Rare-42. leptomeningeal dissemination in malignant glioma: a case series
We report a case series of 11 patients over 18 months at a single institution. Demographic, radiologic, and treatment data from the medical record are reported, and molecular characteristics of the primary tumor identified where available. Demographic and clinical features were strikingly variable. Time from initial diagnosis to LM diagnosis varied from synchronous to 30 years. Time to LM development from initial diagnosis was significantly longer for those whose tumors were initially low grade. There was no predilection for tumor location and all but the two patients (both diagnosed synchronously) received radia...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Amidei, C., Nicholas, M., Sica, R., Kim, S., Arzbaecher, J., Mohapatra, G. Tags: RARE TUMORS Source Type: research
Rare-40. bing-neel syndrome: diagnostic & therapeutic issues
We present two cases that present both diagnostic and therapeutic issues in patients with known Waldenstrom’s Macroglobulinaemia (WM), also known as Lymphoplasmocytic Lymphoma. CASE 1: This patient had a past history of breast carcinoma and had long-standing WM found asymptomatically. She had completed chemotherapy for WM with CVP and developed numbness and gait difficulties initially attributed to vincristine. She was allergic to Rituximab. She had features of a myelopathy and MRI showed diffuse myelopathy with enhancement and oedema with leptomeningeal enhancement. ON CSF flow cytometry the WM clone noted previousl...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Cher, L., Griffiths, J. Tags: RARE TUMORS Source Type: research
Rare-39. intravascular lymphoma affecting the central nervous system: features and outcomes in a case series of the primary cns lymphoma collaborative group (ipcg)
We present a retrospective case series of 72 adults with IVL from 16 cancer centers in 6 countries. IVL was present in the CNS in 55 cases, with a mean age of 61.6 years and more than 90% of patients having neurological deficits at the time of diagnosis. There was a mean diagnostic delay of 20 weeks after presentation of stroke-like symptoms or myelopathy. Eighty-five percent had a poor functional status with an Eastern Cooperative Oncology Group (ECOG) performance status ≥2 with another eight percent diagnosed post-mortem. Serum LDH was elevated in 87% of available patients with no correlation with treatment outco...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Zukas, A., Bennani, N., Chou, C., Johnston, P., ONeill, B. P., Nijland, M., Batchelor, T., Nayak, L., Mrugala, M., Low, J., Omuro, A., Ferreri, A., Nishikawa, R., Mishima, K., Fox, C., Wilson, W., Houillier, C., Chamberlain, M., Schiff, D. Tags: RARE TUMORS Source Type: research
Rare-38. novel targets in ependymal tumors
CONCLUSIONS:If predicated on tumor expression, our data suggest that therapeutics directed toward ERCC1, ALK, PTEN, TOPO1, EGFR, TUBB3, and RRM1 could be considered for conventional treatment-refractory ependymal tumor patients. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Sanai, N., Xiu, J., Kim, L., Kesari, S., Zhou, S., Groot, J. d., Heimberger, A. Tags: RARE TUMORS Source Type: research
Rare-37. surgical management of incidental diffusely infiltrating gliomas: a single institutional experience
CONCLUSION:This retrospective cohort of incidentally discovered surgically managed DIGs demonstrates that the majority of lesions are IDH mutated and low grade. Incidentally discovered lesions are usually of small size and can be managed with low surgical risk. Ongoing follow-up will be required in order to determine progression-free and overall survival in this group of patients. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Opoku-Darko, M., Kelly, J. Tags: RARE TUMORS Source Type: research
Rare-36. pleomorphic xanthoastrocytoma with anaplastic features: a case report
Pleomorphic xanthoastrocytoma (PXA) is a rare WHO Grade II neoplasm that often presents with seizures in children and young adults and has a supratentorial, typically temporal, predilection. The latest WHO classification includes a rare subtype with anaplastic features. These tumors demonstrate high mitotic activity of > 5 mitosis per 10 high powered field, focal positivity of CD-34, synaptophysin, MIB-1 labeling index, and P53 reactivity. Histologically, they exhibit spindle cells admixed with multinucleated giant cells showing lipidized vacuolated cytoplasm, intranuclear cytoplasmic inclusion, and eosinophilic granula...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Iqbal, M., Turner, S. Tags: RARE TUMORS Source Type: research
Rare-35. patient characteristics, patterns of care and survival outcome in patients with pediatric extra ventricular neurocytoma: an individual patient data analysis
CONCLUSION:Pediatric EVN is a very rare tumor. Most common site of tumor is supra tentotial. The patients are generally treated with surgery alone. The outcome in these patients is very good with a median PFS of 15 years. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Benson, R., Mallick, S., Melgandi, W., Rath, G. K. Tags: RARE TUMORS Source Type: research
