Thalamic rosette-forming a glioneuronal tumor in an elderly patient: Case report and literature review.

The rosette-forming glioneuronal tumor (RGNT) is a novel type of brain tumor recently listed in the WHO 2007 classification of central nervous system (CNS) tumors. We report the case of a 75-year-old woman harboring a thalamic RGNT with third ventricle dissemination. Age and location make the present case exceptional and which has never previously been reported. A review of the clinical, pathological and radiological features is presented along with the relevant literature.

Intraventricular craniopharyngiomas: surgical management and outcome analyses in 24 cases.

OBJECTIVE: Pure intraventricular craniopharyngioma is a rare subtype of craniopharyngioma that attaches frequently to the hypothalamus. The main challenge in tumor removal is protection of hypothalamic structure. The aim of our study was to set up a feasible risk-evaluation approach to help the surgeons make an individual treatment plan. METHODS: We reviewed retrospectively 24 patients with pure intraventricular craniopharyngioma who underwent surgical therapy. Third ventricular deformation and thalamic attachment of the tumor were assessed by preoperative magnetic resonance imaging and intraoperative inspection. Correlations between the outcome, extent of removal, and different attachment grades were analyzed. RESULTS: Preoperative magnetic resonance imaging grade had a significant correlation with functional outcome. Attachment grade was significantly correlated with endocrine, functional, and radiologic outcome. In the gross total removal (GTR) group, significantly more newly developed endocrinologic deficits were observed compared with patients who underwent subtotal removal (STR). All 6 GTR cases with the greatest attachment grade (grade 2) acquired newly developed endocrine insufficiency. CONCLUSIONS: Attachment and deformation grade evaluated based on our criteria are significantly correlated with postoperative outcome. GTR of tightly attached tumor is associated with worse endocrinologic, functional, and radiologic outcomes compared with STR. Hence, we suggest that individual surgical plans should be made according to the grade of tumor attachment and hypothalamic deformation. STR should used in tumors with a high grade to achieve a good long-term outcome and avoid severe postoperative sequelae.

The transventricular preforniceal approach for exophytic chiasmatic/hypothalamic astrocytomas extending into the anterior third ventricle.

BACKGROUND: Surgical treatment of large exophytic chiasmatic/hypothalamic astrocytomas extending into the anterior third ventricle remains a challenging task for neurosurgeons. In particular, when the tumor extends from the chiasmatic region upward to the foramen of Monro, damage to the fornix and other neurovascular structures is a major concern. OBJECTIVE: To describe the technique used in the transventricular preforniceal surgical approach to remove the superior and superoposterior part of the tumor in the third ventricle for treatment of exophytic chiasmatic/hypothalamic astrocytoma. METHODS: The transventricular preforniceal approach was used in two cases of exophytic chiasmatic/hypothalamic astrocytoma. The approach is summarized in 4 procedures: 1) exposure of the anterior horn of the lateral ventricle by the transcallosal approach, 2) identification of the foramen of Monro and the fornix, 3) incision of the septum pellucidum or the wall of the lateral ventricle, in front of the columns of the fornix, and 4) removal of the tumor through the space between the anterior commissure and the columns of the fornix. RESULTS: Because the tumor compressed the foramen of Monro posteriorly and stretched the space between the anterior commissure and the columns of the fornix, the posterosuperior part of the tumor in the third ventricle was successfully removed through the surgical corridor in front of the columns of the fornix. In both cases, tumors were successfully removed using this approach without damaging the fornix and the anterior commissure. Residual tumor was removed using an anterior interhemispheric translamina terminalis approach in a two-stage surgery. CONCLUSIONS: The transventricular preforniceal approach can be applied for removing the superior part of exophytic chiasmatic/hypothalamic astrocytomas, because the space between the anterior commissure and the fornix is stretched by the tumor, providing an appropriate surgical corridor.

Infundibulo-tuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category.

