Skull base angiomatous leiomyoma: a case report and review of literature.

Leiomyomas, benign neoplasms of mesenchymal origin, are common in gastrointestinal and genitourinary tracts. They are comprised of well-differentiated smooth muscle cells with few mitotic figures. Leiomyomas should not exhibit metastatic potential, though metastasis has been reported. Primary intracranial lesions remain rare. Only 4 cases have been reported in immunocompetent patients. Here we report an intracranial leiomyoma in an immunocompetent patient. A 60 year-old woman with unremarkable past medical history presented with a right sixth nerve palsy. On examination, there was a right sixth nerve palsy with numbness over the right V2 and V3 areas. CT scan showed a well-defined lesion within the right middle cranial fossa adjacent to the cavernous sinus with bony remodelling of the lateral wall of the sphenoid sinus and greater wing of the right sphenoid. MRI showed extra axial mass lesion arising from the right Meckel´s cave/cavernous sinus. The lesion was T2 hyperintense and T1 isointense. Homogenously enhancing centrally with little enhancement peripherally. CT CAP showed no primary lesions. Differential diagnosis at that stage was between meningioma, schwannoma or metastasis. The patient underwent craniotomy and debulking of tumour. Histological analysis confirmed leiomyoma. Post-operative MRI showed residual enhancement in the region of Meckel's cave. As residual tumour was significant, the patient underwent STRS. Further MRI at 1 year showed regression of the tumour. Majority of intracranial leiomyomas are discovered in immunocompromised patients incidentally. Histology reveals spindle shaped cells with blunt ends and few mitotic figures. The best treatment option to date for primary intracranial leiomyomas remains surgical gross total resection without adjuvant radiation therapy. Intracranial leiomyoma transitioning to leiomyosarcoma post radiotherapy has been reported before and therefore follow up with serial imaging is warranted.

De novo syrinx formation in a patient with type I Chiari malformation: Case report and review of the literature.

This report highlights the case of a 56-year-old woman with an incidental type I Chiari malformation who on initial presentation had no associated cervical syrinx on magnetic resonance imaging (MRI), but who on subsequent MRI 8 years later was found to have developed a de novo upper cervical cord syrinx.

Syringomyelia secondary to "occult" dorsal arachnoid webs: Report of two cases with review of literature.

In a certain group of patients with syringomyelia, even with the advent of sophisticated magnetic resonance imaging (MRI), no associated abnormality or cerebrospinal fluid (CSF) block is easily identified. This type of syringomyelia is often termed idiopathic. Current literature has less than 10 reports of arachnoid webs to be the causative factor. We present our experience in the management of two cases of syringomyelia secondary to arachnoid webs. Both our patients presented with progressive neurological deterioration with MRI scans demonstrating cervical/thoracic syrinx without Chiari malformation or low-lying cord. There was no history of previous meningitis or trauma. Both patients underwent myelography that demonstrated dorsal flow block implying CSF obstruction. Cord displacement/change in caliber was also noted and this was not evident on MRI scans. Both patients underwent thoracic laminectomy. After opening the dura, thickened/abnormal arachnoid tissue was found that was resected thus widely communicating the dorsal subarachnoid space. Postoperatively at 6 months, both patients had significant symptomatic improvement with follow-up MRI scans demonstrating significant resolution of the syrinx. In patients with presumed idiopathic syringomyelia, imaging studies should be closely inspected for the presence of a transverse arachnoid web. We believe that all patients with idiopathic symptomatic syringomyelia should have MRI CSF flow studies and/or computed tomography (CT) myelography to identify such arachnoid abnormalities that are often underdiagnosed. Subsequent surgery should be directed at the establishment of normal CSF flow by laminectomy and excision of the offending arachnoid tissue.

Relapsing-Remitting Sixth Nerve Palsy in Association with Ollier's Disease.

Relapsing-remitting sixth nerve palsy is usually due to ophthalmoplegic migraine (recurrent cranial nerve palsy) in younger patients and microvascular disease in older patients. There have been isolated reports, however, of it occurring in the presence of a skull base tumour. We report a 20-year-old woman with Ollier's disease who presented with a relapsing-remitting sixth nerve palsy. Neuro-imaging revealed a skull base enchondroma.

FANCD2 re-expression is associated with glioma grade and chemical inhibition of the Fanconi Anaemia pathway sensitises gliomas to chemotherapeutic agents.

Brain tumours kill more children and adults under 40 than any other cancer. Around half of primary brain tumours are glioblastoma multiforme (GBMs) where treatment remains a significant challenge, where survival rates have improved little over the last 40 years, thus highlighting an unmet need for the identification/development of novel therapeutic targets and agents to improve GBM treatment. Using archived and fresh glioma tissue, we show that in contrast to normal brain or benign schwannomas GBMs exhibit re-expression of FANCD2, a key protein of the Fanconi Anaemia (FA) DNA repair pathway, and possess an active FA pathway. Importantly, FANCD2 expression levels are strongly associated with tumour grade, revealing a potential exploitable therapeutic window to allow inhibition of the FA pathway in tumour cells, whilst sparing normal brain tissue. Using several small molecule inhibitors of the FA pathway in combination with isogenic FA-proficient/deficient glioma cell lines as well as primary GBM cultures, we demonstrate that inhibition of the FA pathway sensitises gliomas to the chemotherapeutic agents Temozolomide and Carmustine. Our findings therefore provide a strong rationale for the development of novel and potent inhibitors of the FA pathway to improve the treatment of GBMs, which may ultimately impact on patient outcome.

Radiotherapy for atypical meningiomas.

OBJECT: The role of postoperative radiotherapy in patients undergoing first-time resection of WHO Grade II meningioma remains unclear as reflected by varied practices in published clinical studies and national professional surveys. Much of the relevant literature is based on pre-2000 WHO grading criteria for atypical meningiomas. Authors in this study set out to explore the role of postoperative radiotherapy in patients undergoing first-time surgery for WHO Grade II meningiomas diagnosed using revised WHO 2000 criteria, against a background of otherwise limited published literature on this issue. METHODS: The authors retrospectively collected data on 114 consecutive patients who underwent first-time resection of WHO Grade II atypical meningiomas diagnosed using 2000 WHO criteria, and who variably underwent postoperative radiotherapy according to individual surgeon practices. Outcomes, including radiological recurrence, were submitted to Kaplan-Meier and Cox regression analyses. RESULTS: Postoperative radiotherapy demonstrated a significant benefit only when patients who had undergone gross-total tumor resection and those who had undergone subtotal resection along with postoperative radiosurgery to the tumor remnant were excluded from analysis. CONCLUSIONS: The authors have performed the largest study in the literature to examine the use of radiotherapy for WHO Grade II, atypical, meningiomas following a first-time resection. They suggest that radiotherapy is not appropriate after first-time resection of those lesions in which a gross-total resection (Simpson Grade 1 or 2) has been achieved. They also advise that any tumor remnant radiologically demonstrated on postoperative imaging should be treated with radiosurgery and that postoperative radiotherapy after a first-time resection should be reserved for tumor remnants too large for radiosurgery and for which a second staged operation is not planned.

Vertebral metastatic chemodectoma: imaging and therapeutic octreotide. Case report.

The authors report on the use of external-beam radiotherapy and octreotide in a 32-year-old woman who presented with spinal cord compression secondary to metastatic chemodectoma. Scintigraphy studies were used to confirm the presence of somatostatin receptors. Magnetic resonance imaging, and in particular spinal angiography, were performed to define the extent of spinal metastatic disease. The literature on current investigation and management of vertebral metastatic chemodectoma is reviewed.