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  • Author or Editor: Vivek A. Josan x
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Vivek Josan, Paul Smith, Andrew Kornberg, Christian Rickert and Wirginia Maixner

✓Dysembryoplastic neuroepithelial tumors (DNETs) are benign supratentorial tumors based in the cerebral cortex. They usually are found in children and young adults with seizures that tend to become refractory to medical treatment. In the vast majority of cases resection results in good seizure control, and adjuvant therapy is not required. When tumors thought to be DNETs are not resected due to their proximity to eloquent cortex, lack of change in the clinical and neuro-imaging features over time supports the diagnosis of DNET.

The authors report on a patient in whom a pilocytic astrocytoma developed within a DNET, raising questions regarding the classification of these lesions and the need for lifelong clinical and imaging surveillance. This paper adds to the growing body of literature about the biological behavior of these lesions.

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Lucy O'Connor, Thomas Curl-Roper, Nicola Reeves, Andras A. Kemeny and Vivek A. Josan

The authors present the rare case of complete image-defined resolution of a hypothalamic hamartoma (HH) following Gamma Knife surgery (GKS). A 9-month-old girl presented with an episode of generalized tonic-clonic seizures. Magnetic resonance imaging revealed a left-sided HH, which remained radiologically stable. By 3 years of age the patient had a development delay of 12 months, and experienced 8 gelastic seizures per day while on 2 antiepileptic medications. Thirty-one months after presentation, the patient underwent elective GKS to treat the HH. She has since been seizure free for 22 months, while receiving 3 antiepileptic medications. Twelve months after radiosurgery, MRI revealed complete radiological resolution of the lesion. The authors discuss alternative management options for HH, including microsurgical resection, endoscopic disconnection, stereotactic radiofrequency thermocoagulation, and interstitial radiosurgery. Gamma Knife surgery is a minimally invasive procedure associated with a lower morbidity rate than that of published surgical results. The present case demonstrates the potential for complete image-defined resolution of an HH post-GKS, without long-term neurological sequelae, emphasizing the safety and efficacy of this therapeutic option for the control of epileptic seizures produced by small-volume, surgically inaccessible HHs.

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Vivek Anand Josan, Craig Douglas Timms, Christian Rickert and David Wallace

✓Central precocious puberty in girls is uncommon and tends to be idiopathic in most cases. In about 20 to 30% of cases there is an intracranial mass lesion. The common lesions are hypothalamic hamartomas, optic nerve gliomas, supra-sellar arachnoid cysts, hydrocephalus, germinomas, and other sellar/suprasellar lesions. Central precocious puberty secondary to a cerebellar astrocytoma is extremely rare. The authors report the first case in a girl who presented with several episodes of bleeding per vaginum. There was no clinical or radiological evidence of raised intracranial pressure.

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Raphael H. Sacho, Marco Kogels, Daniel du Plessis, Simon Jowitt and Vivek A. Josan

Primary dural lymphomas are very rare tumors—usually low-grade B-cell lymphomas of mucosa-associated lymphoid tissue type or marginal zone B-cell lymphomas. Primary dural involvement by diffuse large B-cell lymphoma is extremely rare, with only a few cases reported in the literature. The authors present an unusual case of primary dural involvement by a high-grade diffuse large B-cell lymphoma that presented as an acute subdural space-occupying mass and required emergency neurosurgical intervention.

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Nii K. Addo, Ian D. Kamaly-Asl, Vivek A. Josan, Anna M. Kelsey and Edward J. Estlin

The authors report the case of a 14-month-old boy with a large right intraventricular choroid plexus papilloma (CPP) for which the first attempt at resection resulted in life-threatening intraoperative hemorrhage. The tumor was unsuitable for embolization, and neoadjuvant ifosfamide, carboplatin, etoposide (ICE) chemotherapy had no effect on tumor size. However, chemotherapy with vincristine, although not impacting on CT perfusion parameters, resulted in a significant decrease in tumor size, enabling complete resection with manageable blood loss. The mechanism underlying the effect of vincristine in this case is uncertain, but it is a treatment strategy that warrants further evaluation for the treatment of CPPs that are not amenable to embolization.