A Pilot Study of Low-Dose Craniospinal Irradiation in Patients With Newly Diagnosed Average-Risk Medulloblastoma.

Purpose: Medulloblastoma is one of the most common malignant brain tumors in children. To date, the treatment of average-risk (non-metastatic, completely resected) medulloblastoma includes craniospinal radiation therapy and adjuvant chemotherapy. Modern treatment modalities and now risk stratification of subgroups have extended the survival of these patients, exposing the long-term morbidities associated with radiation therapy.

Vision-related quality-of-life in pediatric primary brain tumor patients.

Purpose: Brain tumors are the leading cause of death from childhood cancer. Although overall survival has improved due to earlier detection, better therapies, and improved surveillance, visual dysfunction and impaired vision-related quality-of-life (VR-QOL) are often unrecognized in children. This project investigated VR-QOL in pediatric brain tumor patients.

Impact of executive functioning on health-related quality of life of pediatric brain tumor survivors.

Background: Brain tumor survivors are at risk for significant late effects following treatment completion that may adversely impact health-related quality of life (HRQOL). The current study examines the relationship between executive functioning (EF) and HRQOL in pediatric brain tumor survivors within a longitudinal framework. We hypothesized that early deficits in EF would be related to less optimal HRQOL in this population.

Guidelines for Treatment and Monitoring of Adult Survivors of Pediatric Brain Tumors.

Pathologies of pediatric brain tumors are more varied than those diagnosed in adults and survival outcomes more optimistic. Therapies for pediatric brain tumors are also diverse and treatment options are expanding. The growing number of adult survivors of childhood brain tumors is quite diverse.

Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy.

Introduction: Diffuse leptomeningeal glioneuronal tumors (DLGT) have been recognized in the most recent WHO classification as a distinct entity.

Objective: We describe seven pediatric cases of DLGT and the responses to therapy and outcome.

Methods: We conducted a retrospective review of charts from 1985 to 2013.

Successful treatment of a child with a prolactin secreting macroadenoma with temozolomide.

Prolactinomas are a rare subset of brain tumors in pediatrics. We report a child with a prolactin secreting macroadenoma which was refractory to initial treatment with a dopamine antagonist. Given the location of her tumor she was ineligible for surgical resection.

The feasibility of frameless stereotactic radiosurgery in the management of pediatric central nervous system tumors.

Recurrent malignant primary and metastatic central nervous system (CNS) tumors in pediatric patients are devastating, and efforts to improve outcomes for these patients have been disappointing. Conventional re-irradiation in these patients increases the risk of significant toxicity. We therefore evaluated feasibility and outcomes using frameless radiosurgery (FRS) in children with recurrent primary and metastatic brain tumors.

Diffuse leptomeningeal neuroepithelial tumor: 9 pediatric cases with chromosome 1p/19q deletion status and IDH1 (R132H) immunohistochemistry.

Leptomeningeal dissemination in children is typical of high-grade, and occasionally low-grade, neoplasms. Rare cases of widely disseminated oligodendroglia-like leptomeningeal tumors, sometimes with associated spinal cord lesions, have been described that respond to treatment and follow an indolent course. Whether these lesions represent an established tumor category or are a unique entity remains to be established.

Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation.

Clinical experience suggests that craniopharyngiomas may temporarily increase in size after radiation therapy (RT). The study goal is to determine the incidence and natural history of this response in a cohort of patients managed at Children's Healthcare of Atlanta (CHOA) or Emory Healthcare (EHC). Between 08/1998 and 06/2009, 41 children and young adults were diagnosed with craniopharyngioma at CHOA and/or EHC.

Seventeen-year-old adolescent with pituitary abscess.

Pituitary abscess is a rare but potentially life-threatening infectious process. Diagnosis is challenging as symptoms are non-specific and signs of infection may be absent. We report the case of a previously healthy 17-year-old male who presented with worsening headaches, polyuria, polydipsia and no clinical signs of infection.

A phase II study of metronomic oral topotecan for recurrent childhood brain tumors.

Background: The prognosis for recurrent or refractory brain tumors in children is poor with conventional therapies. Topotecan is a topoisomerase I inhibitor with good central nervous system (CNS) penetration following oral administration. Increased efficacy of topotecan has been demonstrated with prolonged low-dose daily treatment in pre-clinical models.

