No abstract available.
Cranial Irradiation ; Leukoencephalopathies* ; Parkinsonian Disorders*

Pituitary tumors occur in 15-50% of patients with multiple endocrine neoplasia of type 1 (MEN1). To the best of our knowledge, no MEN1 case in which the initial lesion was an invasive giant prolactinoma has been reported from Korea. We describe a patient in whom a skull-base tumor involved the sellar and parasellar spaces. A 49 year-old female presented with headache and diplopia. The tumor was ultimately identified as a giant prolactinoma; the serum prolactin concentration increased from 155.6 ng/mL to 3,234.3 ng/mL after cranial irradiation. She was evaluated in terms of incidental hypercalcemia and was found to have parathyroid hyperplasia. Genetic analysis revealed a missense mutation in the MEN1 gene (c.643G>A, p.Val215Met). Two years of treatment with a dopamine agonist reduced, but did not normalize, the serum prolactin concentration. We highlight the aggressive behavior of the giant skull-base tumor, and the diagnostic delay caused by a high-dose hook effect of the MEN1-related prolactinoma.
Cranial Irradiation ; Diplopia ; Dopamine Agonists ; Female ; Headache ; Humans ; Hypercalcemia ; Hyperplasia ; Korea ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Mutation, Missense ; Pituitary Neoplasms ; Prolactin ; Prolactinoma*

No abstract available.
Child* ; Cranial Irradiation* ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.
Cranial Irradiation ; Down Syndrome ; Humans ; Moyamoya Disease* ; Natural History ; Neurofibromatoses ; Neurofibromatosis 1 ; Thyroid Diseases

We have treated adult acute leukemia 64 patients between January 1990 and October 1991 at the Chungnam National University Hospital. They were examined for the impact of presenting WBC count on the initial course and from them we have chosen twenty patients whose leukocyte count is over one hundred thousands per cubic milimeter. We divided the twenty patients into 4 groups on the base of treatment modalities: conservative therapy only, chemotherapy only, cranial irradiation only, and chemotherapy with cranial irradiation. Early sudden death rate is lower in cranial irradiation with/without chemotherapy groups than the conservative only or chemotherapy only patients. Also the remission rate is high in cranial irradiation with chemotherapy patients. Therefore we suggest that the rapid intervention of cranial irradiation in adult acute leukemia could be helpful in reducing the early sudden death rate and perhaps in increasing the remission rate.
Adult* ; Chungcheongnam-do ; Cranial Irradiation* ; Death, Sudden ; Drug Therapy ; Emergencies* ; Humans ; Leukemia* ; Leukocyte Count ; Leukocytosis*

PURPOSE: To evaluate the MR imaging findings and the usefulness of MR imaging in the diagnosis and followup of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia. MATERIALS AND METHODS: We retrospectively evaluated the MR imaging findings of eight children with white matter abnormalities on MR out of seventeen acute leukemic patients with various neuropsychiatric symptoms who received intrathecal methotrexate administration, with or without cranial irradiation. In all cases, initial MR was performed within a week of the onset of neuropsychiatric symptoms. Follow-up MR was performed one to sixteen months after initial study, and the MR imaging findings were compared with the initial findings. RESULTS: The initial MR imaging findings were classified into three categories: focal or multifocal white matter abnormalities (3/8), and diffuse white matter abnormalities without enhancement (3/8), and diffuse white matter abnormalities with enhancement (2/8). At follow-up MR, diffuse or focal atrophic changes were noted in all children. White matter abnormalities improved in two out of three patients with focal or multifocal white matter abnormalities. In five with diffuse white matter abnormalities, the extent of these showed no significant change, but contrast enhancement was markedly reduced in two children in whom diffuse white matter abnormalities with enhancement had been demonstrated. CONCLUSION: In pediatric leukemia, the MR imaging findings of leukoencephalopathy following CNS prophylaxis therapy are variable, but are specific with the clinical history of neuropsychiatric symptoms after intrathecal methotrexate administration, with or without cranial irradiation. The MR imaging is valuable in the diagnosis and follow-up of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia.
Child ; Cranial Irradiation ; Diagnosis ; Follow-Up Studies ; Humans ; Leukemia* ; Leukoencephalopathies* ; Magnetic Resonance Imaging* ; Methotrexate ; Retrospective Studies

Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.
Cerebral Angiography ; Child ; Cranial Irradiation ; Craniopharyngioma ; Follow-Up Studies ; Humans ; Ischemic Attack, Transient ; Moyamoya Disease

PURPOSE: Acute leukemia with hyperleukocytosis (more than 105/mm3) is at high risk of early sudden death, usually from intracerebral hemorrhage. Emergency cranial irradiation is a relatively simple approach to solve this the problem. We summarized our experience of cranial irradiation in 24 leukemic children who presented with hyperleukocytosis. METHODS AND MATERIALS: Between 1990 and 1998, 40 children with acute leukemia presenting with hyperleukocytosis were referred for emergency cranial irradiation. Among these patients, 24 children were evaluable. There were 16 boys and eight girls, their ages ranged from 2 to 13 years (median 9.5 years). The initial leukocyte counts ranged 109,910/mm3 to 501,000/mm3. Peripheral blood smear was performed in all patients and noted the morphology of the blast. Introduction of emergency cranial irradiation was determined by the leukocyte counts (more than 100,000/mm) and the existence of the blast in peripheral blood smear. All patients were treated with intravenous hydration with alkaline fluid and oral allopurinol. Cranial irradiation started on the day of diagnosis. With 2 Gy in one fraction in 4 patients, 4 Gy in two fractions in 20 patients. RESULTS: The WBC count had fallen in 19 patients (83%) and no intracerebral hemorrhage occurred after irradiation. There were five cases of early deaths. Four patients died of metabolic complications, and one patient with intracerebral hemorrhage. He died 5 hours after cranial irradiation. No patient had any immediate side effect from cranial irradiation. CONCLUSION: Our data suggest, that emergency cranial irradiation can be safely chosen and effective in childhood leukemic patients presenting with high leukocyte counts.
Allopurinol ; Cerebral Hemorrhage ; Child ; Cranial Irradiation* ; Death, Sudden ; Diagnosis ; Emergencies ; Female ; Humans ; Leukemia ; Leukocyte Count

PURPOSE: Growth impairment and growth hormone deficiency have been reported in children treated for acute lymphoblastic leukemia (ALL). This study was undertaken to investigate the growth pattern in children who have been diagnosed and treated ALL. METHODS: We have studied growth during 5-year period after diagnosis in 29 children with ALL who achieved complete continuous first remission following induction chemotherapy from January 1991 to December 1998 at Pusan National University Hospital. Maintenance chemotherapy was given over two or three years in all patients and 10 received additional prophylactic cranial irradiation at a total dose 1,800 to 2,000 cGy. RESULTS: Mean age of patients at diagnosis was 5.81+/-3.44 years, mean height standard deviation score (SDS) at diagnosis was 0.58+/-0.78. During 1 st and 2 nd year of therapy, mean height SDS was significantly decreased to 0.21+/-0.78 (P<0.05), 0.26+/-0.99 (P<0.05). And these decreased mean height SDS during therapy were gradually returned to the baseline levels within 2 years after the end of therapy. During and after therapy, there were no significantly differences in changes of mean height SDS between a group who had prophylactic cranial irradiation and one who had not. There were also no significantly different changes of mean height SDS according to sex or age at initiation of treatment. CONCLUSION: Our data indicate that chemotherapy significantly affects growth of patients treated for ALL and that effects were almost transitory, whereas radiotherapy at the doses used in this study does not potentiate growth impairment.
Busan ; Child* ; Cranial Irradiation ; Diagnosis ; Drug Therapy ; Growth Hormone ; Humans ; Induction Chemotherapy ; Maintenance Chemotherapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Radiotherapy

We report a case of de novo development of cavernous malformation after cranial irradiation. The patient was a 19-year-old male who had been operated on 10 years ago for cerebellar pilocytic astrocytoma. After subtotal tumor removal, he had received cranial irradiation for the residual tumor. He was readmitted for extraocular muscle palsy, dysarthria and facial weakness about 10 years after operation and irradiation. Magnetic resonance imaging of the brain showed an abnormal mixed signal lesion in the lower pons and pontomedullary junction. The patient underwent surgical removal of the hematoma in the pons and the cavernous malformation was identified.
Astrocytoma* ; Brain ; Cranial Irradiation ; Dysarthria ; Hematoma ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasm, Residual ; Paralysis ; Pons ; Young Adult