RARE-34. DIAGNOSIS AND MOLECULAR CHARACTERIZATION OF CSF-CTCs IN LEPTOMENINGEAL DISEASE (LMDz) FROM UVEAL MELANOMA (UM)
CONCLUSIONS & FUTURE DIRECTIONS:It is possible to make a diagnosis and molecular characterization of LMDz when conventional methods fail using CSF-CTCs. A focused mutational analysis for potential therapeutically relevant targets can be used to guide treatment. The relationship of this selected CSF-CTC marker to the population of CSF-CTCs and therapeutic decisions are being clarified. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Fedorenko, I., Evernden, B., Kenchappa, R., Sahebjam, S., Ryzhova, E., Puskas, J., Magliocco, A., Etame, A. B., Tran, N. D., Harbour, W., Smalley, K., Forsyth, P. Tags: RARE TUMORS Source Type: research
Rare-33. intracerebral hemorrhage as rare complication of intravascular lymphoma
Intravascular lymphoma (IVL) is an aggressive multifocal large cell lymphoma, characterized by neoplastic proliferation and vascular occlusion within the lumina of arterioles, capillaries and venules with a predilection for the central nervous system and skin. The clinical presentation is frequently non-specific and may consist of changes in mental status, focal neurological deficits, seizures, fevers of unknown origin and skin changes. Due to the rarity and absence of specific diagnostic procedures apart from biopsy, diagnosis is difficult and often post-mortem. Neuroimaging findings vary widely, ranging from normal to di...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Czap, A., Mantica, M. Tags: RARE TUMORS Source Type: research
Rare-32. clinical outcomes in patients with central neurocytoma
CONCLUSIONS:Central neurocytomas are slow-growing tumors with low rates of recurrence. Genomic characterization of these tumors is needed to identify the molecular features that correlate with recurrence. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Chukwueke, U., Gill, C., Batchelor, T., Brastianos, P. Tags: RARE TUMORS Source Type: research
Rare-31. outcome and prognostic features for pediatric pineal gland tumors
Pineal gland tumor is a rare type of brain neoplasm, which typically occurs in children and young adults. However, systematic study of pineal gland tumor has been difficult given its low incidence. Using NCI Surveillance, Epidemiology, and End Results registry (1973-2012), we identified 593 patients of age 18 or younger with the diagnosis of malignant tumor located in pineal gland. The impact of radiotherapy (RT) and surgery on overall survival (OS) was determined using Log-rank test and multivariate Cox proportional hazard modeling. The major histological categories were germ cell tumor (GCT, 64.9%) and pineal parenchymal...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Yang, K., Kim, L., Recinos, V., Suh, J., Chao, S., Murphy, E. Tags: RARE TUMORS Source Type: research
Rare-30. clinical and molecular characterization of glioneuronal tumors
CONCLUSIONS:Glioneuronal tumors are a lower grade tumor type which tends to affect younger age groups and take on a less aggressive course than tumors of the glioma lineage, with low rates of proliferation on histologic analysis. A subset of these tumors are progressive, however, and further characterization at the molecular level with be helpful to better understanding the behavior. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Miller, J., Gill, C. Tags: RARE TUMORS Source Type: research
Rare-29. high-dose methotrexate (hd mtx)-based treatments of primary cns lymphoma: optimal dose revisited - a retrospective analysis of outcomes in tertiary medical center
CONCLUSION:Treatment with HD MTX at the dose of 8 g/m2improved response rates, OS and PFS in patients with PCNSL after 12, 36 and 60 months, compared with treatment at lower MTX doses. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Bainhoren, O. A., Asna, N., Mendel, L., Blumenthal, D. T., Bokstein, F. Tags: RARE TUMORS Source Type: research
Rare-28. clinical features of cerebellar glioblastoma
CONCLUSIONS:Our data suggested that the cerebellar GBMs are rapid onset of symptoms and the poor prognosis because of their higher proliferative capacities. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Tamai, S., Miyashita, K. Tags: RARE TUMORS Source Type: research
Rare-27. extracranial metastatic glioblastoma presenting with lhermittes phenomenon, torticollis and quadriparesis
CONCLUSION:Subacute onset of cervicalgia and torticollis with Lhermitte’s phenomenon in a patient with glioblastoma may represent epidural cervical spine metastases. Further studies are needed to clarify the prognostic and treatment differences associated with activating mutations in the BRAF kinase gene (V600E). (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Ruff, M., Pittock, S. Tags: RARE TUMORS Source Type: research
