Rare-26. gliomatosis, to be or not to cerebri: an evaluation of host systemic immune factors that potentially predispose to diffuse infiltrative glioma patterns
Gliomatosis cerebri (GC) has been debated as a pathologic entity sui generis versus a simple imaging and histologic pattern. Whereas the World Health Organization (WHO) 2000 classification considered GC a distinct tumor entity, WHO 2007 downgraded GC to a diffuse glioma pattern of extensive CNS infiltration, mostly of astrocytic phenotype involving at least three cerebral lobes, and the WHO 2016 further downgraded GC by removing the chapter. Tumors with distinctive GC presentation have been proven to be molecularly heterogeneous; however, paucity of data defining the pathophysiology and genetic driver(s) of this extensive ...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Gatson, N. T., Matsuoka, C. K., Rodriguez-Linares, Y., Pillainayagam, C., Cachia, D., Chi, T. L., Weinberg, J., Tremont-Lukats, I., Fuller, G., de Groot, J., Loghin, M. Tags: RARE TUMORS Source Type: research
Rare-25. long-term outcome of skull base chordomas in children and adolescents
CONCLUSIONS:The results of this series, which included a sizeable number of patients, treated in a consistent manner, by one surgeon, with a long-term follow-up period, make significant findings of the value of radical surgical resection associated with adjuvant proton/photon radiotherapy for a longer survival of children and adolescents with skull base chordoma. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Hulou, M. M., Rassi, M., Almefty, K., Bi, W. L., Pravdenkova, S., Dunn, I., Smith, T., Al-Mefty, O. Tags: RARE TUMORS Source Type: research
Rare-24. the use of a programmable shunt in a patient, treated with optune(r): feasibility report
In this report we focused on safety of using Optune in a patient with a programmable shunt (Codman®Hakim® Programmable Valve System). We performed an X-ray of the shunt valve prior to the treatment start, one day after the start and 14 days after the start of Optune to ensure that TTFields didn’t change the valve position. We also performed a CT Scan prior to the start and an MRI after the "14 days valve control" without observing any changes. Due to safety reasons we didn’t select a patient with a hydrocephalus for this first case feasibility report. This GBM patient received the shunt after ...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Schreiber, L., Wettig, M., Jung, S., Kinzel, A., Scholz, M. Tags: RARE TUMORS Source Type: research
Rare-23. patterns of care and survival outcomes in patients with extra ventricular: an individual patient data analysis
CONCLUSION:EVN is a very rare tumor. Most common site of tumor is frontal lobe. PFS is significantly better for younger patients with disease inside cerebrum and MIB labelling index <8%. However, extent of surgery and adjuvant radiation had no impact on survival. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Mallick, S., Benson, R., Rath, G. K., Julka, P. Tags: RARE TUMORS Source Type: research
Rare-22. medulloblastoma in a child with neurofibromatosis-1: case report and review
We report a case of Medulloblastoma in a genetically proven case of NF-1.Our patient was diagnosed to have NF-1 in infancy based on clinical / radiological features (Café-au-lait macules, axillary freckling, cutaneous / plexiform neurofibromas and sphenoid wing dysplasia).In the past 2 years prior to presentation she started having intermittent episodes of dizziness and ataxia which gradually worsened and was associated with headache and vomiting. MRI of the brain revealed a midline posterior fossa mass originating from the floor of the fourth ventricle. She underwent a complete surgical resection of the mass a...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Vanan, M. I., McDonald, P., Marles, S., Frosk, P., Krawitz, S., Moffat, H. Tags: RARE TUMORS Source Type: research
Rare-21. a national cancer database analysis of the impact of race and insurance status on receipt of observation for acoustic neuroma
CONCLUSIONS:AN patients on Medicaid, treated in an integrated network, residing in the central United States, or with a Charlson/Deyo score of one were less likely to be treated solely with observation. Conversely elderly patients (by 120%), African-Americans (by 50%), patients on Medicare (by 80%), or patients treated in a community hospital (by 200%), were more likely to be treated solely with observation for AN. Given that African-American race and lack of private insurance are independent risk factors for in-hospital morbidity/mortality following AN surgery (Curry et al., 2010), the finding from the present analys...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: McClelland, S., Murphy, J. D., Thomas, C. R. Tags: RARE TUMORS Source Type: research
Rare-20. pediatric tectal plate gliomas with pilomyxoid histologic features. report of three cases
Pediatric tectal plate gliomas (TPG) are generally considered indolent lesions that uncommonly progress. The majority of these patients require cerebrospinal fluid (CSF) diversion via ventriculostomy or shunting, but oftentimes no tissue is retrieved for pathologic diagnosis. The few reports of TPG histology describe mainly low grade astrocytomas, with few reports of ependymoma, malignant glioma, or pilocytic astrocytoma. We have encountered three pediatric TPGs with pilomyxoid histologic features. This cohort includes two females (ages 3.5 and 7 years) and one male (age 3.8 years). All of these children presente...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Fuller, C., Fouladi, M. Tags: RARE TUMORS Source Type: research
Rare-19. a case report of adenoid cystic carcinoma in the brain mimicking malignant glioma on neuroimaging
