Prechiasmatic transection of the optic nerve in optic nerve glioma: technical description and surgical outcome.

Optic pathway glioma (OPG) encompasses a spectrum of findings ranging from lesions confined to the optic nerve only, lesions affecting the optic chiasm and hypothalamus, and lesions with diffuse involvement of a large part of the optic pathway and neighboring structures. The majority of pediatric low-grade astrocytomas in the optic/chiasmatic region are typical pilocytic astrocytoma. The rest of them (10 %) may be other gliomas such as fibrillary pilomyxoid astrocytoma (grade 2 WHO). The postsurgical local recurrence rate of 55 to 76 % has been reported in some histological subtypes such as pilomyxoid astrocytoma (grade 2). Performing a prechiasmatic transection might offer a new surgical option to avoid further tumor growth toward the chiasm in the optic nerve glioma with predominantly orbital manifestations. In this retrospective study, four patients (three children, two without neurofibromatosis type 1 (NF1), and one with NF1 and one adult without NF1) with optic nerve glioma without involvement of the chiasm but blindness, disfiguring proptosis, and pain of the affected eye were included. The surgical approach was performed as a combined approach from pterional extradural and intradural. Without any exceptions, vision of the contralateral eye could be preserved and did not show any deterioration after surgery or during the follow-up time between 17 and 106 months. Furthermore, in all patients, gross total tumor resection could be achieved. During follow-up observation in all patients, no further tumor progress or recurrences could be observed. None of the patients were treated postoperatively by radiotherapy or chemotherapy. Prechiasmatic transection of the optic nerve in optic nerve glioma without affecting the chiasm might offer a surgical treatment option to control tumor growth and to preserve vision of the contralateral eye.

Chiasmatic-hypothalamic masses in adults: a case series and review of the literature.

Chiasmatic-hypothalamic masses are commonly seen in children with neurofibromatosis type 1 and often demonstrate a benign clinical course. These masses are, however, rare in adults and require tissue sampling for diagnosis. Here, we present four cases of chiasmatic-hypothalamic masses in adults with pathologically proven diagnoses. We also provide a review of the literature and propose a limited differential in this age group.

Pilocytic astrocytoma of the optic pathway: a tumour deriving from radial glia cells with a specific gene signature.

Pilocytic astrocytomas are WHO grade I gliomas that occur predominantly in childhood. They share features of both astroglial and oligodendroglial lineages. These tumours affect preferentially the cerebellum (benign clinical course) and the optic pathway, especially the hypothalamo-chiasmatic region (poor prognosis). Understanding the molecular basis responsible for the aggressive behaviour of hypothalamo-chiasmatic pilocytic astrocytomas is a prerequisite to setting up new molecular targeted therapies. We used the microarray technique to compare the transcriptional profiles of five hypothalamo-chiasmatic and six cerebellar pilocytic astrocytomas. Validation of the microarray results and comparison of the tumours with normal developing tissue was done by quantitative real-time PCR and immunohistochemistry. Results demonstrate that cerebellar and hypothalamo-chiasmatic pilocytic astrocytomas are two genetically distinct and topography-dependent entities. Numerous genes upregulated in hypothalamo-chiasmatic pilocytic astrocytomas also increased in the developing chiasm, suggesting that developmental genes mirror the cell of origin whereas migrative, adhesive and proliferative genes reflect infiltrative properties of these tumours. Of particular interest, NOTCH2, a gene expressed in radial glia and involved in gliomagenesis, was upregulated in hypothalamo-chiasmatic pilocytic astrocytomas. In order to find progenitor cells that could give rise to hypothalamo-chiasmatic pilocytic astrocytomas, we performed a morphological study of the hypothalamo-chiasmatic region and identified, in the floor of the third ventricle, a unique population of vimentin- and glial fibrillary acidic protein-positive cells highly suggestive of radial glia cells. Therefore, pilocytic astrocytomas of the hypothalamo-chiasmatic region should be considered as a distinct entity which probably originates from a unique population of cells with radial glia phenotype.

Chordoid glioma of the third ventricle attached to the optic chiasm. Successful removal through a trans-lamina terminalis approach.

Chordoid glioma of the third ventricle constitutes a rare, very recently recognized histological entity. Most reports of this neoplasm, focused on its distinct histological features, have hypothesized about a probable origin of the lesion at the third ventricle floor and/or the lamina terminalis. We report on a new case, presenting neuroradiological and intraoperative pictorial evidences of the tumoral attachment, limited to the chiasm-lamina terminalis junction. A 53-year-old woman debuted with acute symptoms of obstructive hydrocephalus, visual disturbances and confusion. MRI investigation showed a large solid-cystic third ventricle mass bulging through the lamina terminalis and ventricular floor. After placing a ventriculoperitoneal shunt, the tumor was completely removed through a trans-lamina terminalis approach. A tight tumoral attachment to the junction of the posterior chiasm to the lamina terminalis was identified and dissected. No other adhesions to the third ventricle boundaries were found. A chordoid glioma was diagnosed on histological examination. One year after the surgical procedure the patient does not present new neurological deficits, and there are no signs of tumoral regrowth on the follow-up postoperative MRI. Chordoid glioma should be included in the differential diagnosis of third ventricle tumors. Preoperative neuroradiological suspicion of this lesion should alert the neurosurgeon about the presence of a tight tumoral adherence at the level of the chiasm-lamina terminalis junction. The trans-lamina terminalis approach provides a suitable route for an early control of this attachment under direct vision, allowing a safe dissection of the mass from the third ventricle.

[A case of primary central nervous system malignant lymphoma developing from the optic chiasma and hypothalamus].

A 63-year-old woman was admitted to our hospital with a 1-month history of visual field defect and reduced bilateral visual acuity. Neurological examination revealed enlargement of bilateral central scotoma and blurred vision, and brain MRI showed masses in the optic chiasma and right hypothalamus on Ti-weighted image with contrast enhancement. Although the patient was treated with high-doses of methylprednisolone four times, the masses spread to the right internal capsule and the vicinity of the right lateral ventricle. An open biopsy was performed and microscopic examination of the sample revealed lymphoplasmacytic lymphoma. Although primary central nervous system lymphoma (PCNSL) developing from the optic chiasma and hypothalamus is rare, PCNSL should be considered in patients with multiple lesions in the optic chiasma and hypothalamus.

[Pilocytic astrocytoma and diencephalic syndrome in an adult with neurofibromatosis type 1].

We report a rare case of pilocytic astrocytoma and diencephalic syndrome occurring together in a 30-year-old woman with neurofibromatosis type 1 (NF-1). Diencephalic syndrome included emaciation and somnolence. Gadolinium-enhanced magnetic resonance imaging revealed a brain tumor in the optic chiasmal-hypothalamic region, which invaded the thalamus, brain stem and cerebellum. A biopsy specimen from the chiasmal tumor was compatible with pilocytic astrocytoma. Although she received radiation therapy, she died 22 months after the onset of the disease.