Primary spinal atypical teratoid/rhabdoid tumour presenting with hematomyelia and subarachnoid haemorrhage-a case report.

Atypical teratoid/rhabdoid tumours (AT/RTs) are highly aggressive and uncommon malignant tumours of the central nervous system (CNS) affecting children younger than 3 years of age. Primary spinal cord involvement is an extremely rare presentation. AT/RTs show necrosis and haemorrhages on histopathology frequently. However, spinal atypical teratoid/rhabdoid tumour (AT/RT) with hematomyelia and spinal subarachnoid haemorrhage (SAH), as seen in our case, has never been reported in the literature in the paediatric age group. We report a case of primary spinal AT/RT in a 3-year-old male child presenting acutely with hematomyelia and spinal SAH and try to elucidate its pathophysiological basis.

Management Dilemma in A Rare Case of Bilateral Temporo-Insular Glioma.

Multicentric gliomas are uncommon pathological entities, although well described in the literature. The aim of this study was to highlight the management issues in a rare case of multicentric glioma occurring in a bilateral temporo-insular region. A 35-year-old farmer with no comorbid illness presented with a history of progressively increasing holocranial headaches, intermittent episodes of vomiting, and memory disturbances from the last 3 months. Radiological findings were suggestive of multicentric glioma involving bilateral temporo-insular regions. Patient underwent tumor decompression first on the left side followed by the right side. Postoperative course was uneventful. Management of multicentric gliomas is challenging.

Diencephalic Syndrome as Presentation of Giant Childhood Craniopharyngioma: Management Review.

Diencephalic syndrome (DES) is an extremely uncommon occurrence, and approximately 100 cases have been reported. It presents as a failure to thrive in infants and children but rarely occurs in adult population. The characteristic clinical features of DES include severely emaciated body, normal linear growth and normal or precocious intellectual development, hyperalertness, hyperkinesis, and euphoria usually associated with intracranial sellar-suprasellar mass lesion, usually optico-chiasmatic glioma or hypothalamic mass. DES as a presentation of craniopharyngioma is extremely uncommon but can also occur with brain stem mass. Detailed PubMed and MEDLINE search for craniopharyngioma associated with DES yielded only six cases in children below 6 years of age. Thus, we reviewed a total of seven cases including previously published six cases and added additional our own case. Overall, the mean age at diagnosis was 4.15 years with male:female ratio of 4:3, the mean time interval between symptom of DES appearance and final diagnosis was 6.6 months. The most commonly observed symptom of DES was weight loss (85%). The clinical feature, imaging, and management of such rare syndrome along with pertinent literature are briefly reviewed.