Cases reported "Medulloblastoma"

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1/17. A case of atypical absence seizures induced by leuprolide acetate.

    We report a case of a 13-year-old female with atypical absence seizures induced by prolonged administration of long-acting leuprolide acetate (LA). This patient had brain involvement resulting from chemotherapy and radiotherapy for a medulloblastoma. At 13 years of age, administration of long-acting LA was started. After the third dose of long-acting LA, atypical absence seizures appeared. After discontinuing long-acting LA, the seizures stopped without administration of any antiepileptic drugs. However, 2 years, 6 months later, the same seizures again appeared. On the basis of the findings of endocrinologic investigations and the reported data of pharmacokinetics of LA, we speculate that her seizures were induced by LA and that the seizures were associated with the presence of brain damage in the patient. Care should therefore be taken when using long-acting LA or other gonadotropin-releasing hormone analogues for pediatric patients with diffuse brain damage.
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2/17. Shunt-related abdominal metastases in an infant with medulloblastoma: long-term remission by systemic chemotherapy and surgery.

    This is the first reported case of long remission of abdominal metastases spread through a ventriculo-peritoneal shunt in an infant diagnosed, four years ago, at age 1 year and 10 months, to have cerebral medulloblastoma. Two years later, while in second complete remission of his cerebral tumor, he showed abdominal metastases, successfully treated by platinum based chemotherapy and surgery. One year later, a second abdominal relapse and hepatic metastases were treated by doxorubicin administration and surgery. Since then the child remained in continuous complete remission. This unusual favorable outcome can be explained by an extreme responsiveness of the tumor, unprotected by the blood brain barrier, to systemic chemotherapy, particularly to doxorubicin administration. The need for careful surveillance of patients with ventriculo-peritoneal shunts is emphasized. Searching for new tools, such as entrapment of doxorubicin in liposomes, able to overcome the blood-brain barrier and to expose brain tumors to effective drugs, probably represents the best choice for future treatment strategies of CNS tumors.
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3/17. Disappearing cystic cerebellar medulloblastoma: the ghost tumour.

    A case of medulloblastoma, which disappeared following steroid administration and a single sitting of radiotherapy (8Gy) is presented. At surgical exploration tumour was not found, multiple biopsies were negative. Post operative CT scan too did not show the tumour. Five months later, tumour reappeared in left cerebellar lobe in cystic and solid form. Patient was reoperated and tumour was decompressed. The biopsy showed medulloblastoma. To our knowledge, medulloblastomas are not known to be completely responsive to either of these modalities.
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4/17. medulloblastoma as a secondary malignancy after radiotherapy-free treatment for acute lymphoblastic leukemia.

    Malignant brain tumors have been reported to occur in survivors of childhood acute lymphoblastic leukemia (ALL) more frequently than in the noncancer control population. The strongest risk factor seems to be cranial radiotherapy, used as central nervous system (CNS) prophylaxis. We report the case of a 9-year-old girl affected with metastatic medulloblastoma that developed 6 years after a diagnosis of acute lymphoblastic leukemia. CNS prophylaxis for ALL consisted of intrathecal methotrexate plus cytarabine (20 administrations) and 4 courses of high-dose methotrexate (5g/m2). No prophylactic cranial radiotherapy was administered. The child, in first complete remission, was well until the occurrence of a second tumor. She was treated for medulloblastoma with craniospinal radiotherapy and chemotherapy. At present, she is alive but with disease. As the unusual association of these 2 malignancies in this patient, the p53 status was investigated using FISH analysis by specific dna probe; no p53 mutation was detected.
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5/17. A case of treatment-related leukoencephalopathy: sequential MRI, CT and PET findings.

    A case of treatment-related leukoencephalopathy is presented. A patient with medulloblastoma was postoperatively treated with craniospinal axis irradiation. One month after irradiation, weekly intrathecal administration of methotrexate was performed 4 times to treat cerebrospinal fluid dissemination of the tumor. Two months after the initiation of intrathecal chemotherapy, the patient became somnolent and developed decerebrate posturing. magnetic resonance imaging showed diffuse leukoencephalopathy. Positron emission tomography revealed a diffuse decrease in glucose uptake in the deep white matter. Auditory evoked potential also showed diffuse abnormalities, not only in the cerebrum, but also in the brain stem. High dose intravenous leucovorin rescue was attempted without any neurologic improvement.
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6/17. Genetic analyses permit the differentiation between reactive malfunctions ('promyelocyte arrest') and arising promyelocyte leukemia in a pregnant patient with a history of a medulloblastoma.

    We report the case of a 24-year-old woman with a history of radiotherapy for a cerebellar medulloblastoma 2 years prior to detection of a lymph node metastasis of the former disease and a pancytopenia in the peripheral blood. On bone marrow (BM) examination promyelocyte leukemia vs. a reactive 'promyelocyte arrest' were discussed. The translocation t(15;17) was found in some nuclei and there was a PML-RARalpha gene rearrangement detectable by RT-PCR. Furthermore, there was BM infiltration by the primary cancer. All these results led to the diagnosis of a relapse of the medulloblastoma and of a beginning promyelocyte leukemia. As the patient was pregnant, she had to be parted with the baby to facilitate intensive chemotherapy. She did not respond to a therapeutic regimen specific for promyelocytic leukemia but achieved complete remission of the medulloblastoma as well as the leukemia after the administration of polychemotherapy specific for medulloblastoma. One year later, she suffered from a relapse of her leukemia. Now nearly all cells showed a t(15;17) aberration. Immunophenotype analyses showed a shift to a more undifferentiated blast phenotype that was, however, still HLA-DR negative. The patient again received chemotherapy for leukemia but developed a sepsis 3 months later and died of pancytopenia ensuing her leukemia. There was no clinical evidence for recurrence of the medulloblastoma.
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7/17. Three-year recurrence-free survival in a patient with recurrent medulloblastoma after resection, high-dose chemotherapy, and intrathecal yttrium-90-labeled DOTA0-D-Phe1-Tyr3-octreotide radiopeptide brachytherapy.