PURPOSE: This study investigates retrospectively the clinical, neuroradiological, pathological and surgical evidence verifying the infundibulo-tuberal topography for craniopharyngiomas (CPs). Infundibulo-tuberal CPs represent a surgical challenge due to their close anatomical relationships with the hypothalamus. An accurate definition of this topographical category is essential in order to prevent any undue injury to vital diencephalic centres. METHODS: A systematic review of all scientific reports involving pathological, neuroradiological or surgical descriptions of either well-described individual cases or large series of CPs published in official journals and text books from 1892 to 2011 was carried out. A total of 1,232 documents providing pathological, surgical and/or neuroradiological evidence for the infundibulo-tuberal or hypothalamic location of CPs were finally analysed in this study. FINDINGS: For a total of 3,571 CPs included in 67 pathological, surgical or neuroradiological series, 1,494 CPs (42%) were classified as infundibulo-tuberal lesions. This topography was proved in the autopsy of 122 non-operated cases. The crucial morphological finding characterizing the tubero-infundibular topography was the replacement of the third ventricle floor by a lesion with a predominant intraventricular growth. This type of CP usually presents a circumferential band of tight adherence to the third ventricle floor remnants, formed by a functionless layer of rective gliosis of a variable thickness. After complete surgical removal of an infundibulo-tuberal CP, a wide defect or breach at the floor of the third ventricle is regularly observed both in the surgical field and on postoperative magnetic resonance imaging studies. CONCLUSIONS: Infundibulo-tuberal CPs represent a major topographical category of lesions with a primary subpial development at the floor of the third ventricle. These lesions expand within the hypothalamus itself and subsequently occupy the third ventricle; consequently, they can be classified as not strictly intraventricular CPs. A tight attachment to the hypothalamus and remnants of the third ventricle floor is the pathological landmark of infundibulo-tuberal CPs.

Interhemispheric endoscopic resection of large intraventricular and thalamic tumors.

A transfrontal route is the traditional endoscopic approach to intraventricular tumors. Small lesions can be biopsied using the parallel port channel of the endoscope. For larger tumors a ventriculoport can be used for resection. This technique nevertheless requires traversing the brain tissue, is difficult in the setting of small ventricles, and allows only limited mobility. The authors describe the endoscopic resection of large intraventricular tumors via an interhemispheric route using rigid suction with a mounted endoscope, and thus circumventing some of the problems with the traditional approach.

Feasibility of neuroendoscopic biopsy of pediatric brain tumors.

PURPOSE: The purpose of this study was to investigate the potential value of neuroendoscopic biopsies in pediatric patients with peri- or intraventricular tumors. METHODS: From 2001 to 2008, 49 pediatric patients (mean age, 12.16 years) with tumors located in the intraventricular or paraventricular areas underwent neuroendoscopic biopsy, with or without simultaneous endoscopic third ventriculostomy. Neuroendoscopic biopsies were performed to verify the histological diagnosis of neoplasms and to establish pathological diagnoses necessary for planning appropriate treatment strategies. RESULTS: In 45 of 49 patients (91.8%) neuroendoscopic biopsy specimens were appropriate for diagnosis and revealed 27 germinomas, 11 astrocytomas, and one ependymoma, etc. The tumor location included the pineal gland (n = 28), thalamus (n = 7), intraventricle (n = 3), hypothalamus (n = 3), suprasellar area (n = 2), and diffuse multifocal area (n = 3). In two patients (4.1%) biopsy specimens were informative but not diagnostic. Tumor tissue specimens were undiagnostic in two patients (4.1%). There were eight transient morbidities, including four EOM limitations, two central DI, one EVD infection, and one CSF leakage. One patient experienced postoperative tumor bleeding requiring emergent operation. There was no case of operative mortality. CONCLUSION: Neuroendoscopic biopsy can be considered as the first choice for tissue sampling of periventricular and intraventricular tumors with acceptable risks.

Simultaneous choroid plexus carcinoma and pilocytic astrocytoma in a pediatric patient.

Simultaneous primary brain tumors in pediatric patients without prior chemotherapy or radiotherapy, phacomatosis, or known familial history are a rare occurrence. The authors report the case of a 4-year-old boy with simultaneous choroid plexus carcinoma and pilocytic astrocytoma with features of oligodendroglioma. Magnetic resonance imaging studies revealed diffuse heterogeneously enhancing left intraventricular and posterior fossa tumors initially believed most consistent with multicentric choroid plexus carcinomas. A multiple staged resection was carried out for each tumor and gross-total resection was achieved. Upon gross inspection intraoperatively as well as postoperative histological analysis, 2 distinct simultaneous tumors were identified: choroid plexus carcinoma and pilocytic astrocytoma. To the authors' knowledge this is the first case report published identifying 2 distinct tumor types with similar radiological appearances in a pediatric patient with no prior history of radiotherapy, chemotherapy, or phacomatosis.

Rare primary tumours of the hypothalamus in adults: clinical course and surgical treatment.

