Management trends and outcomes of pineal germinoma in a multi-institutional Australian cohort.

Pineal germinoma is rare with high cure rates following craniospinal radiotherapy. Efforts to reduce the radiotherapy dose and field via combination with chemotherapy suggest comparable disease control and reduced neurocognitive impairments, while the efficacy of immunotherapy in pineal germinoma remains undetermined. This report aimed to review clinical outcomes in patients treated for pineal germinoma in Queensland, Australia, and assess for Programmed Death-Ligand1 (PD-L1) expression. Patients who commenced radiation and/or chemotherapy for pineal germinoma from 2005 to 2017 were retrospectively identified using Queensland Oncology Online database. Demographic, diagnostic, treatment, and outcome data was obtained from electronic medical records. PD-L1 immuno-histochemistry was performed on available specimens. Eighteen patients with long-term follow-up data were identified. Median age at diagnosis was 16.8 years (range 9-46 years). Diagnosis was made histologically in fifteen patients, and radiologically in three. All patients underwent radiotherapy (median 36 Gy (range 21-54 Gy)) with lower median dose delivered with whole ventricle irradiation (12/18patients) than craniospinal irradiation (5/18patients). Sixteen patients received chemotherapy preceding radiotherapy. All patients are alive at median 7.25 years from primary treatment completion (range 2.03-13.1 years). Relapse occurred in three patients (16.67%) following treatment response, all of whom achieved remission following high-dose chemotherapy with stem-cell support and craniospinal radiotherapy. Post-treatment functional outcomes were similarly excellent. PD-L1 expression was low (1-49% cells) or negative in 87% of tumours tested but results were confounded by specimen quality and availability. Reduced-dose radiotherapy with chemotherapy does not compromise outcome and is standard of care at this institution. Immunotherapy is unlikely to become standard treatment in the near future.

Multifocal Gorham-Stout disease associated with Chiari I malformation and recurrent aseptic meningitis: Case report and review of literature.

Gorham-Stout disease is a rare condition of uncertain aetiology characterised by lymphatic proliferation within osseous structures and subsequent massive osteolysis. This report describes the index case of a patient with multifocal Gorham-Stout disease involving the skull base with Chiari I malformation and recurrent aseptic meningitis without fistula. A five-year-old male presented following decompression of a Chiari I malformation with headaches, vomiting, and stiff neck and cerebrospinal fluid pleocytosis without growth of a pathogenic organism. Ongoing symptoms prompted a further three presentations over several months revealing persistent aseptic cerebrospinal fluid monocytic pleocytosis. Further investigation revealed multifocal osseous cystic disease and subsequent bone biopsy suggested Gorham-Stout disease. Suboccipital decompression was not repeated despite craniocervical junction re-stenosis. A literature review demonstrated the extreme rarity of Gorham-Stout disease associated with Chiari I malformation and meningitis. Potential mechanisms of these entities occurring in concert are discussed. Consideration of Gorham-Stout disease as a secondary cause for Chiari I malformation is important amid local bone changes or cerebrospinal fluid leakage prior to pursuing suboccipital decompression considering the poor outcomes reported.

Traumatic brain injuries in a paediatric neurosurgical unit: A Queensland experience.

Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children with a broad injury spectrum and associated continuum in the level of care required. A dearth of data exists regarding children requiring inpatient neurosurgical admission following TBI. A retrospective study of children 0-16 years-old admitted to the neurosurgical unit of a level-1 paediatric trauma hospital in Queensland, Australia following TBI was conducted focusing on the demographics, clinical characteristics, and management of these patients to guide those involved in their management, and identify areas for improvement in injury prevention and trauma system management. Over 48 months, 671 patients were identified (62.6% male) with median age 5.0 years, the majority transferred from peripheral centres. Falls (47.2%) and traffic accidents (21.5%) were the most common mechanisms. Non-displaced skull fracture was the most common injury. Moderate or severe TBI (GCS 3-12) was seen in 14.8% of whom were more likely to require surgery, intensive care, or suffer polytrauma. Clinically significant TBI, defined as moderate/severe TBI, polytrauma, death, requiring neurosurgery, intensive care admission, intubation, or admission three or more nights was detected in 57.97% with higher rates in transferred patients (62.9%) versus primary presentations (50.6%). Mechanisms involving low kinetic forces especially low-height falls and children with non-surgical pathology were less likely to meet criteria for clinically significant TBI. Opportunity exists to optimise triage and transfer practices within the trauma network to minimise the economic and social implications of over-triage with many children requiring only brief observation.

Diffuse midline glioma metastasis to the peritoneal cavity via ventriculo-peritoneal shunt: Case report and review of literature.

Extraneural dissemination of primary intracranial tumours to the peritoneal cavity via ventriculoperitoneal shunts is rare, with medulloblastoma and germ-cell tumours most common and gliomas seldom implicated. This report is the first described case of a diffuse midline glioma H3 K27M-mutant disseminating to the peritoneal cavity via a shunt. A four-year-old female presented with a large solid-cystic lesion centred on the suprasellar cistern, histologically revealed to be diffuse midline glioma H3 K27M-mutant. The patient received multiple courses of radiotherapy to the primary lesion and metachronous spinal metastases, and underwent bilateral ventriculoperitoneal shunts. She presented fourteen months following diagnosis with acute hydrocephalus and massive ascites revealed to be due to histologically confirmed intra-abdominal glioma metastasis secondary to shunting. Bilateral ventriculoatrial shunts along with targeted abdominal radiotherapy and repeated ascitic drainage were performed. The patient died one month later. A literature review demonstrated that intra-abdominal glioma metastasis is an extremely rare complication of cerebrospinal fluid diversion predominantly affecting paediatric patients with high-grade lesions within the first year after diagnosis and portends poor prognosis. Predisposition to metastasis is likely associated with tumour proximity to cerebrospinal fluid spaces and tumour biology. Contraindicating shunting in the presence of an intracranial tumour cannot be endorsed but rather shunt-related metastasis should be an acknowledged risk, and not-to-be-forgotten presentation.