Intracranial large vessel vasculopathy and anaplastic meningioma 19 years after cranial irradiation for acute lymphoblastic leukaemia.
ABSTRACT A child was diagnosed in 1969 as having acute lymphoblastic leukaemia (ALL) and received chemotherapy. On bone marrow relapse in 1973, he was treated with cranial irradiation (20 Gy) in addition to chemotherapy. He continues in complete remission 19 years after his relapse. At age 25 years, he presented with headaches and left hemiparesis. Computerised tomograph demonstrated a large, enhancing right-sided intracranial tumour. Angiography was performed and showed the right internal carotid artery was occluded. Most of the right hemisphere was supplied from the external carotid via the middle meningeal artery. The left posterior cerebral artery and the left anterior cerebral artery were absent presumably as a result of radiation-induced arteritis. A resection of an anaplastic meningioma arising from the right sphenoidal ridge was achieved. There was a rapid improvement in function and he returned to work. Vasculopathy of the large intracranial arteries has been described after high dose radiation. It may occur as in this case after moderate dose radiation. There is a correlation with meningioma. There is a possibility that large artery vasculopathy will be present in a proportion of patients irradiated for ALL. The long lag time between irradiation and the development of meningioma may mean that, as survivors of childhood ALL enter their third decade since cure, this tumour may be seen increasingly.
- [Show abstract] [Hide abstract]
ABSTRACT: Radiation-induced glioma is a rare but serious complication of radiotherapy. Underlying radiation-induced mutations in oncogenes or tumor suppressor genes have not previously been described. A 16-year-old female patient developed a glioblastoma in the right frontal lobe 10 years after treatment of a suprasellar germ cell tumor with 50 Gy ionizing radiation. The glioblastoma was undetectable on a high-resolution magnetic resonance image obtained 3 months before diagnosis. A p53 functional assay was used to examine the transcriptional competence of the p53 tumor suppressor gene. This assay scores the content of mutant p53 alleles in tumor and blood samples quantitatively as a percentage of red yeast colonies. The glioblastoma contained 95% mutant p53 alleles, whereas blood from the patient and her parents contained only normal background levels of red colonies. Sequencing revealed that the mutation in the tumor was a 3-base pair deletion affecting codons 238 and 239. Intragenic deletion within the p53 deoxyribonucleic acid binding domain is uncommon in sporadic tumors but would be entirely consistent with misrepair of a radiation-induced double-strand deoxyribonucleic acid break in this case. This is the first case in which a causative underlying genetic event has been identified in a radiation-induced glioblastoma. We infer that mutation of one p53 allele occurred at the time of radiotherapy, and the sudden appearance of the tumor 10 years later occurred after loss of the remaining wild-type allele and/or other genetic alterations, such as chromosome 10 loss and epidermal growth factor receptor gene amplification.Neurosurgery 03/1997; 40(2):393-6. DOI:10.1097/00006123-199702000-00034 · 3.03 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Therapeutic advances in the treatment of pediatric neoplasms have improved the prognosis but have also increased the risk of developing rare second malignant neoplasms (SMNs). Primary neoplasms that are often associated with SMNs include lymphoma, retinoblastoma, medulloblastoma, neuroblastoma, and leukemia. The most common SMNs are central nervous system (CNS) tumors, sarcomas, thyroid and parotid gland carcinomas, and leukemia, particularly acute myeloblastic leukemia. Genetic predisposition, chemotherapy, and especially radiation therapy are implicated as pathogenic factors in SMN. All survivors of childhood cancer should have lifelong follow-up, preferably with magnetic resonance imaging, which does not require ionizing radiation and provides greater anatomic detail and resolution in the head and neck region and the CNS. A new or progressive lesion may represent recurrence of the primitive neoplastic process, late radiation injury, or, more infrequently, an SMN. Differential diagnosis can be very difficult, and outcome is often fatal. Treatment protocols should be modified to reduce the risk for SMN without compromising the effectiveness of initial therapy. Clinicians should individualize treatment for patients who are genetically predisposed to SMN. In addition, radiologists should be familiar with the long-term consequences of antineoplastic therapy to facilitate diagnosis and anticipate adverse outcomes.Radiographics 09/2003; 23(5):1155-72. DOI:10.1148/rg.235035040 · 2.73 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Radiation-induced cerebrovascular injury is a well-known phenomenon. We analyze reported cases of delayed radiation-induced cerebrovasculopathy that present as moyamoya syndrome and/or intracerebral hemorrhage and to statistically analyze the relationship between radiation dose and the interval period between radiation and the presentation of cerebrovasculopathy. Patients ages <21 years at the time of radiation were included in analysis. A review of previous publications yielded 77 cases of delayed radiation-induced cerebrovasculopathy consisting of 45 cases of moyamoya syndrome, 30 cases of intracerebral hemorrhage, and two cases of both. The median age at radiation was 4.8 years, with a range of 0.5-20 years. Approximately, 75% of these patients received radiation at the age of <9 years. The median interval period for moyamoya cases was 3.3 years (range: 0.3-20; P < 0.001), whereas the median interval period from radiation to presentation for intracerebral hemorrhage cases was 7.5 years (range: 0.8-27). There was significant association between radiation dose and interval from radiation to moyamoya syndrome (P < 0.001), whereas for patients with intracerebral hemorrhage, the association was insignificant (P = 0.31). Pediatric patients who presented with moyamoya generally presented earlier than those who presented with intracerebral hemorrhage, suggesting that moyamoya may be a factor that predisposes the patient to intracerebral hemorrhage. In patients who presented with moyamoya, there was a statistically significant correlation between increasing doses of radiation and shorter time from radiation to disease presentation.Pediatric Neurology 01/2014; 50(6). DOI:10.1016/j.pediatrneurol.2013.09.018 · 1.50 Impact Factor