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Object This study is a retrospective analysis of 60 surgically treated patients with 64 peripheral nerve sheath tumors (PNSTs) at the second cervical (C-2) nerve root. The anatomical subtleties of these tumors and their implications for surgical strategy when compared with other spinal PNSTs and other tumors in the foramen magnum region are reviewed. Methods Sixty patients with C-2 PNSTs treated surgically in the Department of Neurosurgery at King Edward VII Memorial Hospital and Seth Gordhandas Sunderdas Medical College between 1992 and 2006 were studied. All patients underwent magnetic resonance imaging. Tumors were divided into 3 groups depending on their anatomical location identified during surgery. Those tumors located within or extending into the spinal dural tube were called Type A, those located within the dural tube of the C-2 ganglion were labeled as Type B, and tumors extending laterally into the paraspinal region were labeled as Type C. Follow-up durations ranged from 6 months to 15 years (mean 64 months). Results There were 38 male and 22 female patients in the study, who ranged in age from 6 to 62 years (mean 28 years). Nine patients had clinical features indicative of neurofibromatosis (NF). The mean duration of symptoms at the time of presentation was 27 months (range 4 days–5 years). Two patients had no specific symptoms related to the C-2 PNST, 6 patients had only local symptoms such as neck pain or stiffness, and 52 patients had symptoms of varying degrees of myelopathy. There were 5 solely Type A tumors, 7 Type A + B tumors, 31 Type B tumors, and 21 Type B + C tumors. All Type A, A + B, and B tumors were totally resected. Seven of 21 Type B + C tumors were partially resected, and the remainder were completely resected. All patients postoperatively reported varying improvement in their preoperative symptoms. Except for patients with NF who were disabled by other tumors, the rest of the patients resumed their normal life style. There have been no cases of symptomatic tumor recurrence. Conclusions The majority of PNSTs located at the C-2 level in these patients probably arose from the large C-2 ganglion and are limited within the dural confines or are interdural in location. In contrast to other spinal PNSTs, the location of C-2 PNSTs is in most cases posterior to the lateral mass of the atlas and axis and the atlantoaxial joint and is exposed to the posterior without any bone cover. Radical tumor resection is safe, resolution of clinical symptoms is rapid, and recurrence rates are extremely low. In a selected number of cases, bone work for tumor exposure and resection can be entirely avoided.

Aim:Medulloblastoma is one of the most common posterior fossa tumors in childhood. The treatment-related side effects as well as predictive outcome still remain as a major challenge. The improved understanding of the disease and advances in molecular biology is changing the treatment paradigms from Chang's staging system to molecular risk stratification. However, surgery still remains as an important mainstay of therapy and is formidable. The role of radical surgery has always been a crucial factor in the outcome of these patients, the best survival being reported in patients who had total excision of the tumor and with no metastasis.Patient and Methods:An analysis of 365 patients (age<18 years) of medulloblastoma who underwent treatment at the Seth G.S. Medical College and King Edward VII Memorial hospital (KEM), Mumbai over a 25- year period (1985-2000 and 2001-2010) is presented. The clinical profile, radiological features, pathology and surgical nuances are discussed.Results:The most common age group affected was between 3 and 12 years. 75.3% presented with headaches, vomiting and 63.2% with papilledema. Sitting position was used in majority of cases. A total of 8 patients underwent shunting; all of them were in the postoperative period (5.19%). 92.2% (142 cases) had classical medulloblastoma, 5.1% (8 cases) had desmoplastic variant, 1.9% (3 cases) had anaplastic changes and 0.6% (1 case) had glial differentiation. The 5-year and 10-year progression free survival rate was 73 and 41% for average risk disease while for high risk disease rate it was 34%. The mortality rate was 2%. The quality of life was enhanced in patients who survived 5-10 years after treatment.Conclusion:Surgery for medulloblastoma is formidable. The option of sitting position for medulloblastoma surgery is still viable. A vigilant neuroanesthesiologist and a safe surgery are necessary to achieve a good postoperative result. Radiological characteristics are helpful adjuncts for determining effective surgical strategy. Permanent CSF drainage can be avoided in majority of patients and can be definitively considered in progressive symptomatic hydrocephalus. A safe maximal resection and a good Karnofsky score are paramount to ensure compliance with adjuvant therapy and contribute to an overall survival advantage.

The authors report the case of a 35-year-old man who had polyarthritic affliction with rheumatoid disease. He presented with complaints of quadriparesis that had progressed over the course of 2 years. Investigations revealed telltale evidence of rheumatoid disease of the craniovertebral junction with retroodontoid pannus, basilar invagination, and “fixed” atlantoaxial dislocation. The patient underwent lateral mass reconstruction with distraction of the facets and impaction of a spiked metal spacer and bone graft within the joint. Investigations done in the immediate postoperative phase showed complete disappearance of retroodontoid pannus in addition to reduction of basilar invagination and atlantoaxial dislocation. He had remarkable and sustained relief from symptoms. The authors also review the pathogenesis and treatment of retroodontoid pannus.

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