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Robert E Isaacs - One of the best experts on this subject based on the ideXlab platform.
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn A Hardin, Carlos A Bagley, Robert E IsaacsAbstract:BACKGROUND: Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. OBJECTIVE: To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. METHODS: A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. RESULTS: Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P < .0001) were all predictive of functional neurological outcomes. CONCLUSION: Tumor histology is the most important predictor of neurological outcome after surgical resection because it predicts resectability and recurrence.
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn Hardin, Carlos A Bagley, Robert E IsaacsAbstract:Abstract Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn Hardin, Carlos A Bagley, Robert E IsaacsAbstract:Abstract Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P
Jeanmichel Vignaud - One of the best experts on this subject based on the ideXlab platform.
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immunohistochemical markers to distinguish between Hemangioblastoma and metastatic clear cell renal cell carcinoma in the brain utility of aquaporin1 combined with cytokeratin ae1 ae3 immunostaining
The American Journal of Surgical Pathology, 2008Co-Authors: Nicolas Weinbreck, Beatrice Marie, Aude Bressenot, Karine Montagne, Antony Joud, Cedric Baumann, O Klein, Jeanmichel VignaudAbstract:Distinguishing Hemangioblastomas from metastatic clear-cell renal cell carcinomas (CCRCCs) in the brain is a diagnostic challenge owing to similar clinical and morphologic presentations. Inhibin-alpha and aquaporin1 were shown as positive markers of Hemangioblastoma, but are not totally reliable distinguishing Hemangioblastoma from metastatic CCRCC. This study shows that the diagnosis can be achieved using a combination of markers. To identify the panel of markers useful for this differential, 67 Hemangioblastomas and 34 metastatic CCRCCs were analyzed using a panel of antibodies including aquaporin1, inhibin-alpha, D2-40, cytokeratin AE1/AE3, epithelial membrane antigen, and CD10. The study confirms the usefulness of aquaporin1 (97% sensitivity, 83% specificity) and inhibin-alpha (88% sensitivity, 79% specificity) as positive markers of Hemangioblastoma and shows that aquaporin1 is a superior positive marker versus inhibin-alpha for the differential. Positivity of tumor cells with cytokeratin AE1/AE3 is the signature of a metastatic CCRCC (100% specificity, 88% sensitivity) and CD10 expression as well (100% specificity, 79% sensitivity). The combined use of aquaporin1 and AE1/AE3 yields a high degree of sensitivity and specificity to differentiate between Hemangioblastoma and metastatic CCRCC. All tumors but one aquaporin1 positive and cytokeratin AE1/AE3 negative (65/66) correspond to Hemangioblastomas (97% sensitivity, 97% specificity, 98.5% diagnostic positive predictive value). Tumors with the opposite profile, aquaporin1 negative, and cytokeratin AE1/AE3 positive, (25/25), correspond to metastatic CCRCC (74% sensitivity, 100% specificity, 100% diagnostic positive predictive value). In summary, aquaporin1 is the most sensitive positive marker of Hemangioblastoma. Despite its moderate specificity, when used in combination with epithelial marker AE1/AE3, it allowed to reliably distinguish Hemangioblastoma from metastatic CCRCC.
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immunohistochemical markers to distinguish between Hemangioblastoma and metastatic clear cell renal cell carcinoma in the brain utility of aquaporin1 combined with cytokeratin ae1 ae3 immunostaining
The American Journal of Surgical Pathology, 2008Co-Authors: Nicolas Weinbreck, Beatrice Marie, Aude Bressenot, Karine Montagne, Antony Joud, Cedric Baumann, O Klein, Jeanmichel VignaudAbstract:Distinguishing Hemangioblastomas from metastatic clear-cell renal cell carcinomas (CCRCCs) in the brain is a diagnostic challenge owing to similar clinical and morphologic presentations. Inhibin-α and aquaporin1 were shown as positive markers of Hemangioblastoma, but are not totally reliable disting
Edward H Oldfield - One of the best experts on this subject based on the ideXlab platform.
