A 45-year-old gentleman presented with headache, dizziness, and unsteadiness of gait. On imaging, he was found to have a cerebellar and a spinal intramedullary hemangioblastoma. To rule out Von Hippel–Lindau (VHL) disease, we did screening of the whole abdomen with ultrasonography followed by contrast-enhanced computed tomography of the abdomen, and surprisingly, the patient was having bilateral adrenal pheochromocytoma and a hepatic cyst. A diagnosis of VHL disease was made on this basis. The patient was operated and he made a very good recovery. We are reporting this interesting case because of its typical findings and its rarity. Disease management and review of literature were also discussed at the end.

Diprosopus is an extremely rare form of craniofacial malformation seen in newborns where there is duplication of face which may be partial or complete. The baby usually has a single trunk and normal limbs. We report a case of diprosopus admitted in our institute and analyze the anomalies and clinical problems and outcome of the case.

Background: Hydrocephalus is a relatively common occurrence in a children suffering from the posterior fossa tumour (PFTm). However, there is a divided opinion regarding the ventriculoperitoneal shunt (VPS) surgery before the posterior fossa tumour resection in a child. For the better clinical outcome, we should be able to predict which patient will require VPS following the resection of PFTm. Purpose of our retrospective analysis is to analyse various factors that predicts the necessity of VPS following PFTm resection.Methods: A consecutive series of twenty-six patients who underwent PFTm resection without undergoing VPS preoperatively are analysed in our series.Results: In our series, we found that the younger age at presentation, incomplete tumour resection, longer period of artificial ventilatory support, insertion of external ventricular drain (EVD) and its duration during the postoperative period correlate the necessity of VPS following PFTm resection. However, the severity of hydrocephalus prior to tumor surgery, tumour size, anatomical location of the tumour, tumour dissemination, use of Dural grafts during closure and histopathological type do not predict the requirement of the VPS following PFTm.Conclusions: Patients who are younger at diagnosis should be treated with utmost importance. Gross total resection should be the goal. Factors which predict the likelihood of the EVD which parallels the likelihood of postresection hydrocephalus must be prevented for the better clinical outcome.

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant syndrome manifested by a spectrum of tumours in the central nervous system (CNS) and other visceral organs. We herein report a case of 35 years aged newly diagnosed diabetic female patient presented with headache, gait instability, loss of vision in both eyes, left sided hearing impairment and subsequently diagnosed to have VHL disease. The pathophysiology involves the inactivation of the VHL tumour suppressor gene. Early recognition and treatment remains the mainstay of management. Even many years after the complete tumour excision, newer neoplasms may develop. Increasing knowledge about the molecular enabled us to investigate the role of anti-angiogenic drugs. Continuous surveillance at regular interval must be conducted in patients with VHL disease.

Introduction Medulloblastoma is a common embryonal childhood tumor predominantly seen in children (1-3). Medulloblastoma can be classified in different histological and molecular subgroups, each of them characterized by discrete clinical presentation, demographic features, prognosis, and outcome. This may help in patient stratification, treatment strategy, design, and implementation of the targeted therapy. Aims and Objectives We aimed to study the patients included in the study with regard to variables such as age, sex, clinical features, and various modes of management in our hospital. We also studied histological and molecular variations in the group. Materials and Methods A prospective study was carried out in the Department of Neurosurgery, Gauhati Medical College and Hospital, Guwahati, from January 2016 to August 2017. Sixty-five patients diagnosed as medulloblastoma were included in the study. We studied the spectrum of clinical findings, radiological and pathologic characteristics, modes of surgical management, and postoperative complications. Study of molecular biology was done in the Department of Bioengineering, Gauhati University. Results and Observation Results and observations were obtained based on the age and radiological, pathological, histological, and molecular variants. Conclusion Medulloblastoma is seen more in pediatric age group and males. Classical variant was the most common type with mostly vermian location followed by desmoplastic variant which was paravermian to lateral in location. Postoperatively, radiotherapy and chemotherapy have proved very useful. Statistical analysis suggested a significant correlation between age groups and complications and also between histological types and associated complications. P53 mutation was noted in a section of the study population but majority cases did not show mutations in selected genes.