Outcomes of pediatric low-grade gliomas treated with radiation therapy: a single-institution study.

Radiation therapy is often considered the treatment of choice for low-grade gliomas. However, given the long-term effects of radiation on the developing brain, the appropriate use of radiation therapy in pediatric patients remains controversial. The purpose of this study was to evaluate progression-free survival (PFS) of pediatric low-grade glioma patients treated with radiation therapy. Data were obtained through a retrospective chart review of patients treated between 1991 and 2008 from a single tertiary care center in the midwest. The study population consisted of 17 patients, of whom 8 (47%) had tumor recurrence after radiation therapy. The median follow-up time was 8.2 years, with a range of 2.3 to 17.2 years. The median age at diagnosis was 5.4 years, and the median age at radiation therapy was 9.4 years. The 3- and the 10-year PFS were 69%± 11.7% and 46%± 13.3%, respectively. A significant difference in PFS was seen when comparing brainstem tumors with hypothalamic/optic pathway tumors (P=0.019). Differences in PFS based on the age at diagnosis, the extent of initial surgery, and indication for radiation therapy were not significant. A larger multicenter study is needed to better assess PFS in these patients.

Widely Metastatic Choroid Plexus Carcinoma Associated with Novel TP53 Somatic Mutation.

BACKGROUND: Choroid plexus carcinoma (CPC) is a rare, malignant tumor occurring more commonly in children than adults. This case report describes the clinical course of a 3-year-old boy with a rare case of metastatic CPC with a novel TP53 mutation. CASE DESCRIPTION: A 3-year-old boy presented with postconcussive symptoms after a fall. Computed tomography and magnetic resonance imaging revealed lesions in the suprasellar cistern, left lateral ventricle, and cauda equina. The tumor was diagnosed as choroid plexus carcinoma with a novel TP53 V216M somatic mutation. The patient underwent resection of the left lateral ventricle lesion. CONCLUSION: We describe a case of CPC with highly metastatic characteristics and a novel TP53 mutation. Our report implicates TP53 in the pathogenesis of pediatric CPC, and we emphasize that CPC in children should prompt careful consideration of TP53 status to inform prognosis and clinical treatment.

Pilot Evaluation of a Palliative and End-of-Life Communication Intervention for Parents of Children With a Brain Tumor.

Providing timely palliative and end-of-life care (PC/EOL) information to parents of children with a serious illness is a national health care priority. The goals of this study were to determine feasibility, acceptability, and parent responses related to a PC/EOL communication intervention, titled "Communication Plan: Early through End of Life (COMPLETE)" to parents of children with a brain tumor. The study was a 2-site prospective, single-group pilot study targeting parents' stress and coping outcomes. The sample included 13 parents of 11 children (ie, 11 families). During the first 6 months postdiagnosis, we evaluated parent outcomes at 4 time points (baseline and 3 post-sessions). Our findings included significant decline in decision regret ( P = .0089); strong, significantly increased hope ( P ≤ .0001); and significantly decreased uncertainty ( P = .04). Over time, more than half of the parents (61.5%) preferred to receive information about their child's current condition and PC/EOL options. Our findings provide evidence to suggest that the COMPLETE intervention is feasible and acceptable and produces promising effects on 3 parent outcomes (ie, decision regret, hope, and uncertainty) in parents of children with a brain tumor. Further research is indicated to evaluate COMPLETE with a larger sample of parents of children with cancer and with a control group.

MRI detection of early bone metastases in b16 mouse melanoma models.

Bone metastasis causes significant morbidity in cancer patients, including bone pain, pathologic fractures, nerve compression syndrome, and hypercalcemia. Animal models are utilized to study the pathogenesis of skeletal metastases and to evaluate potential therapeutic agents. Previously published methods for imaging bone metastasis in rodent models have focused on identifying advanced stage metastasis using simple X-rays. Here we report MRI as a method for detecting early bone metastases in mouse models in vivo. B16 mouse melanoma cells were injected into the left cardiac ventricle of C57BL/6 mice and magnetic resonance (MR) images were obtained of the left leg following the development of metastatic disease, when tumor associated bone destruction was histologically present but not visible by X-ray. T1 and T2 relaxation times of bone marrow were measured in healthy control mice and B16 melanoma tumor-bearing mice. Mean T2 values for normal marrow were 28 ms (SD 5) and for diseased bone marrow were 41 ms (SD 3). T2 relaxation time of diseased bone marrow is significantly longer than that of normal bone marrow (P < 0.0001) and can be used as a marker of early bone metastases. These studies demonstrate that MR imaging can detect bone marrow metastases in small animals prior to development of cortical bone loss identified by X-ray.

Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report.

Medulloblastoma is a malignant embryonal tumor that arises in the cerebellum and invades the fourth ventricle, often resulting in obstructive hydrocephalus. Patients typically present with symptoms related to increased intracranial pressure and cerebellar dysfunction. The authors report a rare case of classic medulloblastoma with central precocious puberty (CPP) as its only presenting symptom. A 7-year-old boy with no prior history of medulloblastoma presented with Tanner Stage IV testicular enlargement and a 4-month history of acne and pubic hair. Laboratory tests of blood samples demonstrated highly elevated luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone. Admission MRI of the brain revealed a mass in the posterior fossa, which bordered and compressed the fourth ventricle. The patient also exhibited mild lateral and third ventriculomegaly. Surgical options were discussed with the neurosurgical department. A suboccipital craniotomy and C-1 laminectomy were performed. A large mass was seen arising from the inferior surface of the vermis, and lying within the fourth ventricle. Gross-total microsurgical resection of the mass was performed. Histopathological investigation characterized the tumor as classic medulloblastoma. Follow-up laboratory tests of blood samples demonstrated a reduction of LH, FSH, and testosterone back to prepubertal levels. The patient then began radiation and chemotherapy. This report demonstrates that mild obstructive hydrocephalus due to a posterior fossa tumor may present with unexpected symptoms, such as CPP. To the authors' knowledge, precocious puberty has not yet been associated with medulloblastoma, although it has been found with other posterior fossa tumors. Extensive imaging of the CNS for patients presenting with CPP is recommended.

Evaluation of Physician and Nurse Dyad Training Procedures to Deliver a Palliative and End-of-Life Communication Intervention to Parents of Children with a Brain Tumor.

When a child's prognosis is poor, physicians and nurses (MDs/RNs) often struggle with initiating discussions about palliative and end-of-life care (PC/EOL) early in the course of illness trajectory. We describe evaluation of training procedures used to prepare MD/RN dyads to deliver an intervention entitled: Communication Plan: Early Through End of Life (COMPLETE) intervention. Our training was delivered to 5 pediatric neuro-oncologists and 8 pediatric nurses by a team of expert consultants (i.e., in medical ethics, communication, and PC/EOL) and parent advisors. Although half of the group received training in a 1-day program and half in a 2-day program, content for all participants included 4 modules: family assessment, goal-directed treatment planning, anticipatory guidance, and staff communication and follow-up. Evaluations included dichotomous ratings and qualitative comments on content, reflection, and skills practice for each module. Positive aspects of our training included parent advisers' insights, emphasis on hope and non-abandonment messages, written materials to facilitate PC/EOL communication, and an MD/RN dyad approach. Lessons learned and challenges related to our training procedures will be described. Overall, the MDs and RNs reported that our PC/EOL communication-training procedures were helpful and useful. Future investigators should carefully plan training procedures for PC/EOL communication interventions.

Rapidly progressive primary leptomeningeal atypical teratoid/rhabdoid tumor: a report of 2 cases.

Atypical teratoid/rhabdoid tumor is a rare, highly malignant central nervous system tumor most commonly occurring in very young children. Atypical teratoid/rhabdoid tumor most often presents as an expanding mass with symptoms consistent with the location of the tumor and may present with metastatic leptomeningeal disease. The authors describe 2 cases of rapidly progressive, diffuse leptomeningeal atypical teratoid/rhabdoid tumor without a solid primary mass. These cases demonstrate a clinical picture that can easily be confused with a basilar meningitis, encephalomyelitis, or vasculitis.