Aneurysmal bone cyst of the lumbar spine.

An aneurysmal bone cyst (ABC) is a benign, locally proliferative vascular disorder of non-neoplastic osseous lesions in children and young adults. Seventy-five percent of ABCs occur before the age of 20 years. They comprise 1.4% of all primary bone tumors, and commonly occur in the long bones. Spinal ABCs are much rarer. We present to you one such rare case of ABC involving the lumbar spine which was successfully treated with surgery. The clinical pathological and radiological features are described. The treatment options available are discussed.

Complication avoidance in transcallosal transforaminal approach to colloid cysts of the anterior third ventriclen: An analysis of 80 cases.

OBJECT: The objective of the present study is to analyze the complications and their avoidance in a series of 80 patients operated by transcallosal transforaminal approach to colloid cysts of the anterior third ventricle. MATERIALS AND METHODS: The surgical outcome and complications of 80 patients operated by transcallosal transforaminal approach for colloid cysts in the anterior third ventricle was analyzed. A detailed pre- and post-operative neurological assessment was done in all patients. Neurocognitive assessment of corpus callosal function was done in the last 22 cases. CT scan of the brain was done in all patients, before and after surgery. RESULTS: All patients underwent transcallosal transforaminal approach. Total excision of the lesion was achieved in 79 patients and subtotal in one. The complications encountered were postoperative seizures in six, acute hydrocephalus in four, venous cortical infarct in four, transient hemiparesis in four, transient memory impairment, especially for immediate recall in nine, mutism in one, subdural hematoma in one, meningitis in three, and tension pneumocephalus in one patient. There were two mortalities. There was no incidence of postoperative disconnection syndrome. CONCLUSION: Colloid cyst is surgically curable. With good knowledge of the regional anatomy and meticulous microsurgical techniques, there is a low mortality and minimum morbidity, when compared to the natural history of the disease. With increasing experience, most of the complications are avoidable. The limited anterior callosotomy does not result in disconnection syndromes.

Isolated skull base primary Ewing's sarcoma: an extremely rare location.

A 16-year-old boy presented with history of headache and blurring of distant vision. He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone. He underwent gross total excision of the lesion. Histopathology and immunohistochemistry were suggestive of skull base Ewing's sarcoma. Bone scan was done to rule out primary involvement of other extracranial location. We have discussed the epidemiology, presentation, management and prognosis of such cases.

Primary intrasellar schwannoma.

Intracranial schwannomas commonly arise from the eighth cranial nerve in the cerebellopontine angle. Schwannoma arising in the sella and extending into the suprasellar region is very rare and is easily mistaken for pituitary adenoma. To our knowledge, there have been only 12 previous reports. We present a patient with primary intrasellar schwannoma that clinically and radiologically resembled a pituitary adenoma (PA). Intra-operative findings differed from a PA, as the tumour had a firmer consistency. Gross total excision of the lesion was done via a transethmosphenoidal approach. Post-operatively the patient improved in visual acuity and visual fields. We have reviewed the literature and described the characteristics of such lesions.

Pediatric multifocal glioblastoma multiforme with fulminant course.

Glioblastoma multiforme (GBM) is the most common primary malignant brain tumor. GBM in children is less common than in adults and has a better prognosis. Pediatric GBM is a rare entity, and a multifocal development in a pediatric GBM is much rarer. We report to you one such rare case of pediatric multifocal GBM in a 5-year-old child who developed rapidly increasing multiple lesions after radiotherapy. More studies are required to study the genetic analysis, tumor behavior, management and outcome of these rare tumors.

Orbitotemporal neurofibromatosis: case report.

Plexiform neurofibromas occur in about 60% of neurofibromatosis type 1(NF-1) patients and can lead to severe morbidity by disfigurement or compression of vital structures. Moreover, these tumors can undergo malignant transformation. Both focal and localized bone abnormalities are part of the phenotypic expression of NF-1. We report a rare case of severe cranioorbital plexiform neurofibromatosis in a young male and discuss the classification, clinical features, and various treatment options of orbit-temporal neurofibromatosis type 1.