DISCUSSION:Adenoid cystic carcinoma is found primarily in the salivary glands and rarely discovered in the brain tissue. The gold standard of treatment of ACC tumors is surgical resection followed by radiation treatment. The patient described above is a rare case of ACC in the brain who presented with symptoms of headache, eye pain and leg weakness now with symptoms of cranial nerve six palsy and trigeminal neuralgia. These tumors can frequently mimic malignant gliomas on MRI studies, therefore pathological examination of tissue is necessary to make the correct diagnosis. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Liedke, M., Kowalska, A. Tags: RARE TUMORS Source Type: research
RARE-18. DETECTION, MOLECULAR PROFILING AND CULTURE OF CSF-CTCs IN LEPTOMENINGEAL DISEASE (LMDz) IN MELANOMA
CONCLUSIONS:These results indicate the potential value of CSF as a biopsy surrogate for assessing genetic makeup of CSF malignancies. Future directions involve the single cell analysis of CSF-CTCs, in vitro drug screens and in vivo LMDz models. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Evernden, B., Kenchappa, R., Puskas, J., Ryzhova, E., Caceres, G., Fedorenko, I., Tran, N. D., Etame, A. B., Sahebjam, S., Magliocco, A., Smalley, K., Forsyth, P. Tags: RARE TUMORS Source Type: research
Rare-16. a case report of autograft olfactory stem cell transplant for spinal cord injury developing into a persistent spinal mass
We report a case of olfactory mucosal stem cell transplantation for traumatic SCI at the level of C6-7. Three years after the transplant, the patient developed new neurological symptoms of arm weakness. MRI showed a multi-lobulated mass at the site of the stem cell transplant. The tumor was surgically removed and pathological analysis revealed mucosal cells within the mass suggesting that it had originated from the olfactory mucosal transplant. The patient had to undergo several resections due to regrowth of the mass and recurrent symptoms. To our knowledge this is only the third reported case of mass formation following s...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Bernatowicz, R., Sabharwal, N., Bennett, E., Ghodsi, A., Healy, A., Benzel, E., Stevens, G. Tags: RARE TUMORS Source Type: research
Rare-15. postoperative managements for adult pilocytic astrocytomas: efficacy of upfront radiation therapy and radiosurgery
This study was designed to evaluate the surgical outcome of adult PAs and to assess the efficacy of the upfront radiation therapy (RTx) and radiosurgery for residual tumors. A total of 63 consecutive adult patients with newly diagnosed PAs were retrospectively reviewed. Thirty-Two patients (50.8%) were male, and the median patient age was 28 years (range, 18 – 79 years). There were 21 supratentorial hemisphere tumors (33.3%), 11 supratentorial axial tumors (17.5%), and 31 infratentorial tumors (49.2%). Fifty-four patients (85.7%) underwent surgical resection and 9 patients (14.3%) did only biopsy....
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Kim, Y. H., Kim, J. H. Tags: RARE TUMORS Source Type: research
Rare-13. diffuse leptomeningeal glioneuronal tumor of ganglioglioma type with v600e mutation in the braf gene
A 2-year-old patient with hydrocephalus, VP shunt and a history of diffuse brain and spinal cord leptomeningeal lesions without definitive diagnosis after biopsy was referred to our institution. Contrast enhanced magnetic resonance imaging (MRI) of the brain and spinal cord was acquired. Brain MRI showed multiple enhancing supra and infratentorial leptomeningeal nodules with involvement of cranial nerves and basal cisterns. Spinal cord MRI showed pseudonodular meningeal thickening that enhanced after contrast administration and generated mass effect with compression of the spinal cord. The results of the cultures and immun...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Paday, M. E. Tags: RARE TUMORS Source Type: research
Rare-12. adult pilocytic astrocytoma: an institutional series and systematic literature review for extent of resection and recurrence
CONCLUSIONS:Though rare, adult pilocytic astrocytoma can present over a wide range of ages. GTR should be the goal of surgery when possible as tumor recurrence is more likely after STR. However, this is not always feasible. Prognosis is generally favorable and overall survival can be excellent. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Bond, K. M., Hughes, J. D. Tags: RARE TUMORS Source Type: research
Rare-11. perinatal diffuse infiltrating brainstem glioma: case report and review of the literature
We describe the clinical presentation of a male infant born at 38 4/7 weeks by normal spontaneous vaginal delivery to a 32 year-old female. Labor and delivery was induced due to maternal hypertension but was otherwise unremarkable. Apgar scores were 8 and 9 at 1 minute and 5 minutes, respectively. During initial breast feeding in the delivery room the patient began having expiratory grunting, sternal retractions, and desaturations. A MRI obtained on day 4 of life revealed an abnormal mass-like expansion involving the pons and medulla with T2-hyperintensity and no restricted diffusion or contrast enhancement. Fami...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Miller, C., Moertel, C. Tags: RARE TUMORS Source Type: research
Rare-08. clinical radiological and neuropathologic findings of posterior fossa gangliogliomas in childhood
CONCLUSIONS:Gangliogliomas of the infratentorial space and brainstem in pediatrics are not uncommon and may present with findings more typical of localized mass effect or infiltration of the brainstem, rather than with an epileptic process. This should be considered in the differential diagnosis of an exophytic brainstem lesion. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Geller, T., Guzman, M. Tags: RARE TUMORS Source Type: research
