Supratentorial and cerebellar liponeurocytomas: report of four cases with review of literature.

Liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification in 2000, six cases with similar radiological, histomorphological and immunohistochemical features have also been described in the lateral ventricles. In the present study, we report clinical, radiological and pathological findings of three supratentorial and one cerebellar liponeurocytoma from our records, evaluated with an extensive panel of immunohistochemistry, and review published cases in the literature. The immunohistochemical pattern of supratentorial and infratentorial liponeurocytomas are almost identical, which indicates that these tumors are homologous.

Management outcome of the transcallosal, transforaminal approach to colloid cysts of the anterior third ventricle: an analysis of 78 cases.

BACKGROUND: Colloid cysts are not common brain lesions and account for 0.2-2.0% of all brain lesions. Transcallosal, transforaminal approach is a safe route and the most direct path to excise third ventricular colloid cyst, without dependence on hydrocephalus. AIM: To assess the surgical outcome of patients with colloid cysts of the anterior third ventricle treated by the transcallosal, transforaminal approach. PATIENTS AND METHODS: Seventy-eight patients operated by the above approach over a period of 20 years were analyzed. A pre- and postoperative neurological assessment was done in all the patients. Neuro-cognitive evaluation of corpus callosum function was done in the last 20 patients. Computer tomography scan of the brain was done in all patients pre- and postoperatively. RESULTS: Clinical features of raised intracranial pressure without localizing signs were the commonest presenting feature in 52 (66.7%) patients. Hydrocephalus was present in 65 (83.3%) patients. All patients underwent the transcallosal, transforaminal approach, and total excision of the lesion was achieved in 77 patients and subtotal in 1. Four patients required a postoperative shunt for acute hydrocephalus. There was no incidence of postoperative disconnection syndrome. In two patients, there was recurrence of the lesion after 2 and 6 years, respectively. Two patients died in the postoperative period. CONCLUSION: Colloid cyst is surgically curable. Early detection and total excision of the lesion can be a permanent cure with low mortality and minimum morbidity, when compared to the natural history of the disease. The limited anterior callosotomy does not result in disconnection syndromes or behavioral disturbance.

Invasive rhino-cerebral fungal granuloma.

BACKGROUND: Increased incidence of fungal infection has been reported globally in the recent years. Fungal infection of the central nervous system remains one of the most difficult diseases to treat and requires multi-modality intensive therapeutic strategies. MATERIALS AND METHODS: Retrospective analysis of case records of patients with confirmed skull base fungal granuloma treated at a tertiary hospital between 1988-2008. An attempt was made to stage the extent of skull base fungal granuloma based on neuroimaging, operative findings and course of the disease on serial follow-up. RESULTS: Thirty-three patients with skull base fungal granuloma were treated surgically during the study period. The mean age at presentation was 33.2 years and diabetes was a major predisposing factor. Eight patients expired in the first two months following surgical intervention due to flare-up of the disease. Eighteen patients who underwent grossly total excision had a mean progression-free survival (PFS) of 43 months and seven patients with subtotal excision had a mean PFS of 23 months. Better survival probability was noted in those patients who underwent total excision at surgery and received complete course of amphotericin. CONCLUSION: Total surgical excision with complete course of antimycotic drug therapy increases PFS. A better antimycotic drug with less toxicity and high efficacy with fungicidal property can make a difference in the outcomes of the disease.

Exophytic choroid plexus papilloma of the fourth ventricle presenting with cerebrospinal fluid rhinorrhea: a case report.

BACKGROUND: Choroid plexus papillomas are rare benign neoplasms that arise from the ventricular choroid plexus and are considered to be of ependymal origin. They are slow-growing lesions, are commonly associated with hydrocephalus, and are prone to hemorrhage spontaneously. Most of them are located in the posterior fossa in adults, the fourth ventricle being the most common location. CASE DESCRIPTION: We report a case of a 61-year-old male patient with a fourth ventricular exophytic choroid plexus papilloma extending caudally into the foramen magnum causing obstructive hydrocephalus. In February 2005, he presented with spontaneous CSF rhinorrhea to an ENT surgeon and underwent an endoscopic transnasal repair. Six months later, he came to us with progressive loss of vision due to raised intracranial pressure. CONCLUSION: The lesion may not be detected on CT scans and MRI scan is the imaging modality of choice. Excision of the tumor takes precedence over any attempt to repair the fistula, as many a time, the CSF leak may stop.

Suboccipital segment of the vertebral artery: a cadaveric study.

OBJECTIVE: To study the course, relationships, branches and possible anomalies of the vertebral artery in the suboccipital region in adult Indian cadavers. MATERIALS AND METHODS: Twenty-one suboccipital segment vertebral artery specimens from embalmed, Indian adult cadavers were dissected and studied. Dissection was performed using microsurgical instruments and was carried out from the skin up to the vertebral artery in layers. The course, relationships and the branches of the vertebral artery were studied and measurements were taken using Vernier calipers. The readings obtained were corroborated with the measurements derived from the digital images using a computer. OBSERVATIONS: All the vertebral arteries had a tortuous course and were covered with rich venous plexuses. None of the specimens had an anomalous course. The artery was divided into a vertical segment (Vv) between C2 and C1 vertebra and a horizontal segment (Vh) from the C1 transverse foramina to its dural entry. The mean diameter of the artery was 4.8 mm. The shortest distance of Vv segment from the dural tube was 16.1 mm, and the distance from the C2 ganglion was 7.2 mm. The average length of the Vv segment was 15 mm and the average length of the Vh segment was 35.6 mm. The average of the shortest distance between the vertebral artery and the midline was 13.4 mm. CONCLUSION: The vertebral artery has a tortuous course and is prone to accidental iatrogenic injury, which can result in devastating neurological sequelae depending on contralateral vertebral artery flow. A thorough anatomical knowledge of this segment is essential for the surgeon who intends to operate in this area.

