, ClinicalTrials.

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Article Synopsis
  • * Of the 39 patients treated, most were young males who received median radiation doses of 18 Gy to the brain and 12 Gy to the spine, with a 100% CNS-response rate in patients with CSF-positive disease.
  • * Results showed that CSI was well-tolerated with a low rate of CNS local recurrence (14%) after two years, highlighting that managing systemic disease is crucial for preventing CNS relapses.
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Article Synopsis
  • The case series examines three children diagnosed with Medulloblastoma, a type of brain cancer, and highlights the differences between somatic and germline mutations, specifically focusing on the ATM gene mutation.
  • It discusses the implications of these mutations for treatment and prognosis in the context of pediatric oncology.
  • The findings aim to improve understanding of genetic factors in medulloblastoma, potentially guiding personalized treatment options for affected children.
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This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on considerations for the comprehensive care of AYA patients with cancer. Compared with older adults with cancer, AYA patients have unique needs regarding treatment, fertility counseling, psychosocial and behavioral issues, and supportive care services. The complete version of the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.

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Purpose: Atypical teratoid/rhabdoid tumors (ATRT) of the central nervous system (CNS) are rare tumors with a poor prognosis and variable use of either focal or craniospinal (CSI) radiotherapy (RT). Outcomes on the prospective Pediatric Proton/Photon Consortium Registry (PPCR) were evaluated according to RT delivered.

Methods: Pediatric patients receiving RT were prospectively enrolled on PPCR to collect initial patient, disease, and treatment factors as well as provide follow-up for patient outcomes.

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Purpose: Total body irradiation (TBI), a form of immunomodulation, improves treatment outcomes for rapidly progressive scleroderma. The landmark Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial used strict 200-cGy lung and kidney dose restrictions to limit the likelihood of normal tissue toxicity. The protocol as written did not specify how or where the 200-cGy limit was to be measured, opening the door to variable techniques and outcomes.

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Article Synopsis
  • - The study analyzed data from 218 patients with orbital rhabdomyosarcoma (ORMS) enrolled in Children's Oncology Group trials between 1997 and 2013, focusing on demographics, disease characteristics, and outcomes across different subgroups of ORMS.
  • - For patients with low-risk ORMS (192 individuals), the 10-year event-free survival (EFS) rate was 85.5%, and the overall survival (OS) rate was 95.6%, indicating excellent outcomes.
  • - Even patients with recurrent ORMS showed promise, with a 10-year OS rate of 69.4% following recurrence, suggesting that a significant number may achieve long-term survival despite earlier challenges.
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Article Synopsis
  • - CNS cancers represent about 25% of all pediatric tumors and are the leading cause of cancer deaths in children, with over 4,000 diagnoses each year.
  • - The NCCN Guidelines for Pediatric CNS Cancers specifically address the diagnosis and management of diffuse high-grade gliomas, which are highly aggressive with a poor 5-year survival rate of less than 20%.
  • - Advances in molecular profiling have led to targeted therapies, but clinical trial participation remains the preferred treatment option for eligible patients.
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Purpose: Proton radiation therapy (PR) is well established in the treatment of pediatric malignancies in the central nervous system (CNS) with dosimetric advantages that reduce late radiation therapy (RT) effects. In this analysis, we sought to evaluate the utilization of PR in children with primary CNS malignancies and characterize the clinical and sociodemographic factors predictive of receipt of PR.

Methods And Materials: The National Cancer Database was queried to identify all pediatric patients with primary CNS malignancies treated with curative intent RT from 2004 to 2017.

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Background: Children with diffuse intrinsic pontine gliomas (DIPG) have a dismal prognosis. Adavosertib (AZD1775) is an orally available, blood-brain barrier penetrant, Wee1 kinase inhibitor. Preclinical efficacy against DIPG is heightened by radiation induced replication stress.

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