De novo germline TP53 mutation presenting with synchronous malignancies of the central nervous system.

We present a case of a 14-year-old male with a germline TP53 mutation who presented with synchronous primitive neuroectodermal tumor and choroid plexus carcinoma. Identification of synchronous brain tumors prompted genetic testing for predisposition to malignancy. Within 5 months of presentation, the child developed widely metastatic alveolar rhabdomyosarcoma.

Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma.

Purpose: To investigate the incidence, risks, severity, and sequelae of posterior fossa syndrome (PFS) in children with medulloblastoma.

Methods And Materials: Between 1990 and 2007, 63 children with medulloblastoma at Emory University and Children's Healthcare of Atlanta were treated with craniectomy followed by radiation. Fifty-one patients were assigned to a standard-risk group, and 12 patients were assigned to a high-risk group.

Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor.

Purpose: Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months. Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT.

Patients And Methods: Treatment was divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, and continuation therapy.

Childhood intracranial ependymoma: twenty-year experience from a single institution.

Background: Because few large studies of pediatric ependymoma treatment are available, the authors believed that a retrospective review of treatment outcomes from a single institution would yield potentially valuable information regarding potential prognostic factors. In this article, they report their 20-year institutional experience with this disease.

Methods: Medical records were reviews of patients with intracranial ependymoma who received their initial treatment at the Children's Hospital of Philadelphia (CHOP)/Hospital of the University of Pennsylvania (HUP) between January 1980 and December 2000.

Congenital glioblastoma: a clinicopathologic and genetic analysis.

Congenital central nervous system (CNS) tumors are uncommon, accounting for 1% of all childhood brain tumors. They present clinically either at birth or within the first 3 months. Glioblastoma (GBM) only rarely occurs congenitally and has not been fully characterized.

Adult heights attained by children with hypothalamic/chiasmatic glioma treated with growth hormone.

Hypothalamic/chiasmatic gliomas (H/CG) in children are commonly accompanied by endocrine dysfunction due to mass effects of the tumor itself or as a consequence of tumor therapy, with GH deficiency (GHD) being the most common disorder. We report the height outcomes of GH-treated H/CG patients with GHD. We reviewed the records of 14 GHD patients with H/CG who were treated with human GH.

Amifostine for children with medulloblastoma treated with cisplatin-based chemotherapy.

In adult patients, amifostine appears to ameliorate cisplatin-related nephrotoxicity and ototoxicity. We assessed the safety and efficacy of amifostine in 11 children with newly diagnosed medulloblastoma/primitive neuroectodermal tumor treated with radiotherapy and vincristine, lomustine, and cisplatin. Amifostine was administered immediately prior to and 4 hr into the cisplatin infusion.

Near complete surgical resection predicts a favorable outcome in pediatric patients with nonbrainstem, malignant gliomas: results from a single center in the magnetic resonance imaging era.

Background: Because few reports on outcome in patients with pediatric malignant gliomas during the magnetic resonance imaging era were available, the authors studied the outcomes of children with these tumors at their institution.

Methods: The medical records of 39 patients with nonbrainstem, malignant gliomas who were treated at the Hospital of the University of Pennsylvania/Children's Hospital of Philadelphia between February 1, 1989 and December 31, 2000 were reviewed retrospectively. Magnetic resonance imaging was used to assess tumors at presentation and at follow-up.

Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry.

Purpose: Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor.

Endocrine outcome in children with medulloblastoma treated with 18 Gy of craniospinal radiation therapy.

Craniospinal radiation therapy (CSRT) combined with chemotherapy results in significant endocrine morbidity. Between 1987 and 1990, a trial using 18 Gy was conducted to treat 10 young children with medulloblastoma. There were 7 survivors.

Optic radiation involvement in optic pathway gliomas in neurofibromatosis.

Purpose: Optic pathway gliomas (pilocytic astrocytomas) in neurofibromatosis type 1 (NF-1) typically involve some combination of the optic nerves, chiasm, or optic tracts. Involvement of the optic radiations is rare.

Design: This paper describes seven patients with NF-1 with gliomas involving the pregeniculate optic pathway in addition to the optic radiations.