Background: Glomus tumors around the jugular foramen and inner ear can have variable presentations, including lower cranial nerve palsies, tinnitus, hearing loss, or palpable neck mass. In general, these tumors are benign paragangliomas with the definitive treatment consisting of radiosurgery or surgery. Endovascular embolization can be added as a critical adjunctive therapy to reduce the tumor vascularity before surgical resection.
View Article and Find Full Text PDFPediatr Blood Cancer
February 2024
Background And Purpose: Emerging data suggest immune checkpoint inhibitors (ICI) and stereotactic radiosurgery (SRS) or radiotherapy (SRT) may work synergistically, potentially increasing both efficacy and toxicity. This manuscript characterizes factors associated with intracranial control and radiation necrosis in this group.
Materials And Methods: All patients had non-small cell lung cancer, renal cell carcinoma, or melanoma and were treated from 2013 to 2021 at two institutions with ICI and SRS/SRT.
Radiotherapy (RT) is essential for multimodality treatment of pediatric renal tumors, particularly in higher-risk and metastatic disease. Despite decades of use, particularly for Wilms tumor, there remain controversies regarding RT indications, timing, dose, and targets. To align global management, we address these issues in this international HARMONIsation and CollAboration (HARMONICA) project.
View Article and Find Full Text PDFBackground And Importance: Aggressive pituitary adenomas (APAs) are pituitary tumors that are refractory to standard treatments and carry a poor prognosis. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy. Temozolomide is the only chemotherapeutic agent with documented effectiveness and is recommended for APA in European Society of Endocrinology clinical guidelines.
View Article and Find Full Text PDFThe survival of childhood Wilms tumor is currently around 90%, with many survivors reaching reproductive age. Chemotherapy and radiotherapy are established risk factors for gonadal damage and are used in both COG and SIOP Wilms tumor treatment protocols. The risk of infertility in Wilms tumor patients is low but increases with intensification of treatment including the use of alkylating agents, whole abdominal radiation or radiotherapy to the pelvis.
View Article and Find Full Text PDFThe purpose of this study was to compare dose to anterior organs at risk (OARs) and quantify the risk of developing secondary malignancy (SMN) in pediatric patients treated with vertebral-body-sparing (VBS) vs vertebral body (VB) pencil beam scanning proton craniospinal irradiation (CSI). Comparative plans of VBS and VB CSI were created for 10 previously treated patients. Dose-volume histograms were used to evaluate dose to OARs.
View Article and Find Full Text PDFThe use of radiation therapy is an important part of multimodality treatment for rhabdomyosarcoma. The specific doses, treatment volumes, and techniques used in radiation therapy can vary dramatically based upon a number of factors including location, tumor size, and molecular characteristics, resulting in complex decisions in treatment planning. This article reviews the principles of evaluation and management for head and neck rhabdomyosarcoma including a summary of the historical studies upon which current management is based.
View Article and Find Full Text PDFIntroduction: I-meta-iodobenzylguanidine ( I-MIBG) is effective in relapsed neuroblastoma. The Children's Oncology Group (COG) conducted a pilot study (NCT01175356) to assess tolerability and feasibility of induction chemotherapy followed by I MIBG therapy and myeloablative busulfan/melphalan (Bu/Mel) in patients with newly diagnosed high-risk neuroblastoma.
Methods: Patients with MIBG-avid high-risk neuroblastoma were eligible.
Purpose: Cyclin-dependent kinase-retinoblastoma (CDK-RB) pathway is dysregulated in some diffuse intrinsic pontine gliomas (DIPG). We evaluated safety, feasibility, and early efficacy of the CDK4/6-inhibitor ribociclib, administered following radiotherapy in newly-diagnosed DIPG patients.
Methods: Following radiotherapy, eligible patients received ribociclib in 28-day cycles (350 mg/m; 21 days on/7 days off).
J Thromb Thrombolysis
May 2021
Cardiac disease following radiation therapy represents a major consideration in the treatment of a variety of malignancies. Damage to the heart can manifest in a variety of pathologies including ischemic cardiac disease, cardiomyopathy, valvular dysfunction, arrhythmias, and pericarditis. This damage has been shown to directly relate to cardiac radiation dose and to stem from a range of cellular pathways that are often related to fibrosis.
View Article and Find Full Text PDFInt J Radiat Oncol Biol Phys
February 2021
Purpose: Treatment with radiation therapy (RT) can cause anxiety and distress for pediatric patients and their families. Radiation oncology teams have developed strategies to reduce the negative psychological impact. This survey study aimed to characterize these methods.