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infratentorial craniospinal irradiation for von hippel lindau a retrospective study supporting a new treatment for patients with cns Hemangioblastomas
Neuro-oncology, 2011Co-Authors: Charles B Simone, Edward H Oldfield, Russell R Lonser, John Ondos, Kevin Camphausen, Nicole L SimoneAbstract:Patients with von Hippel–Lindau (VHL) syndrome with diffuse CNS Hemangioblastomas have morbidity related to their disease and require a lifetime of surgical resections. Ninety-seven percent of tumors progress, and 5-year surgery rates are 20%–60%. Stereotactic radiosurgery and fractionated radiotherapy have had limited success. For the first time, we have used infratentorial craniospinal radiation therapy (ICSRT) for VHL patients with CNS Hemangioblastomas. Consecutive VHL patients treated at the National Institutes of Health with radiographic evidence of Hemangioblastomas were included if they received ICSRT. Patients underwent neurologic examinations and imaging at 3- to 12-month intervals. Seven patients with 84 Hemangioblastomas met eligibility criteria. ICSRT was commonly administered to 43.2 Gy in 24 fractions. Mean pre-ICSRT tumor volume was 5.48 cm3. At a mean follow-up of 73.8 months, mean post-ICSRT tumor volume was 6.87 cm3, and 91 tumors were identified. Complete radiographic resolution was achieved in 17.9% of lesions. Although many patients were no longer optimal surgical candidates, only 4 surgeries were needed for symptomatic lesions after ICSRT, compared with 33 prior. Acute toxicity was mild and no patient developed grade ≥1 late spinal cord toxicity according to the criteria of the Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer, despite the high dose that the entire spinal cord received. Clinical and radiographic stability or resolution was demonstrated in the majority of tumors. Tumor growth rate in this study was less than reported in natural history studies, and the rate of surgical intervention was reduced. ICSRT was well tolerated, can decrease Hemangioblastoma growth rate, and is a potential therapeutic option for VHL patients that warrants further investigation.
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long term stability after multilevel cervical laminectomy for spinal cord tumor resection in von hippel lindau disease
Journal of Neurosurgery, 2011Co-Authors: Ashok R Asthagiri, Martin Baggenstos, Gautam U. Mehta, Edward H Oldfield, John A. Butman, Russell R LonserAbstract:Object Despite the frequent multiplicity and development of new spinal cord Hemangioblastomas that require multiple resections in patients with von Hippel-Lindau (VHL) disease, the long-term effects of spinal surgery on spinal column stability in this neoplasia disorder are not known. To determine the effect of multilevel cervical laminectomy for spinal cord tumor resection in VHL, the authors analyzed long-term clinical and radiographic outcomes. Methods The authors included consecutive patients enrolled in a prospective VHL disease natural history study who underwent cervical laminectomy(s) for spinal cord Hemangioblastoma resection. Serial clinical examinations, neck disability indices, and radiographs (static and dynamic), as well as operative records, were analyzed. Results Twenty-five adult patients (16 female, 9 male) with VHL disease underwent 34 operations (mean 1.4 ± 0.7 [± SD]/patient) for the resection of cervical spinal cord Hemangioblastomas (mean number of lamina removed/surgery 3.0 ± 1.3)....
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pituitary stalk Hemangioblastomas in von hippel lindau disease
Journal of Neurosurgery, 2009Co-Authors: Russell R Lonser, John A. Butman, Ruwan Kiringoda, Debbie K Song, Edward H OldfieldAbstract:Object Pituitary stalk Hemangioblastomas are rare, and information on them is limited to a small number of case reports. To gain insight into the incidence, clinical effects, and management of pituitary stalk Hemangioblastomas, the authors analyzed a series of patients with von Hippel–Lindau (VHL) disease. Methods Patients with VHL disease who were enrolled in a prospective National Institutes of Health natural history study were included. Clinical, imaging, and laboratory findings were analyzed. Results Two hundred fifty patients were included in the study (120 male and 130 female patients). In 8 patients (3%), 8 pituitary stalk Hemangioblastomas were identified on MR imaging. This anatomical location was the most common supratentorial site for these lesions; 29% of all supratentorial Hemangioblastomas were found there. The mean (± standard deviation) pituitary stalk Hemangioblastoma volume was 0.5 ± 0.9 cm3 (range 0.08–2.8 cm3). Results of endocrine laboratory profiles were normal in all patients. All p...