BACKGROUND AND PURPOSE: The paper presents the operative technique and the results of treatment of adult patients with primary tumours of the hypothalamus, including rare ones. The aim of the study was to show the possibility of safe surgical treatment of rare tumours of the hypothalamus through a bifrontal basal interhemispheric trans-lamina terminalis approach. MATERIAL AND METHODS: Five patients with tumours of the hypothalamus were operated on in the Neurosurgical Clinic in Sosnowiec between 1990 and 2008. There were 2 patients with craniopharyngiomas located exclusively in the third ventricle, and single patients with gemistocytic astrocytoma, Langerhans cell histiocytosis X and hamartoma of the hypothalamus each. The patients were treated surgically with a bi-frontal basal interhemispheric trans-lamina terminalis approach. In two cases, the neuronavigation system with the use of tractography (DTI) was used to determine the location of the lamina terminalis, the posterior surface of the optic chiasm and the optic tracts. RESULTS: All lesions were resected totally, except for partially resected hamartoma of the hypothalamus. The most common postoperative complication was diabetes insipidus, which was transient in two cases. A long-lasting follow-up of all the patients operated on did not reveal regrowth of the lesion. CONCLUSIONS: The bifrontal basal interhemispheric trans-lamina terminalis approach allows for radical resection of primary tumours of the hypothalamus while avoiding serious post-operative deficits. This approach enabled the preservation of the olfactory bulb and tract and prevented damage of the frontal lobes. The use of DTI helped to establish the location and borders of the lamina terminalis, to establish the posterior surface of the optic chiasm and the optic tracts, and to save the anterior and lateral wall of the hypothalamus.

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.

[Case report of germinoma with synchronous lesions in the thalamus and para lateral ventricle].

A 25-year-old man presented with headache and consciousness disturbance. MRI revealed well enhanced mass lesions using gadolinium at bilateral thalamus and right para lateral ventricle with mild perifocal edema. Obstructive hydrocephalus was observed. On neuroendoscopic observation, the tumor was not appeared on the surface of the ventricle, therefore biopsy of the lesion was not sufficient to make a definitive pathological diagnosis. Third ventriculostomy was successful and the patient's consciousness improved. One week later, a stereotactic biopsy for right para lateral ventricle lesion made possible the histological diagnosis of typical germinoma. Post operative course was uneventful. Chemotherapy (CDDP and etoposide) following 30 Gy whole ventricle irradiation resulted in the complete response of the lesion. Four years later, he has no neurological deficit except for mild disturbance in his eye movement. Our experience suggests that a synchronous lesion at thalamus and para lateral ventricle seems to be due to subependymal infiltration other than CSF dissemination.

Suprasellar hemangioblastoma.

A 59-year-old woman presented with disturbance of consciousness and decreased visual acuity caused by a suprasellar mass identified on MRI. A bifrontal interhemispheric approach allowed removal of the top and lateral sides of the tumor from the wall of the third ventricle. The hypothalamus appeared to be the origin of the mass, which proved to be hemangioblastoma, a rare tumor in this location.

Primary hypothalamic-third ventricle lymphoma. Case report and review of the literature.

Primary central nervous system lymphomas (PCNSL) are infrequent tumors and their presentation as a solitary hypothalamic-third ventricle mass can be considered exceptional. We report the case of a 57-year-old woman with progressive visual deterioration, diabetes insipidus and mental confusion. She had a diffuse and homogeneous tumoral lesion involving the third ventricle and the adjacent hypothalamic area with marked enhancement after contrast administration on both, competed tomography scan and magnetic resonance images. It was approached and partially resected by the translamina terminalis route. Histological diagnosis proved to be a diffuse non-Hodgkin lymphoma and the patient subsequently was treated with adjuvant radiotherapy and chemotherapy. Followup examination showed visual acuity recover but persistent confessional state. Eight similar well described cases reported in the literature are reviewed with a description of the major diffenciating features of this neurological entity. Treatment of PCNSL remains a challenge, and the topographical location within the hypothalamic-third ventricle area is even more complex.

Neurofibromatosis type 1-associated unusual pleomorphic astrocytoma displaying continual malignant progression.

Patients with neurofibromatosis type 1 (NF1) often have gliomas as a complication, most of which are benign pilocytic astrocytomas which have arisen in optic pathways. In the present case, a 17-year-old girl (at death) with stigmata of NF1, initially had a bulky tumor mass in the left thalamus, developing into the lateral ventricle, at 13 years of age. Partially resected tissue samples showed pleomorphic astrocytoma with abundant xanthoma cells and degenerative structures such as Rosenthal fibers (RF) and eosinophilic granular bodies. Fine eosinophilic granules identical to RF, both immunophenotypically and ultrastructurally, were also seen. The residual tumor was subtotally resected 6 months later, and the tumor histology was essentially similar as before, accompanying the regenerative structures; this was believed to be a good prognostic indicator. However, several anaplastic features such as mitosis, necrosis and vascular proliferation appeared even in areas rich in the regenerative structures. After a 2-year, disease-free interval, multiple tumor relapse occurred in June 1997. Partially resected tumor tissues were composed of monotonous small anaplastic cells with prominent proliferative activity. Surprisingly, the tumor cells had retained eosinophilic granules within the cell bodies. Postoperative chemotherapy with procarbazine, MCNU and vincristine (PCV) suppressed the residual tumor dramatically, but the regrowing tumor finally became uncontrollable, leading to the patient's death. TP53 mutation was not detected, while p27 immunopositivity was constantly high during malignant progression, suggesting acquisition of proliferative activity to overcome p53 and p27 inhibitory functions. A review of previously published reports failed to reveal any cases of this type.