View Article and Find Full Text PDFThe objectives for the treatment of Wilms tumor in both the Children's Oncology Group (COG) and the International Society of Paediatric Oncology (SIOP) have focused on improving cure rates and minimizing toxicity by limiting the use of radiation and doxorubicin. Although the timing of surgery is different in COG (upfront surgery) and SIOP (upfront chemotherapy with delayed surgery), both are effective strategies and have the same survival. Fewer patients are treated with radiotherapy in the SIOP trials but with higher doses.
View Article and Find Full Text PDFSCCOHT is an aggressive malignancy linked to alterations of SMARCA4. We describe the diagnosis and therapy of a 32 year old who received multi-agent chemotherapy and underwent a second look operation with HIPEC followed by high-dose chemotherapy with stem cell transplant. Supportive care, oncofertility, and genetic counseling are described.
View Article and Find Full Text PDFPurpose: Craniospinal irradiation (CSI) is an important component of therapy for many pediatric central nervous system malignancies. Proton therapy is increasingly available and used for minimizing radiation exposure to normal tissues. The absence of an exit dose with proton therapy mandates decisions regarding coverage of the vertebral bodies (VB) in non-skeletally mature patients.
View Article and Find Full Text PDFPurpose: Single-fraction stereotactic radiosurgery(SRS) for meningioma has high rates of symptomatic perilesional edema in some settings. Fractionated stereotactic radiosurgery(fSRS) could decrease edema rates while maintaining tumor control.
Methods And Materials: Patients at an institution were retrospectively reviewed(2013-2017).
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft-tissue sarcoma with an unfavorable prognosis and limited therapeutic options. MPNSTs can be sporadic, but are often associated with neurofibromatosis (NF) 1 and usually arise from preexisting neurofibromas. MPNSTs in patients with NF2 have been reported in only exceedingly rare cases, and the mechanisms underlying transformation into an MPNST have not been fully elucidated.
View Article and Find Full Text PDFHead and neck rhabdomyosarcoma lymph node staging is challenging due to varied patterns of lymphatic drainage and the suboptimal predictive value of available imaging modalities. Furthermore, regional relapse rates are unacceptably high, and the toxicity of empiric radiation is undesirable in the pediatric and young adult population. In an attempt to improve locoregional control without excess morbidity, we have adopted routine sentinel lymph node biopsy in head and neck rhabdomyosarcoma, which is safe and feasible in pediatric patients.
View Article and Find Full Text PDFRadiotherapy plays an important role in the management of childhood cancer, with the primary aim of achieving the highest likelihood of cure with the lowest risk of radiation-induced morbidity. Proton therapy (PT) provides an undisputable advantage by reducing the radiation 'bath' dose delivered to non-target structures/volume while optimally covering the tumor with tumoricidal dose. This treatment modality comes, however, with an additional costs compared to conventional radiotherapy that could put substantial financial pressure to the health care systems with societal implications.
View Article and Find Full Text PDFIn patients with pituitary adenomas (PA) who are unable to undergo complete surgical resection, radiation therapy (RT), specifically stereotactic radiosurgery (SRS), results in excellent local control. However, the utility of radiosurgery may be limited by the proximity of the lesion to the optic chiasm (OC). We evaluate the efficacy of debulking surgery in increasing the PA-OC separation to convert patients into SRS candidates.
View Article and Find Full Text PDFRhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood with 250-350 cases diagnosed annually in the United States. Biliary tract rhabdomyosarcoma is rare, representing <1% of the RMS cases. Due to its location, resection is clinically challenging, and functional complications exist and persist from biliary obstruction.
View Article and Find Full Text PDFPurpose: Diffuse intrinsic pontine glioma (DIPG) is a devastating pediatric disease, with a median survival of <1 year. Here, we review our institution's DIPG experience over an 8-year interval and perform a systematic review of the literature, specifically evaluating reports of reirradiation (reRT) for DIPG.
Methods And Materials: We retrospectively reviewed the medical records of 26 patients who underwent definitive intensity modulated radiation therapy (IMRT) for DIPG at a single institution between 2007 and 2015.
Neurofibromatosis type 2 (NF-2) represents the complex issue of hearing restoration after treatment for a patient with bilateral acoustic neuromas. This scenario is difficult for skull base teams considering that all treatment options (including observation of tumors) pose a risk to the patient for further or total hearing loss. In this case of a patient with bilateral deafness, restoration options were auditory brainstem or cochlear implantation (CI).
View Article and Find Full Text PDFBackground: Recent studies questioning the benefit of prostate-specific antigen (PSA) screening have increased the need for evaluating factors contributing to variance in levels and their clinical relevance. An inverse relationship between body mass index (BMI) and PSA has been illustrated, however the clinical implications have not been specified. We performed a retrospective review of patients screened through our free screening clinic to delineate any relationship between PSA and BMI in an attempt to understand its possible clinical significance.
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