Rare-63. multicentric glioma in ollier disease: a case report and review of the literature
CONCLUSIONS:This case reinforces the association between OD and gliomagenesis. Additionally, multicentric glioma in OD is exceptional. The mutant isocitrate dehydrogenase (IDH) 1 pathway in this patient’s gliomas may represent the driving oncogenic pathway for his condition. Increasing evidence supports the concept of somatic heterozygous mutations of IDH1 and IDH2 playing a central role in tumorigenesis in OD, with this aberrant pathway contributing to the development of both mesenchymal and glial tumors. Further characterization of gliomas in OD is essential to our understanding of their pathogenesis, and may illum...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Harrison, R. A., Fuller, G. N., Prabhu, S. S., Slopis, J. M., Penas-Prado, M. Tags: RARE TUMORS Source Type: research
Rare-62. intravascular lymphoma with cns involvement successfully treated with r-codox-m/ivac
This report will describe clinical presentation, response to treatment, complications, and progression and overall survival of these patients. Their presentations highlight the diagnostic and therapeutic challenge in the management of this occult entity. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Schulte, J., Sawas, A. Tags: RARE TUMORS Source Type: research
Rare-61. clinical, radiologic, and pathologic review of astroblastoma: the md anderson experience
CONCLUSION:This case series describes the clinical, radiographic, and pathologic features seen with astroblastoma, contributing to our knowledge on this rare tumor and potentially facilitating diagnosis. Continued efforts to publish these cases will further our understanding of this unique tumor entity. Longer follow up is needed to better delineate the clinical course of these patients. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Harrison, R. A., Nam, J. Y., Fuller, G. N., Johnson, J. M., OBrien, B. J. Tags: RARE TUMORS Source Type: research
Rare-60. atypical rhabdoid teratoid tumor: a case in pregnancy and analysis of adult cases to date
We present the 48th case to date, the third such during pregnancy. The disease tends to follow a biphasic course i.e. if growth is not arrested following the first relapse/ progression, death is almost universal. While typically rapidly fatal, as in our case, long-term remission appears possible. Uni-variable Cox proportional-hazards models of adult cases showed significantly worse survival with: glial differentiation (HR 7.3, p<0.01), leptomeningeal dissemination (HR 4.3, p<0.01) - these were highly correlated (chi-squared <0.01) and pregnancy (HR 3.9, p=0.09). The finding that glial differentiation worsens outco...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Dardis, C., Milton, K. Tags: RARE TUMORS Source Type: research
Rare-57. giant intraosseous hemangioma of the petrous bone: a case report and literature review
CONCLUSION:Few cases have been reported in the literature of temporal hemangioma, herein we present the largest intraosseous hemangioma located in the apex of petrous bone of the skull base causing brainstem compression. This case illustrates the diversity of proliferating cell masses and suggests that the growth of these masses and size may not necessarily be localized to conventional areas. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Garces, J., Mathkour, M., Bui, C. J., Sulaiman, O. A. R., Ware, M. Tags: RARE TUMORS Source Type: research
Rare-55. langerhans cell histiocytosis (lch) associated with leptomeningeal acute myeloid leukemia (aml): a case report
We describe a 59-year-old patient with LCH of the right frontal sinus metastatic to regional lymph nodes and colon who presented with acute meningoencephalitis and was found to have AML isolated to the CNS. The patient initially presented with antibiotic-refractory recurrent sinusitis and was diagnosed with LCH following sinus biopsy. He was treated with 2 cycles of cytarabine (100 mg/m2 daily x 5 days) but progressed with metastatic disease to lymph nodes and colon (biopsy confirmed). He received 2 cycles of clofarabine with partial response on CT/PET, which was complicated by neutropenia (WBC 0.95, ANC 0.47). He was...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Trevino, C., Thomas, A., Wood, M., Landrigan, G., Lunde, J., Devitt, K., Singh, H. Tags: RARE TUMORS Source Type: research
Rare-54. first case of pleomorphic xanthoastrocytoma of the pineal region in a patient with neurofibromatosis type i
CONCLUSION:To the best of our knowledge, there are 8 prior reported cases of pineal region PXA with such lesions rarely occurring in individuals with NF1. These masses present similarly to dysplastic tumor types, and should be considered as a rare etiology of NF1 associated tectal masses. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Mathkour, M., Garces, J., Scullen, T., Boehm, L., Hanna, J., Sulaiman, O. A. R., Bui, C. J. Tags: RARE TUMORS Source Type: research
Rare-52. sacral lipomatosis of the nerve: a rare case presentation and literature review