The fate of the cerebrospinal fluid after neuroendoscopic third ventriculostomy.

This paper describes the case of a 9-year-old girl with a posterior thalamic/pineal region lesion and secondary obstructive hydrocephalus. The hydrocephalus was treated by neuroendoscopic third ventriculostomy (NTV), and she underwent simultaneous transendoscopic biopsy. The tumour biopsy was haemorrhagic, but the bleeding settled with constant irrigation. The patient remained neurologically unchanged, but subsequent imaging revealed an asymptomatic thoracolumbar spinal subdural haematoma thought to be due to blood flowing out through the NTV into the subdural space. This demonstrates the subdural location of the cerebrospinal fluid after NTV.

Diffuse bilateral thalamic astrocytomas as examined serially by MRI.

We report the case of a 13-year-old girl with diffuse bilateral thalamic astrocytomas. Incoordination was observed at the onset. Cranial computed tomography (CT) showed enlarged thalami, and magnetic resonance imaging (MRI) revealed these lesions to be symmetrically enlarged with high intensity on the T2-weighted image. Owing to these atypical findings in the neuroimaging studies, we had difficulty in making the correct diagnosis of a brain tumor. After the diagnosis of diffuse bilateral thalamic astrocytomas was obtained, we performed hyperfractionated radiotherapy followed by chemotherapy. Radiation therapy was effective for a while, but the girl's condition deteriorated again and she died 8 months after admission. Although diffuse bilateral thalamic astrocytomas are difficult to diagnose because they do not resemble most other neoplasms on neuroimaging studies, pediatricians should keep this entity in mind in order to arrive at a precise and prompt diagnosis.

Surgery of giant gliomas of chiasma and IIIrd ventricle.

During a period of 17 years (from 1976 till now) 45 patients with giant gliomas of the chiasma and the IIIrd ventricle out of a total amount of 120 patients with hypothalamic gliomas were operated. The following classification of tumours was used: I) tumours with predominant anterior growth; II) tumours which infiltrate chiasma and penetrate into the IIIrd ventricle; III) gliomas of the floor of the IIIrd ventricle and the chiasma, growing into the ventricle cavity; IV) tumours of the chiasma, optic tract and thalamus. The authors come to the conclusion, that surgical removal of giant tumours of the chiasma and the IIIrd ventricle, though risk, may result in an improvement or stabilisation of visual functions (77%) and a long period free from recurrencies (9.5%). The postoperative period is relatively favourable and the mortality is low (6%). The main contraindication in our opinion is a wide infiltration of adjacent brain structures by the tumour and spreading along both optical tracts. We consider the giant size of a tumour in itself a sufficient indication for surgery.

Pathology of midline brain tumors. Immunocytochemical tumor markers and classificatory aspects.

Our understanding of the pathology of human brain tumors has considerably increased in recent years thanks to investigation of nervous tissue markers which can be demonstrated by immunocytochemical techniques in sections of neurosurgical tissue specimens. This development is presented here in a review of the most important entities among midline brain tumors. Detection of neural differentiation markers such as glial fibrillary acidic protein (GFAP), S 100 protein (S 100 p), neurofibrillary proteins and neuron-specific enolase (NSE) greatly contributed to clarifying the histogenesis and differentiation potential of undifferentiated small-cell tumors grouped as primitive neuroectodermal tumors (PNETs) which include cerebellar medulloblastomas and pinealoblastomas as well as neuroblastomas and ependymoblastomas. Among medulloblastomas, the desmoplastic variety (formerly called by some "arachnoidal sarcoma of the cerebellum") shows frequent glioneuronal differentiation. Monoclonal antibodies recognizing antigenic determinants specific for tumor types and grades of malignancy/differentiation will gain significance in brain tumor diagnosis, as demonstrated in some examples. Despite the development of more objective ("scientific") criteria for typing human brain tumors, the "art" of classical histopathologic evaluation is not replaced but supplemented by the wealth of data supplied by such modern accomplishments.

Gangliocytoma of third ventricle: hyperphagia, somnolence, and dementia.

A 55-year-old man had hydrocephalus caused by a third-ventricular tumor. Mentation improved after ventricular shunting and radiation therapy. Progressive hyperphagia, obesity, memory impairment, and hypersomnolence developed 13 months later, and he died 2 years after diagnosis. At necropsy, a gangliocytoma filled the third ventricle, resulting in compression and necrosis of the ventromedial and posterior hypothalamic nuclei. Clinical manifestations were attributed to these lesions.