Spinal intradural extramedullary granular cell tumor.

Background: Granular cell tumors (GCTs) are uncommon peripheral nerve sheath tumors of Schwann cell origin that may occur throughout the body. However, they rarely occur in the spinal canal. Case Description: A 49-year-old male presented with burning sensation in the left knee. The MRI of the lumbar spine showed an L3-L4 intradural extramedullary tumor. Complete surgical resection was successfully performed and the L3 root burning improved. Histopathologically, the lesion proved to be a benign GCT. Conclusion: Spinal GCTs are rare benign tumors that may be found in an intradural extramedullary location in the spine. The preferred treatment is complete surgical resection as subtotal/partial resection may result in recurrence warranting radiation therapy.

Trigeminal neuralgia secondary to onyx embolization of a right occipital arteriovenous malformation.

BACKGROUND: Trigeminal neuralgia is a debilitating chronic condition characterized by severe recurrent hemifacial pain which is often caused by compression of the trigeminal nerve by an adjacent vessel loop. Microvascular decompression (MVD) surgery is an effective procedure that can lead to full symptomatic relief. Intracranial arteriovenous malformations (AVMs) are primarily congenital abnormalities that may be asymptomatic or manifest as seizures or focal neurologic deficits. They may cause intracranial bleeding and hence are promptly treated, often by endovascular embolization. This procedure is safe but may have a multitude of unpredictable complications. CASE DESCRIPTION: A 33-year-old female presented with medically refractory trigeminal neuralgia secondary to Onyx embolization of a right occipital AVM 3 years prior. She underwent surgical exploration and MVD of the trigeminal nerve root which was found to be compressed by the previously embolized superior cerebellar artery. The procedure was successful and full symptomatic resolution was immediately achieved. CONCLUSION: Postprocedural trigeminal neuralgia is a procedural complication of Onyx endovascular embolization. It may be treated by MVD surgery regardless of the presence or absence of a compressive vascular loop on imaging.

PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report.

Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and PTEN gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR.

Craniocervical Rosai-Dorfman Disease Involving the Vertebral Artery: Case Report and Literature Review.

BACKGROUND: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-Langerhans cell reactive histiocytic disorder that rarely occurs in the central nervous system (CNS). Extranodal RDD most frequently involves the skin, upper respiratory tract, soft tissue, gastrointestinal tracts, bones, breast, and CNS. CASE DESCRIPTION: RDD of the CNS infiltrates most commonly the dura of the sella, cavernous sinus, and the periclival regions. It is usually clinically and radiologically mistaken for meningioma because of its focal dural-based aspect. RDD is confirmed histologically by lymphoplasmacytic cells and histiocytes of varying size showing emperipolesis (lymphocytophagocytosis). To date, only 4 cases of RDD displaying spinal cord compression secondary to craniocervical junction involvement have been reported. CONCLUSION: We report the case of a patient diagnosed with RDD localized at the foramen magnum extending to the base of the odontoid process and involving the V4 segment vertebral artery.

A Surgical Case of Complete Resection of the Focal Cortical and Subcortical Dysplasia in the Motor Cortex.

BACKGROUND: Focal cortical dysplasias (FCDs) are highly epileptogenic and frequently associated with medically refractory focal epilepsy. FCDs are frequently located in the frontal lobe, making its complete resection highly challenging when in proximity to the motor cortex. CASE DESCRIPTION: We report a case of a 25-year-old woman with medically refractory epilepsy secondary to a focal cortical dysplasia in the motor cortex and extending deeply into the subcortical white matter. A detailed presurgical evaluation and invasive electroencephalographic monitoring performed at our epilepsy monitoring unit, along with the use of motor mapping, functional magnetic resonance imaging, diffusion tensor imaging, and the Stealth navigation system resulted in the complete resection of the lesion without a permanent postoperative motor deficit. The patient remained seizure-free at a 63-month follow-up while being maintained on a single antiepileptic drug. CONCLUSION: A detailed presurgical evaluation, accurate mapping of the functional and dysplastic cortex, and a well-planned tailored and complete surgical resection of the cortical dysplasia can result in a favorable outcome with relatively little risk of postoperative neurologic deficit.

