Cases reported "Neoplasms, Second Primary"

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1/117. Gliomatosis cerebri with secondary glioblastoma formation: report of two cases.

    The clinicopathological features of two cases of gliomatosis cerebri associated with secondary glioblastoma formation are reported. In both cases, glial cells were diffusely distributed in the supra- and infratentorial regions and underlying brain structures were preserved from the onset. In spite of such diffuse distribution of neoplastic glial cells, similar to that observed in low-grade astrocytoma, in both cases the tumor underwent complete remission after radiotherapy. However, the tumor recurred as a localized glioblastoma in both cases, 37 months (case 1) and 7 months (case 2) after the radiotherapy. In both cases, recurrence was accompanied by prominent dissemination of CSF. The recurrent tumors were radiation resistant, and the patients' conditions deteriorated rapidly after recurrence. The present two cases demonstrated that gliomatosis cerebri, classified among brain tumors of unknown origin by the world health organization, may transform into highly proliferative circumscribed tumors, in spite of their good response to radiotherapy. Examination of pathological features and their correlation with MRI findings may allow us to better understand the response to radiotherapy and the process of recurrence.
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2/117. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?

    Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.
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3/117. Clinical delivery of intensity modulated conformal radiotherapy for relapsed or second-primary head and neck cancer using a multileaf collimator with dynamic control.

    BACKGROUND AND PURPOSE: Concave dose distributions generated by intensity modulated radiotherapy (IMRT) were applied to re-irradiate three patients with pharyngeal cancer. patients, MATERIALS AND methods: Conventional radiotherapy for oropharyngeal (patients 1 and 3) or nasopharyngeal (patient 2) cancers was followed by relapsing or new tumors in the nasopharynx (patients 1 and 2) and hypopharynx (patient 3). Six non-opposed coplanar intensity modulated beams were generated by combining non-modulated beamparts with intensities (weights) obtained by minimizing a biophysical objective function. Beamparts were delivered by a dynamic MLC (Elekta Oncology Systems, Crawley, UK) forced in step and shoot mode. RESULTS AND CONCLUSIONS: Median PTV-doses (and ranges) for the three patients were 73 (65-78), 67 (59-72) and 63 (48-68) Gy. Maximum point doses to brain stem and spinal cord were, respectively, 67 Gy (60% of volume below 30 Gy) and 32 Gy (97% below 10 Gy) for patient 1; 60 Gy (69% below 30 Gy) and 34 Gy (92% below 10 Gy) for patient 2 and 21 Gy (96% below 10 Gy) at spinal cord for patient 3. Maximum point doses to the mandible were 69 Gy for patient 1 and 64 Gy for patient 2 with, respectively, 66 and 92% of the volume below 20 Gy. A treatment session, using the dynamic MLC, was finished within a 15-min time slot.
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4/117. meningioma after radiotherapy for Hodgkin's disease.

    The most common second primary tumors after treatment of childhood Hodgkin's disease are leukemia, lymphoma, breast cancer, soft tissue sarcoma, and thyroid cancer. Although intracranial meningioma has been reported after radiotherapy to the scalp for benign conditions and for intracranial primary brain tumors, this appears to be an extremely rare sequelae of treatment for Hodgkin's disease. The authors describe a 15-year-old boy who underwent radiotherapy for Hodgkin's disease and in whom a meningioma developed in the posterior fossa 27 years later.
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5/117. glioblastoma multiforme in a mature ovarian teratoma with recurring brain tumours.

    AIMS: We report a case study to highlight the occurrence of glioblastoma multiforme in an ovarian teratoma. methods AND RESULTS: A 10-year-old girl presented with a left frontal lobe primitive neuroectodermal tumour which was successfully treated. After 6 uneventful years, she developed glioblastoma multiforme located posterior to the site of the initial tumour. Six years later, she presented with a mature cystic teratoma containing glioblastoma multiforme. CONCLUSIONS: glioblastoma in an ovarian teratoma is an exceptional event, which might have an initial clinical presentation as a metastatic brain tumour. Alternatively, recurring glial tumours may occur in a genetically predisposed person; the role of radiation and chemotherapy in this context remains to be elucidated.
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6/117. T cell malignant lymphoma in the central nervous system after acute lymphoblastic leukemia in a child.

    A malignant lymphoma developed in the central nervous system (CNS) of a 7-year-old boy 5 years after he had received chemotherapy and cranial irradiation for acute lymphoblastic leukemia (ALL). Bone marrow analysis of the original leukemia showed a pre-B cell phenotype, whereas the resected brain tumor showed a T cell phenotype on immunophenotypic analysis. Because of this difference in immunophenotype, and because the patient had received multiple-drug chemotherapy and 1,800 cGy of cranial irradiation for the original ALL, we consider that the malignant lymphoma was a second neoplasm. This is a very rare case in two respects: it was a malignant lymphoma arising in the CNS as a second neoplasm after ALL and a T cell lymphoma occurring in a child.
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keywords = central nervous system, nervous system, brain
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7/117. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.

    A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.
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keywords = central nervous system, nervous system, brain
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8/117. Malignant fibrous histiocytoma after allogeneic bone marrow transplantation.

    A 24-year-old woman with CML underwent allogeneic BMT in August 1995 from a one-antigen HLA mismatched brother. Conditioning included BuCy2 and CsA and MTX were used to prevent GVHD. In July 1997 she developed right leg pain, lytic bone lesions of distal femur and a solid mass of soft tissue. Histological diagnosis of malignant fibrous histiocytoma was made. Despite treatment with surgery and chemotherapy (doxorubicin and ifosfamide), the patient died 1 year later with local recurrence of the tumor and liver, lung and brain metastases. The CML was in CR.
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9/117. Multiple oligodendroglioma: case report.

    An 18-year-old female patient was hospitalized with headache and disturbance of consciousness. magnetic resonance imaging (MRI) revealed a tumor in the left parieto-occipital lobe. The tumor was totally removed, and postoperative radiation therapy was administered locally at 50 Gy. Ten months later, she experienced sudden onset of unconsciousness and headache. Computed tomography (CT) and MRI demonstrated multiple mass lesions in the whole brain. Following the systemic chemotherapy, removal of the largest tumor was performed. Histological examination proved all excised tumors to be oligodendroglioma without evidence of malignant change.
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10/117. Brain tumors as second malignant neoplasms in patients with osteosarcoma treated with adjuvant and neoadjuvant chemotherapy: report of 2 cases.

    A malignant, primary brain tumor developed as Second Malignant Neoplasm (SMN) in 2/490 long-term-survivor osteosarcoma patients treated at our Institute over a 20-yr period. They developed the brain tumor (one astrocytoma and one glioblastoma) 3 and 5 yr after treatment, (chemotherapy and surgery), for localized osteosarcoma of the extremity.
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