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Infratentorial craniospinal irradiation for von Hippel-Lindau: a retrospective study supporting a new treatment for patients with CNS hemangioblastomas.
Neuro Oncol. 2011 Sep; 13(9):1030-6.NO

Abstract

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 cm(3). At a mean follow-up of 73.8 months, mean post-ICSRT tumor volume was 6.87 cm(3), 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.

Authors+Show Affiliations

National Institutes of Health, National Cancer Institute, Radiation Oncology Branch, Bethesda, MD 20892, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Intramural

Language

eng

PubMed ID

21798886

Citation

Simone, Charles B., et al. "Infratentorial Craniospinal Irradiation for Von Hippel-Lindau: a Retrospective Study Supporting a New Treatment for Patients With CNS Hemangioblastomas." Neuro-oncology, vol. 13, no. 9, 2011, pp. 1030-6.
Simone CB, Lonser RR, Ondos J, et al. Infratentorial craniospinal irradiation for von Hippel-Lindau: a retrospective study supporting a new treatment for patients with CNS hemangioblastomas. Neuro Oncol. 2011;13(9):1030-6.
Simone, C. B., Lonser, R. R., Ondos, J., Oldfield, E. H., Camphausen, K., & Simone, N. L. (2011). Infratentorial craniospinal irradiation for von Hippel-Lindau: a retrospective study supporting a new treatment for patients with CNS hemangioblastomas. Neuro-oncology, 13(9), 1030-6. https://doi.org/10.1093/neuonc/nor085
Simone CB, et al. Infratentorial Craniospinal Irradiation for Von Hippel-Lindau: a Retrospective Study Supporting a New Treatment for Patients With CNS Hemangioblastomas. Neuro Oncol. 2011;13(9):1030-6. PubMed PMID: 21798886.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Infratentorial craniospinal irradiation for von Hippel-Lindau: a retrospective study supporting a new treatment for patients with CNS hemangioblastomas. AU - Simone,Charles B,2nd AU - Lonser,Russell R, AU - Ondos,John, AU - Oldfield,Edward H, AU - Camphausen,Kevin, AU - Simone,Nicole L, Y1 - 2011/07/28/ PY - 2011/7/30/entrez PY - 2011/7/30/pubmed PY - 2011/12/13/medline SP - 1030 EP - 6 JF - Neuro-oncology JO - Neuro Oncol VL - 13 IS - 9 N2 - 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 cm(3). At a mean follow-up of 73.8 months, mean post-ICSRT tumor volume was 6.87 cm(3), 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. SN - 1523-5866 UR - https://www.unboundmedicine.com/medline/citation/21798886/Infratentorial_craniospinal_irradiation_for_von_Hippel_Lindau:_a_retrospective_study_supporting_a_new_treatment_for_patients_with_CNS_hemangioblastomas_ L2 - https://academic.oup.com/neuro-oncology/article-lookup/doi/10.1093/neuonc/nor085 DB - PRIME DP - Unbound Medicine ER -