Primary spinal intradural extramedullary lymphoma causing cauda equina syndrome.

We report a case of lumbar intradural extramedullary lesion in an 11-year-old boy who presented with cauda equina syndrome and acute bladder disturbance. He underwent emergency surgical resection of the lesion, which was proved to be a lymphoma by histopathology and immunohistochemistry. He has improved neurologically and after 1 year, he is leading a normal life with near normal neurological functions. This is the second case of primary spinal intradural extramedullary lymphoma. This is the first such case in the pediatric age group and causing cauda equina syndrome. We describe the characteristics of such tumors along with pathogenesis and management.

Primary spinal epidural lymphomas.

An epidural location for lymphoma is observed in 0.1-6.5% of all the lymphomas. Primary spinal epidural lymphoma (PSEL) is a subset of lymphomas, where there are no other recognizable sites of lymphomas at the time of diagnosis. The incidence of this subset of lymphomas is much less. It, however, is increasingly diagnosed, due to the increased use of more sensitive imaging modalities. For the electronic search, Pubmed was used to identify journals that enlisted and enumerated PSEL from 1961 to January 2011. The following combination of terms: "primary," "spinal," "epidural," and "lymphoma" were used. The most significant articles and their bibliographies were analyzed by the authors. The symptoms, pathogenesis, diagnostic workup, histopathology, treatment, and outcome have been analyzed in a systematic manner.

Multiple burr hole surgery as a treatment modality for pediatric moyamoya disease.

OBJECTIVE: To re-emphasize that indirect revascularization surgery alone, where multiple burr holes and arachnoid openings are made over both cerebral hemispheres, is beneficial in the treatment of moyamoya disease (MMD) in children. CLINICAL PRESENTATION: We report a 10-year-old boy who presented with complaints of episodic headache for the last 5 years. At the peak of his headache he had visual disturbances and acute onset weakness of left-sided limbs, recovering within a few minutes. He had no focal neurological deficits. Radiological investigations revealed abnormal findings, demonstrating the features of MMD. SURGICAL MANAGEMENT: He underwent bilateral multiple burr holes, dural and arachnoid opening over the frontal, parietal and temporal regions of each hemisphere. The elevated periosteal flap was placed in contact with the exposed brain through each burr hole. RESULTS: On 6-months follow-up he had only one episode of transient ischemic attack. Postoperative four vessel angiogram demonstrated excellent cerebral revascularization around the burr hole sites, and single photon emission computerized tomography imaging showed hypoperfusion in the right temporo-occipital area suggestive of an old infarct with no other perfusion defect in the rest of the brain parenchyma. CONCLUSION: In children with MMD this relatively simple surgical technique is effective and safe, and can be used as the only treatment without supplementary revascularization procedures. This procedure can be done in a single stage on both sides and the number of burr holes made over each hemisphere depends on the extent of the disease.

Intracranial Rosai Dorfman Disease: report of three cases and literature review.

BACKGROUND: Rosai-Dorfman Disease (RDD) is a rare idiopathic non-neoplastic histioproliferative disease characterized clinically by massive painless cervical lymphadenopathy, fever and weight loss. Extranodal involvement has also been recognized. Central nervous system (CNS) manifestations are extremely rare and patients with intracranial involvement usually present with clinical and radiological findings suggestive of a meningioma. CASE DESCRIPTION: We report our experience in the management of three patients with RDD. Two patients had dural based lesions, radiologically in favour of a meningioma, and one patient had a parenchymal lesion suggestive of a tuberculous granuloma. Treatment consisted of total excision in one case, and subtotal excision followed by conventional radiotherapy in two cases. The diagnosis was confirmed by histopathology and immunochemistry which is essential for a definite diagnosis of RDD.