Concomitant cervical and lumbar intradural intramedullary lipoma.

BACKGROUND: Lipomas of the spinal cord are often a component of spinal dysraphic states. Nondysraphic intramedullary spinal cord lipomas are rare, and concomitant isolated cervical and lumbar intradural intramedullary lipomata are very rare. One patient with concomitant isolated nondysraphic cervical and lumbar spinal cord lipomata has been reported and management options discussed. CASE DESCRIPTION: A young girl presented with insidious-onset diffuse neck pain and early myelopathic signs. Conventional radiographs were normal. Magnetic resonance imaging of the whole neuraxis revealed concomitant cervical and lumbar intradural intramedullary lipoma. Subtotal resection of the lesion was performed at both levels, after which the patient improved symptomatically and was ambulant independently. CONCLUSION: Concomitant intradural lipomas at 2 different locations unassociated with a dysraphic state is very rare. Magnetic resonance imaging with fat suppression study is the investigation of choice. Adequate decompression with subtotal removal is the treatment of choice.

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.

Delayed anterior cervical plate dislodgement with pharyngeal wall perforation and oral extrusion of cervical plate screw after 8 years: A very rare complication.

We report a patient with congenital anomaly of cervical spine, who presented with clinical features suggestive of cervical compressive spondylotic myelopathy. He underwent C3 median corpectomy, graft placement, and stabilization from C2 to C4 vertebral bodies. Postoperative period was uneventful and he improved in his symptoms. Eight years later, he presented with a difficulty in swallowing and occasional regurgitation of feeds of 2 months duration and oral extrusion of screw while having food. On oral examination, there was a defect in the posterior pharyngeal wall through which the upper end of plate with intact self-locking screw and socket of missed fixation screw was seen. This was confirmed on X-ray cervical spine. He underwent removal of the plate system and was fed through nasogastric tube and managed with appropriate antibiotics. This case is presented to report a very rare complication of anterior cervical plate fixation in the form of very late-onset dislodgement, migration of anterior cervical plate, and oral extrusion of screw through perforated posterior pharyngeal wall.

Intradural extramedullary cysticercal abscess of spine.

Neurocysticercosis (NCC) is one of the most common parasitic diseases affecting the central nervous system. Typically spinal NCC involvement has a concomitant cranial involvement. Spinal involvement by NCC, either intramedullary or extramedullary is very uncommon. The authors report a case of D12-L1 intradural extramedullary lesion in a 38-year-old female patient who presented with complaints of back pain and weakness of lower limbs. She underwent laminectomy and excision of the lesion. Histopathology revealed extramedullary cysticercal abscess. Post-operatively she was treated with albendazole. She had a successful recovery post-operatively and at 8 months follow up had no neurological deficits. This current case presents a very rare case of cysticercal abscess of dorsolumbar spine, without any evidence of cranial involvement. This report is to reemphasize the importance of including NCC as a differential diagnosis in intradural extramedullary lesion at the conus level in endemic areas like India.

Management of pediatric colloid cysts of anterior third ventricle: A review of five cases.

OBJECT: Colloid cysts are congenital benign lesions, associated with more aggressive clinical and radiological picture in children than in adults. We present our experience in management of five pediatric patients with colloid cyst of the anterior third ventricle. They have an excellent chance of surgical cure, or they can be devastating and even fatal, if not recognized on time and treated. MATERIALS AND METHODS: Five pediatric patients (aged 16 years or less) who were surgically treated for a colloid cyst, between 1987 and 2011 were analyzed. The clinical features included raised intracranial pressure in all five cases, of which one patient was brought unconscious with decerebrate posturing. Computed Tomography (CT) scan of the brain was done in all patients. The density of the lesion, enhancement with contrast and the presence of hydrocephalus were analyzed. Four patients underwent a detailed postoperative neurological assessment. RESULTS: Three patients underwent the transcallosal-transforaminal approach and total excision of the lesion. One patient underwent revision of the pre-existing ventriculoperitoneal shunt. One patient who was brought in an unconscious state, an external ventricular drain was inserted and she was ventilated. She died 4 hours after the admission. On follow-up, none of the three patients who underwent the transcallosal-transforaminal approach had disconnection syndromes or behavioral disturbances. CONCLUSION: Colloid cysts in children are rarer and more aggressive than their adult counterparts. It is surgically curable. Early detection and total excision of the lesion is a permanent cure with minimum morbidity, when compared with the natural history of the disease. The limited anterior callosotomy does not result in disconnection syndromes or behavioral disturbance.

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.

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 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.

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.

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.

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 epithelioid hemangioendothelioma.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is an uncommon soft-tissue vascular neoplasm. Although a well-defined entity outside the neuraxis, its intracranial occurrence is rare. Literature review revealed 35 reported cases till date. CASE REPORT: The authors report a case of intracranial EHE in a 15-year-old girl, who presented with a short history and unusual radiology. Following radical removal, the lesion recurred within 1 month and progressed to terminal stages. This tumor is thought to have an indolent intermediate malignancy potential and such rapid progression has not been reported so far.