scholarly journals Treatment of BRAF V600E mutated ganglioglioma of the third ventricle with dabrafenib

2021 ◽  
Vol 12 ◽  
pp. 529
Author(s):  
Ethan John Kilmister ◽  
Bridget Robinson ◽  
Claudio De Tommasi

Background: Ganglioglioma (GG) of the third ventricle is rare. Surgical excision of tumors in this location is associated with high morbidity due to nearby eloquent brain centers. Alternative treatments, when available, should be considered to reduce risks of surgical treatment. Case Description: We present the case of a 21-year-old female diagnosed with a BRAF V600E mutated GG of the third ventricle. After an endoscopic biopsy and insertion of a ventriculoperitoneal shunt, the patient was started on the BRAF inhibitor dabrafenib, as an alternative to surgery or radiation. Nearly 2 years after starting dabrafenib, her tumor appearance on serial magnetic resonance imaging is stable, and she has maintained a good quality of life with no new neurological symptoms. Conclusion: The disease control thus far suggests targeted medical therapy of GG of the third ventricle with BRAF inhibitors may have efficacy and should be a considered treatment modality.

2021 ◽  
Vol 12 ◽  
pp. 523
Author(s):  
Ragavan Manoharan ◽  
Jonathon Parkinson

Background: Pure epidural spinal cavernous hemangiomas (SCH) account for only 4% of all spinal epidural lesions. Our literature review identified 61 publications reporting on, a total of 175 cases in the magnetic resonance imaging era. Here, we reviewed those cases, and have added our case of what appeared to be a multifocal SCH. Case Description: A 72-year-old male presented with a progressive paraparesis attributed to a T5/T6 dorsolateral extradural mass extending into the right T5/6 foramen. Surgical excision documented the lesion, histologically, was a SCH. A second similar lesion was noted involving the left C7/T1 foramen; as the patient was asymptomatic from this lesion, and no additional biopsy was performed. The patient returned to normal neurological function within 2 months postoperatively. Conclusions: Here, a 72-year-old male presented with a pathologically confirmed T5/T6 epidural SCH and a secondary C7/T1 foraminal lesion suspected to represent a secondary focus of an epidural SCH.


2013 ◽  
Vol 73 (2) ◽  
pp. ons132-ons140 ◽  
Author(s):  
Tomasz Matys ◽  
Avril Horsburgh ◽  
Ramez W. Kirollos ◽  
Tarik F. Massoud

Abstract BACKGROUND: The aqueduct of Sylvius (AqSylv) is a structure of increasing importance in neuroendoscopic procedures. However, there is currently no clear and adequate description of the normal anatomy of the AqSylv. OBJECTIVE: To study in detail hitherto unavailable normal magnetic resonance imaging morphometry and anatomic variants of the AqSylv. METHODS: We retrospectively studied normal midsagittal T1-weighted 3-T magnetic resonance images in 100 patients. We measured widths of the AqSylv pars anterior, ampulla, and pars posterior; its narrowest point; and its length. We recorded angulation of the AqSylv relative to the third ventricle as multiple deviations of the long axis of the AqSylv from the Talairach bicommissural line. We statistically determined age- and sex-related changes in AqSylv morphometry using the Pearson correlation coefficient. We measured angulation of the AqSylv relative to the fourth ventricle and correlated this to the cervicomedullary angle (a surrogate for head position). RESULTS: Patients were 13 to 83 years of age (45% male, 55% female). Mean morphometrics were as follows: pars anterior width, 1.1 mm; ampulla width, 1.2 mm; pars posterior width, 1.4 mm; length, 14.1 mm; narrowest point, 0.9 mm; and angulation in relation to the third and fourth ventricles, 26° and 18°, respectively. Age correlated positively with width and negatively with length of the AqSylv. There was no correlation between AqSylv alignment relative to the foramen magnum and the cervicomedullary angle. CONCLUSION: Normative dimensions of the AqSylv in vivo are at variance with published cadaveric morphometrics. The AqSylv widens and shortens with cerebral involution. Awareness of these normal morphometrics is highly useful when stent placement is an option during aqueductoplasty. Reported data are valuable in guiding neuroendoscopic management of hydrocephalus and aqueductal stenosis.


Neurosurgery ◽  
2008 ◽  
Vol 63 (5) ◽  
pp. E1003-E1003 ◽  
Author(s):  
Rossana Romani ◽  
Mika Niemelä ◽  
Miikka Korja ◽  
Juha A. Hernesniemi

Abstract OBJECTIVE AND IMPORTANCE Colloid cysts of the third ventricle are rare benign tumors of endodermal origin accounting for 1% of all intracranial tumors. Interestingly, a few familial cases have been reported previously. We present the first case of dizygotic twins with a symptomatic colloid cyst of the third ventricle. CLINICAL PRESENTATION A 10-year-old boy was admitted to a local hospital in 1993 because of severe progressive headache. Computed tomographic and magnetic resonance imaging scans revealed acute obstructive hydrocephalus attributable to a third ventricular colloid cyst, which was removed after emergent ventricular drainage. Fourteen years later, a nonidentical twin brother complained of continuous headache with nausea and vomiting. A magnetic resonance imaging scan showed obstructive hydrocephalus and a third ventricle colloid cyst, which was removed by use of the transcallosal approach. INTERVENTION Both twins underwent complete removal of the cyst by the interhemispheric transcallosal approach without postoperative complications. CONCLUSION On the basis of a literature review, 2 cases of colloid cysts of the third ventricle in monozygotic twins and a few familial cases have been reported. Our case is the first in dizygotic twin brothers. These findings suggest that the prevalence of colloid cyst may be higher in twins than in the general population. We believe that the presence of this lesion in a twin necessitates magnetic resonance imaging of the other twin, and a clinical follow-up would be recommended in all other first-degree relatives.


1987 ◽  
Vol 67 (2) ◽  
pp. 220-223 ◽  
Author(s):  
Mohsen Mohadjer ◽  
Elemer Teshmar ◽  
Fritz Mundinger

✓ Colloid cysts in the foramen of Monro and third ventricle account for 0.5% to 1% of all intracranial space-occupying lesions. The introduction of computerized tomography (CT) and magnetic resonance imaging has represented a major advance in the early detection of these cysts. The risks associated with the management of benign space-occupying lesions by open surgical procedures have made it necessary to search for safer techniques. The CT-stereotaxic method provides a simple, precise, and safe method of puncturing deep-seated space-occupying cysts. Between January, 1979, and December, 1984, 12 patients with colloid cysts in the foramen of Monro and third ventricle were operated on by this method. The operations were successful, and there were no intraoperative or postoperative complications. The advantages of the CT-stereotaxic method are discussed and the results are presented.


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