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surgical management of brainstem Hemangioblastomas in patients with von hippel lindau disease
Journal of Neurosurgery, 2008Co-Authors: Robe J Weil, Russell R Lonse, Hetty L Devroom, Joh E Wanebo, Edward H OldfieldAbstract:Object Despite the frequency of cerebellar Hemangioblastomas in von Hippel–Lindau (VHL) disease, their optimum contemporary management has not been defined, and is made complex because of the multiple, progressive, and protean nature of the tumors found in patients with this disorder. To examine modern management and outcomes of cerebellar Hemangioblastomas in VHL disease, the authors reviewed findings in patients with this disease who underwent resection of cerebellar Hemangioblastomas. Methods Consecutive patients with VHL disease who underwent surgery for cerebellar Hemangioblastoma(s) at the National Institutes of Health were included. Eighty consecutive patients (44 female and 36 male patients) underwent 126 operations for removal of 164 cerebellar Hemangioblastomas (age at surgery 37.8 ± 10.3 years, follow-up duration 96.0 ± 60.3 months). Serial clinical examinations, imaging studies, and operative records were analyzed. Results Symptoms and signs included headache (94 operations; 75%), ataxia (55%)...
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clinical and molecular analysis of disseminated Hemangioblastomatosis of the central nervous system in patients without von hippel lindau disease report of four cases
Journal of Neurosurgery, 2002Co-Authors: Robe J Weil, Zhengping Zhuang, Svetlana Pack, Alexander O Vortmeyer, Nicholas Theodore, Robert K Erickson, Edward H OldfieldAbstract:✓ Hemangioblastomas of the central nervous system (CNS) may occur sporadically or in association with von Hippel—Lindau (VHL) syndrome. The authors present four patients with no family history or clinical evidence of VHL syndrome in whom extensive, progressive, en plaque coating of the brainstem and spinal cord with Hemangioblastomas developed 1 to 8 years after complete resection of a solitary cerebellar Hemangioblastoma. Analysis included detailed physical, biochemical, radiological, and pathological examinations in all four patients, combined with family pedigree analysis. In addition, a detailed investigation of the VHL gene was undertaken. Allelic loss, comparative genomic hybridization (CGH), single-stranded conformational polymorphism screening, CpG island methylation status, and X chromosome inactivation clonality analyses were performed. Although there was no evidence of germline alterations in the VHL gene on clinical and radiological examination or in the family history (all four patients) or a...
Isaac O Karikari - One of the best experts on this subject based on the ideXlab platform.
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn A Hardin, Carlos A Bagley, Robert E IsaacsAbstract:BACKGROUND: Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. OBJECTIVE: To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. METHODS: A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. RESULTS: Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P < .0001) were all predictive of functional neurological outcomes. CONCLUSION: Tumor histology is the most important predictor of neurological outcome after surgical resection because it predicts resectability and recurrence.
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn Hardin, Carlos A Bagley, Robert E IsaacsAbstract:Abstract Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P
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impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors a single center experience with 102 patients
Neurosurgery, 2011Co-Authors: Isaac O Karikari, Shahid M. Nimjee, Tiffany R. Hodges, Erin Cutrell, Betsy D. Hughes, Ciaran J. Powers, Ankit I. Mehta, Carolyn Hardin, Carlos A Bagley, Robert E IsaacsAbstract:Abstract Surgical outcomes for intramedullary spinal cord tumors are affected by many variables including tumor histology and preoperative neurological function. To analyze the impact of tumor histology on neurological outcome in primary intramedullary spinal cord tumors. A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009. Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 Hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 Hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), we observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in Hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a Hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a Hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P
Russell R Lonser - One of the best experts on this subject based on the ideXlab platform.
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tumor derived vasculogenesis in von hippel lindau disease associated tumors
Scientific Reports, 2015Co-Authors: Zhengping Zhuang, Jason M Frerich, Kristin Huntoon, Chunzhang Yang, Marsha J Merrill, Ziedulla Abdullaev, Svetlana Pack, Sharon B Shively, Gordon Stamp, Russell R LonserAbstract:von Hippel-Lindau disease (VHL) patients develop highly vascular tumors, including central nervous system Hemangioblastomas. It has been hypothesized that the vascular nature of these tumors is the product of reactive angiogenesis. However, recent data indicate that VHL-associated Hemangioblastoma neoplastic cells originate from embryologically-arrested hemangioblasts capable of blood and endothelial cell differentiation. To determine the origin of tumor vasculature in VHL-associated Hemangioblastomas, we analyzed the vascular elements in tumors from VHL patients. We demonstrate that isolated vascular structures and blood vessels within VHL-associated Hemangioblastomas are a result of tumor-derived vasculogenesis. Further, similar to Hemangioblastomas, we demonstrate that other VHL-associated lesions possess vascular tissue of tumor origin and that tumor-derived endothelial cells emerge within implanted VHL deficient UMRC6 RCC murine xenografts. These findings further establish the embryologic, developmentally arrested, hemangioblast as the tumor cell of origin for VHL-associated Hemangioblastomas and indicate that it is also the progenitor cell for other VHL-associated tumors.
