Intracranial hemangioblastomas: an institutional experience.

BACKGROUND AND AIMS: We present our Institutional experience with intracranial hemangioblastomas. SETTINGS AND DESIGN: A retrospective study. MATERIALS AND METHODS: This study included all patients of intracranial hemangioblastomas admitted in our institution over a period of 11 years from January 1992 through June 2003. RESULTS: There were a total of 69 patients (45 males and 24 females). The average age at presentation was 34.5 years. The tumor was located in the cerebellar hemispheres, vermian and brainstem regions in 42 (60%) patients, 19 (28%) patients and 8 (12%) patients, respectively. Hydrocephalus was seen in 48 (69%) patients. Thirty-three patients underwent CSF diversion procedures prior to surgery on the tumor. All except one underwent definitive surgery. The mortality was 8 (11%). Sixty eight patients underwent surgery on the tumor. The follow-up ranged from 1 month to 11 years. Fifteen patients developed recurrent lesions. CONCLUSION: Lifelong surveillance is necessary in cases with hemangioblastomas to identify recurrences especially in those patients having VHL syndrome.

Outcome of "unknown" head injury patients at a tertiary care neurosurgical centre.

CONTEXT: A large number of patients are admitted to hospitals in large cities without any identification. These "unknown" patients represent a unique problem in developing countries. There is no systematic study in world literature on this subgroup of patients. AIMS: To elucidate the natural history and outcome of traumatic brain injury patients admitted in the department of neurosurgery as "unknowns". SETTINGS AND DESIGN: Retrospective analysis of all traumatic brain injury patients admitted to the department of neurosurgery as "unknown", between January 2002 and March 2005. RESULTS: Three hundred and twenty five patients were admitted as unknowns over a 3 year and 3 months period. Most of the patients were young males and became known during their hospital stay. Overall, 33 patients stayed for longer than one month, with 4 of them staying for longer than 6 months. Mortality in mild, moderate and severe head injury group was 1%, 6% and 46% respectively. CONCLUSIONS: Unknown patients represent a unique subgroup in metropolitan cities like Delhi. Managing them raises several medico-legal issues. Many of them occupy hospital beds for longer duration than required. There is an acute shortage of rehabilitation homes in Delhi for long term care and rehabilitation of such patients.

Sellar Tuberculoma: An unusual infection.

Tuberculomas are common intracranial lesions in our country. However, tubercular infection of pituitary gland is very unusual. We present a case of intrasellar tuberculoma mimicking pituitary adenoma, and suggest the radiological features and management. A 22-year-old woman had presented with generalized dull aching mild to moderate headache for 8 months and decreased vision in both eyes for 6 months. Visual acuity was 6/9 in both eyes but visual fields and fundus examination were normal. There were no other significant findings. CT scan and MRI (brain) showed a sellar- suprasellar lesion. The pituitary stalk was thickened, as was the mucosa of sphenoid sinus, which raised the suspicion of an infectious pathology other than pituitary macroadenoma. Surgery was performed through sublabial transsphenoid route. A firm, rubbery, yellowish, non-suckable and relatively avascular lesion was found in the sella. Only subtotal decompression was done. Frozen section biopsy was suggestive of inflammatory pathology. Histopathology revealed features compatible with tuberculosis. We suggest that tuberculosis should be considered in the differential diagnosis of sellar lesions, especially if associated with contrast enhancement and thickening of sphenoid sinus mucosa or pituitary stalk, particularly in patients from tuberculosis endemic areas. Most of these patients are negative for workup for systemic tuberculosis. Intraoperatively, a frozen section should be sent and if it shows inflammatory pathology, only a decompression for biopsy should be done. We do not advise radical decompression of these lesions as anti-tubercular treatment is sufficient for cure.

Intraventricular rhabdoid tumor.

Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication.

p53 protein alterations in adult astrocytic tumors and oligodendrogliomas.

BACKGROUND: p53 is a tumor suppressor gene implicated in the genesis of a variety of malignancies including brain tumors. Overexpression of the p53 protein is often used as a surrogate indicator of alterations in the p53 gene. AIMS: In this study, data is presented on p53 protein expression in adult cases (>15 years of age) of astrocytic (n=152) and oligodendroglial (n=28) tumors of all grades. Of the astrocytic tumors, 86% were supratentorial in location while remaining 14% were located infratentorially - 8 in the the cerebellum and 13 in the brainstem. All the oligodendrogliomas were supratentorial. MATERIALS AND METHODS: p53 protein expression was evaluated on formalin-fixed paraffin-embedded sections using streptavidin biotin immunoperoxidase technique after high temperature antigen retrieval. RESULTS: Overall 52% of supratentorial astrocytic tumors showed p53 immunopositivity with no correlation to the histological grade. Thus, 58.8% of diffuse astrocytomas (WHO Grade II), 53.8% of anaplastic astrocytomas (WHO Grade III) and 50% of glioblastomas (WHO Grade IV) were p53 protein positive. In contrast, all the infratentorial tumors were p53 negative except for one brainstem glioblastoma. Similarly, pilocytic astrocytomas were uniformly p53 negative irrespective of the location. Among oligodendroglial tumors, the overall frequency of p53 immunopositivity was lower (only 28%), though a trend of positive correlation with the tumor grade was noted - 25% in Grade II and 31.5% in grade III (anaplastic oligodendroglioma). Interestingly, p53 labeling index (p53 LI) did not correlate with the histopathological grade in both astrocytic and oligodendroglial tumors. CONCLUSIONS: Thus, this study gives an insight into the genetic and hence biological heterogeneity of gliomas, not only between astrocytic tumors vs. oligodendrogliomas but also within astrocytic tumors with regard to their grade and location. With p53 gene therapy trials in progress, this will possibly have future therapeutic implications.