Malignant Glioma Arising at the Site of an Excised Cerebellar Hemangioblastoma after Irradiation in a von Hippel-Lindau Disease Patient
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We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple hemangioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas.
Abstract
We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple hemangioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas.
참고문헌 (16)
- Rubinstein JL, "von Hippel-Lindau and the genetics of astrocytoma", J Natl Cancer Inst, 86, pp.142~143 (1994)
- Cornish D, "Metastatic islet cell tumor in von Hippel-Lindau disease", Am J Med, 77, pp.147~150 (1984)
- Ng HK, "Cerebellar astrocytoma associated with von Hippel-Lindau disease: case report with molecular findings", Br J Neurosurg, 13, pp.504~507 (1999)
- Ertas G, "Treatment of recurrent cerebellar hemangioblastoma with external radiotherapy in a patient with von Hippel-Lindau disease: a case report and review of the literature", J Neurooncol, 73, pp.273~275 (2005)
- Aksu G, "Cerebellar and multiple spinal hemangioblastomas and intraventricular meningioma managed with subtotal resection and external beam radiotherapy; report of a case with literature review", J BUON, 10, pp.405~409 (2005)
- Brat DJ, "Molecular genetic alterations in radiation-induced astrocytomas", Am J Pathol, 154, pp.1431~1438 (1999)
- Kanno H, "Somatic mutations of the von Hippel-Lindau tumor suppressor gene and loss of heterozygosity on chromosome 3p in human glial tumors", Cancer Res, 57, pp.1035~1038 (1997)
- Yang SY, "Radiation-induced cerebellar glioblastoma at the site of a treated medulloblastoma: case report", J Neurosurg, 102(4), pp.417~422 (2005)
- Cahan WG, "Sarcoma arising in irradiated bone: report of eleven cases. 1948", Cancer, 82, pp.8~34 (1998)
- Marus G, "Malignant glioma following radiotherapy for unrelated primary tumors", Cancer, 58, pp.886~894 (1986)
- Evans DG, "Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes", J Med Genet, 43, pp.289~294 (2006)
- Suzuki K, "Radiation-induced DNA damage and delayed induced genomic instability", Oncogene, 22, pp.6988~6993 (2003)
- Morgan WF, "Genomic instability induced by ionizing radiation", Radiat Res, 146, pp.247~258 (1996)
- Kaschten B, "Radiation-induced gliosarcoma, in Case report and review of the literature", J Neurosurg, 83, pp.154~162 (1995)
- Simmons NE, "Glioma occurrence after sellar irradiation: case report and review", Neurosurgery, 42, pp.172~178 (1998)
- Nakamizo A, "Evolution of malignant cerebellar astrocytoma at the site of a treated medulloblastoma: report of two cases", Acta Neurochir (Wien), 143, pp.697~700 (2001)