CONCLUSION:LN of the spine should be considered exceedingly rare, with indeterminate diagnostic and prognostic significance. We report the second existing case of LN with spinal involvement, which was treated successfully with surgical resection. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Garces, J., Mathkour, M., Hanna, J., Bui, C. J., Sulaiman, O. A. R. Tags: RARE TUMORS Source Type: research
Rare-51. pleomorphic xanthoastrocytoma of the cerebellum in a patient with neurofibromatosis type i: a case report and literature review
CONCLUSION:To the best of our knowledge, there are 18 prior reported cases in the literature of infratentorial PXA and 4 reports of such cases occurring in individuals with NF1. These lesions present similarly to dysplastic tumor types, and should be considered as a rare etiology of NF1 associated cerebellar masses. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Garces, J., Mathkour, M., Scullen, T., Boehm, L., Hanna, J., Sulaiman, O. A. R., Ware, M. Tags: RARE TUMORS Source Type: research
Rare-50. spindle cell and epithelioid tumor of unknown origin in a pregnant female: a novel case presentation and literature review
CONCLUSION:In the world of modern medicine, genomics, and histochemistry, the ability to categorize even the most esoteric of tumors is rapidly advancing. Still, there occasionally exists the potential for neoplasms of unknown origins, particularly in histologically variable regions. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Mathkour, M., Garces, J. Tags: RARE TUMORS Source Type: research
Rare-49. defining relapse patterns in primary cns diffuse large b-cell lymphoma: a multicenter study
CONCLUSIONS:We found that late and systemic relapse were more common than previously reported. Only half of the relapses were detectable clinically by symptoms. These results support current consensus guidelines of clinical and radiographic surveillance up to 10 years after diagnosis. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Wang, N., Fadul, C., Davis, M., Nayak, L., Baehring, J., Batchelor, T. Tags: RARE TUMORS Source Type: research
Rare-48. papillary tumor of the pineal region in pediatric population associated with trisomy 21: first case report and literature review
CONCLUSION:PTPRs have been linked with a number of chromosomal and genetics abnormalities, which is pertinent in our patient’s case. Due to age and the developmental changes of the CNS in children, the application of adult treatment protocols may not be possible; however the principle of complete surgical resection is standardized across age groups. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Mathkour, M., Garces, J., Hanna, J., Cormier, I., Sulaiman, O. A. R., Bui, C. J. Tags: RARE TUMORS Source Type: research
Rare-45. progressive leptomeningeal dissemination of a dnt after west nile virus meningoencephalitis
DISCUSSION:A disseminated DNT has rarely been reported. The development of this aggressive course following West Nile virus infection was noted. It has been established that West Nile virus causes S100B pathway activation in astrocytes, which is one of the oncogenic pathways that is activated in dysembryoplastic neuroepithelial tumors. We propose this additive effect may have led to an unusually aggressive behavior in a low grade tumor. Additional studies are needed. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Anderson, M., Vig, P., Leis, A. Tags: RARE TUMORS Source Type: research
Rare-44. one year with optune(r) in routine use: first report on clinical experiences in germany
Tumor Treating Fields (TTFields) are low intensity, intermediate frequency alternating electric fields. TTFields are delivered through 4 transducer arrays that are placed directly on the scalp to target the tumor. The delivery System is called Optune. Optune is an established, non-invasive anti mitotic therapy for the treatment of patients with newly diagnosed or recurrent glioblastoma multiforme (GBM). This is the first report on clinical experiences with Optune in Germany (N=30 patients). All patients received a prescription in the Charité Berlin, one of over 60 certified Optune-centers in Germany. We treated newl...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Misch, M., Onken, J. Tags: RARE TUMORS Source Type: research
Rare-43. search for druggable targets in primary nervous system melanotic tumors: melanocytomas, melanotic schwannomas, primary melanomas
Due to the extreme rarity of primary nervous system melanotic tumors, relatively limited studies have been conducted attempting to identify distinguishing mutations and/or druggable targets. Melanocytomas share GNAQ mutations with uveal melanoma (Brain Pathol. 25:202-8, 2015; Acta Neuropathol 119:317-323, 2010); clinical trials in uveal melanomas have shown that MEK inhibition may result in clinical benefit in tumors with these mutations. BRAF V600E mutations (paralleled by BRAF VE1 immunohistochemistry) are targetable by vemurafenib, but large numbers of primary nervous system melanomas have not been assessed. Adding to t...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Lillehei, K., Davies, K., Aisner, D., Kleinschmidt-DeMasters, B. K. Tags: RARE TUMORS Source Type: research