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infratentorial craniospinal irradiation for von hippel lindau a retrospective study supporting a new treatment for patients with cns Hemangioblastomas
Neuro-oncology, 2011Co-Authors: Charles B Simone, Edward H Oldfield, Russell R Lonser, John Ondos, Kevin Camphausen, Nicole L SimoneAbstract:Patients with von Hippel–Lindau (VHL) syndrome with diffuse CNS Hemangioblastomas have morbidity related to their disease and require a lifetime of surgical resections. Ninety-seven percent of tumors progress, and 5-year surgery rates are 20%–60%. Stereotactic radiosurgery and fractionated radiotherapy have had limited success. For the first time, we have used infratentorial craniospinal radiation therapy (ICSRT) for VHL patients with CNS Hemangioblastomas. Consecutive VHL patients treated at the National Institutes of Health with radiographic evidence of Hemangioblastomas were included if they received ICSRT. Patients underwent neurologic examinations and imaging at 3- to 12-month intervals. Seven patients with 84 Hemangioblastomas met eligibility criteria. ICSRT was commonly administered to 43.2 Gy in 24 fractions. Mean pre-ICSRT tumor volume was 5.48 cm3. At a mean follow-up of 73.8 months, mean post-ICSRT tumor volume was 6.87 cm3, and 91 tumors were identified. Complete radiographic resolution was achieved in 17.9% of lesions. Although many patients were no longer optimal surgical candidates, only 4 surgeries were needed for symptomatic lesions after ICSRT, compared with 33 prior. Acute toxicity was mild and no patient developed grade ≥1 late spinal cord toxicity according to the criteria of the Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer, despite the high dose that the entire spinal cord received. Clinical and radiographic stability or resolution was demonstrated in the majority of tumors. Tumor growth rate in this study was less than reported in natural history studies, and the rate of surgical intervention was reduced. ICSRT was well tolerated, can decrease Hemangioblastoma growth rate, and is a potential therapeutic option for VHL patients that warrants further investigation.
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long term stability after multilevel cervical laminectomy for spinal cord tumor resection in von hippel lindau disease
Journal of Neurosurgery, 2011Co-Authors: Ashok R Asthagiri, Martin Baggenstos, Gautam U. Mehta, Edward H Oldfield, John A. Butman, Russell R LonserAbstract:Object Despite the frequent multiplicity and development of new spinal cord Hemangioblastomas that require multiple resections in patients with von Hippel-Lindau (VHL) disease, the long-term effects of spinal surgery on spinal column stability in this neoplasia disorder are not known. To determine the effect of multilevel cervical laminectomy for spinal cord tumor resection in VHL, the authors analyzed long-term clinical and radiographic outcomes. Methods The authors included consecutive patients enrolled in a prospective VHL disease natural history study who underwent cervical laminectomy(s) for spinal cord Hemangioblastoma resection. Serial clinical examinations, neck disability indices, and radiographs (static and dynamic), as well as operative records, were analyzed. Results Twenty-five adult patients (16 female, 9 male) with VHL disease underwent 34 operations (mean 1.4 ± 0.7 [± SD]/patient) for the resection of cervical spinal cord Hemangioblastomas (mean number of lamina removed/surgery 3.0 ± 1.3)....
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pituitary stalk Hemangioblastomas in von hippel lindau disease
Journal of Neurosurgery, 2009Co-Authors: Russell R Lonser, John A. Butman, Ruwan Kiringoda, Debbie K Song, Edward H OldfieldAbstract:Object Pituitary stalk Hemangioblastomas are rare, and information on them is limited to a small number of case reports. To gain insight into the incidence, clinical effects, and management of pituitary stalk Hemangioblastomas, the authors analyzed a series of patients with von Hippel–Lindau (VHL) disease. Methods Patients with VHL disease who were enrolled in a prospective National Institutes of Health natural history study were included. Clinical, imaging, and laboratory findings were analyzed. Results Two hundred fifty patients were included in the study (120 male and 130 female patients). In 8 patients (3%), 8 pituitary stalk Hemangioblastomas were identified on MR imaging. This anatomical location was the most common supratentorial site for these lesions; 29% of all supratentorial Hemangioblastomas were found there. The mean (± standard deviation) pituitary stalk Hemangioblastoma volume was 0.5 ± 0.9 cm3 (range 0.08–2.8 cm3). Results of endocrine laboratory profiles were normal in all patients. All p...