Management of Pituitary Adenomas: Mononostril Endoscopic Transsphenoidal Surgery.

INTRODUCTION: The endoscopic transsphenoidal approach for pituitary adenomas and other sellar lesions is quickly becoming the procedure of choice in their surgical management. The most common approach is binostril three-hand technique which requires a large exposure and subjects both nasal cavities to potential trauma. To reduce nasal morbidity, we employ a mononostril two-hand technique with the help of the endoscope holder. In this research, we review our series to determine efficacy of this approach in the management of pituitary adenomas. METHODS: We performed a retrospective analysis of our initial series of 64 consecutive patients with pituitary adenomas operated by the same surgical team from 2008 till 2014 using a mononostril endoscopic approach. After categorizing the lesions into microadenomas, noninvasive macroadenomas, and invasive macroadenomas, we reviewed the radiological and biochemical outcomes of the surgeries after 3 months, 12 months, and 18 months. We also assessed recurrences and complications. Extent of resection was divided into gross total resection, near total resection (>90% resection), and partial resection for the remaining. RESULTS: Our results show resection rates comparable to most series in the literature, with a gross total resection of 87% in non-invasive macroadenomas, and surgical disease control in 75% of invasive nonfunctioning adenomas. The remission rate in Cushing's disease was 81%, where it achieved up to 58% surgical remission in growth hormone secreting pituitary adenomas (including the invasive adenomas). The complication rate was very low. CONCLUSION: We conclude that the mononostril endoscopic approach is well suited for most pituitary tumor operations and carries comparable remission and resection rates to most endoscopic series with minimal complications and nasal morbidity.

A Small Glossopharyngeal Schwannoma Presenting with Intractable Vomiting: Case Presentation and Literature Review.

BACKGROUND: Glossopharyngeal schwannomas are rare tumors. Clinical and radiologic presentation closely resembles those of vestibular schwannomas. Their clinical presentation varies from vestibulocochlear dysfunction to glossopharyngeal and vagal nerve dysfunction. CASE DESCRIPTION: We report a case of a small glossopharyngeal schwannoma presenting with intractable recurrent sudden episodes of vomiting that subsided after tumor resection. To our knowledge, this is the only case in the literature of glossopharyngeal schwannoma presenting with nerve irritation in the form of intractable emesis. CONCLUSIONS: Small glossopharyngeal schwannomas can present with irritative symptoms of the ninth cranial nerve causing vomiting refractory to medical treatment. Intracranial imaging should be considered in the investigation of unexplained intractable vomiting even if the gross neurologic examination is normal.

Intracranial hydatid cyst: imaging findings of a rare disease.

Hydatid disease (echinococcosis) is a worldwide zoonosis produced by the larval stage of the Echinococcus tapeworm. The disease is endemic in many parts of the world, particularly in the Middle East, Australia, New Zealand, South America and central and south Europe. Intracranial hydatid disease is considered a rare disease and may be sometimes very difficult to diagnose based on the clinical and laboratory findings. Therefore, it is important to be aware of the condition and the imaging findings even in the non-endemic parts of the world. We report the case of a 12-year-old boy who presented with headache and vomiting for a few months. The mass was totally excised, with no postoperative complications. We present MR spectroscopy (MRS) findings in this operatively proven case of hydatid cyst of the brain. We discuss imaging findings, in particular the findings on MRS, which is rarely reported in the literature.

Lumbar subdural cerebrospinal fluid collection with acute cauda equina syndrome after posterior fossa decompression for Chiari malformation Type I: case report.

This report describes the circumstances of a patient with a cauda equina syndrome due to the development of a lumbar subdural CSF collection with ventral displacement of the cauda equina shortly following posterior fossa decompression for Chiari malformation Type I (CM-I). This unusual, but clinically significant, complication was successfully treated with percutaneous drainage of the extraarachnoid CSF collection. Although there are a few cases of intracranial subdural hygroma developing after surgery for CM-I, often attributed to a pinhole opening in the arachnoid, as far as the authors can determine, a spinal subdural hygroma associated with surgery for CM-I has not been recognized.