    BACKGROUND: Most medulloblastomas express high levels of somatostatin type 2 receptors (sst2). DOTA0-D-Phe1-Tyr3-octreotide (DOTATOC) specifically binds sst2 in the low nanomolar range. The cytotoxic effect is mediated by the chelated, beta-emitting, metallic radionuclide yttrium 90 (90Y). The authors applied this innovative treatment option in a boy age 8 years who presented with a recurrent medulloblastoma of the cauda equina: a prognostically poor condition. Targeted radiotherapy was administered to treat minimal sst2-expressing tumor remnants, which persisted despite conventional and high-dose chemotherapy and intercurrent resection of the lesion. methods: A medulloblastoma arising from the floor of the fourth ventricle had been removed surgically; then, the patient was treated with standard adjuvant chemotherapy and craniospinal irradiation according to the prospective HIT '91 protocol. Complete remission was achieved for 20 months, when a drop metastasis of the cauda equina manifested with sensorimotor lumbosacral deficits and urinary incontinence. After four cycles of neoadjuvant chemotherapy (which consisted of combined ifosfamide, carboplatinum and etoposide), two cycles of high-dose chemotherapy and autologous stem cell transplantation were performed; in between, the responding residual tumor within the lumbosacral nerve fibers was microscopically removed. Thereafter, an indium-111-DOTATOC test injection indicated sst2-expressing tumor remnants within the cauda equina. Consequently, 4 cycles of [90Y]-DOTATOC (4x562.5 megabecquerels) were injected directly into the cerebrospinal fluid in monthly intervals. RESULTS: The consolidating intrathecal brachytherapy using [90Y]-DOTATOC was tolerated well. A complete remission was achieved for a 3-year period. The only remaining deficit was urinary incontinence. CONCLUSIONS: Intrathecal administration of targeted radiopeptide brachytherapy in combination with conventional and high-dose chemotherapy and surgical removal represents a promising new option to treat recurrent medulloblastoma and should be explored further.
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8/17. Demonstration of cerebral radiation injury with metabolic positron emission tomography images.

    A 2-year-old girl with medulloblastoma who had postoperative radiotherapy and intrathecal administration of methotrexate is reported. Five months after radiation and chemotherapy, she developed involuntary movement. Positron emission tomography (PET) demonstrated that the metabolic rate of glucose was depressed markedly in the temporal and occipital lobes, indicative of metabolic depression induced by radiation. Prompt initiation of steroid therapy ameliorated the patient's neurological symptoms. Follow-up PET revealed an increase in 18F-fluorodeoxyglucose uptake in the entire brain, including temporal and occipital lesions. No areas with high accumulation of (11C-methyl)-L-methionine were detectable. We concluded that PET may be useful in establishing an early diagnosis of radiation injury of the brain and in monitoring metabolic changes following radiation in brain tumor patients.
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9/17. Hypercalcaemia in cerebellar medulloblastoma: pathogenesis of solid tumour-associated hypercalcaemia.

    A 7-year-old boy developed acute, severe hypercalcaemia following the partial excision of a cerebellar medulloblastoma. The serum calcium level was extremely high (19.8 mg/100 ml), but a skeletal survey revealed no apparent bone metastatic lesions; such lesions were only detected by X-ray 3 weeks after the onset of hypercalcaemia. Hypercalcaemia was promptly resolved by intravenous mithramycin administration, before which the serum parathyroid hormone level, 1,25-(OH)2-vitamin d level and the nephrogenous cyclic amp level were low. However, the relation between serum calcium levels and urinary calcium excretions indicated that renal calcium reabsorption was increased in association with hypercalcaemia, suggesting that a parathyroid hormone-like effect was operative on the renal tubules. It is possible that a combination of increased bone resorption by metastatic tumour cells and renal tubular handling of calcium presumably mediated by tumour-produced humoral factors was responsible for the acute development of severe hypercalcaemia in this patient with medulloblastoma.
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10/17. Asymptomatic leptomeningeal dissemination of tumor to the spinal cord: report of three cases.

    Three children with a history of malignant tumor (cerebellar medulloblastoma in two cases and adrenal neuroblastoma in one case) are discussed. All three had diffuse leptomeningeal dissemination, which was confirmed at autopsy. Computed tomography (CT) revealed diffuse enhancement of the intracranial subarachnoid spaces after the administration of contrast material. Despite the absence of spinal cord signs and symptoms, autopsy showed severe diffuse seeding of neoplastic cells in the spinal subarachnoid space giving the spinal cord the appearance of a "bamboo stick." myelography is necessary to disclose clinically silent spinal cord lesions when CT or cerebrospinal fluid cytological examination reveals evidence of subarachnoid dissemination of tumor.
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