Cases reported "Cerebellar Neoplasms"

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11/231. Signet-ring cell ependymoma: case report with implications for pathogenesis and differential diagnosis.

    We describe light microscopic, immunohistochemical and ultrastructural features of a signet-ring cell ependymoma (WHO grade II) identified in a surgically resected left cerebellar cystic tumor from a 64-year-old man. Part of the tumor showed clear-cell differentiation. Immunohistochemical coexpression of glial fibrillary acidic protein and epithelial membrane antigen, characteristic of ependymoma, was detected in both components. Sinuous intermediate junctions, cytoplasmic lumina, and scant astroglial filaments were demonstrated by electron microscopy. Signet-ring cell change was shown to be induced by disproportionate cavitation of either microvillus-bearing cytoplasmic lumina or microrosettes. The staining qualities of clear cells were mainly due to paucity and degeneration of subcellular organelles. Therefore, signet-ring cell ependymomas represent a unique anomaly of intra- and extracellular compartmentalization to be distinguished from various unrelated forms of cytoplasmic volume increase, resulting in an optically similar "empty" appearance of tumor cells. As a clinically relevant consequence, signet-ring cell ependymoma must be included in the differential diagnosis of primary or metastatic neoplasms of the central nervous system, having in common a phenotype characterized by overdeveloped optically lucent cell bodies.
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12/231. Cerebellar neuroblastoma in an infant.

    A cerebellar neoplasm in an 8-month-old boy is reported. While this tumour was composed of small cells and had regions resembling desmoplastic medulloblastoma, it showed ultrastructural neuronal characteristics including bundles of microtubules in the cell processes, numerous synaptic vesicles, and occasional abortive or complete synapses. These characteristic features warranted the diagnosis of a neuroblastoma of the cerebellum. The nature of this rare intraparenchymal tumour in infants is also briefly discussed.
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13/231. Cerebral granular cell astrocytomas: a Mib-1, bcl-2, and telomerase study.

    Granular cell (GC) astrocytoma is an uncommon variant of glioma that shares the cytologic features and high cytoplasmic lysosomal content with granular cell tumors elsewhere in the body. While the histogenesis and behavior of these neoplasms was originally in dispute because most were reported as single cases, the accumulated literature on approximately three dozen such lesions has now verified their usual astrocytic lineage and poor prognosis. Although the GC cell is thought to represent a degenerative process, little is known in these tumors about cell cycle regulation, as measured by Mib-1 and bcl-2 immunolabeling, or expression of other biomarkers of malignancy, such as telomerase. In our study, GC astrocytomas were similar to gemistocytic astrocytomas in their bland histology, often prominent perivascular lymphocytic cuffing and low Mib-1 labeling indices. Like gemistocytes, GCs appear to represent senescent, non-cycling cells. Absence of significant bcl-2 immunolabeling in our three cases, however, suggests that unlike gemistocytes, GC astrocytes develop senescence by mechanisms other than bcl-2 mediated apoptosis suppression. In one case in which frozen tissue was available for assay, we noted relatively high quantitative telomerase expression. The level paralleled that seen in other glioblastomas. Demise for our three patients occurred 3-25 months post-biopsy. Like gemistocytes, the presence of non-proliferative GCs signifies severe abnormalities in cell cycle regulation and maybe hallmarks of tumors with poor prognosis.
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14/231. Subclinical thyroid disease after radiation therapy detected by radionuclide scanning.

    PURPOSE: The actuarial risk for developing benign or malignant thyroid disease following radiation therapy (RT) is controversial, but may be as high as 50% at 20 years. An effective screening modality should be specific but not overly sensitive, a limitation of ultrasound. We questioned whether technetium-99 m pertechnetate ((99m)Tc TcO(4)(-)) scanning could detect clinically significant disease in ostensibly disease-free cancer survivors. methods AND MATERIALS: Eligibility criteria included an interval of at least 5 years after RT to the cervical region, a thyroid gland that was normal to palpation, euthyroid status determined by clinical examination, free T4 and TSH. The 34 patients scanned included 16 children (<18 years old) and 18 adults at the time of RT, 16 females and 18 males. The mean age at RT was 20 years (range, 2.1-50.3 years), and the mean age at (99m)Tc TcO(4)-scanning was 33 years (range, 13.6-58 years), providing a mean interval of 13 years (range, 5.3-26.6 years). The mean RT dose to the thyroid was 36.4 Gy (range, 19.5-52.5). Thyroid scanning was performed with a 5 mCi dose of (99m)Tc TcO(4)(-) obtaining flow, immediate and delayed static, and pinhole collimator images. RESULTS: Seven patients (21.6%) had abnormal scans, and the percentage was higher among children (25%) and females (25%) compared to adults (16.7%) and males (16.7%), respectively. Two of 34 patients (5.9%) were discovered to have a thyroid cancer; histopathologies were papillary and follicular carcinoma. CONCLUSION: In this population of clinically normal cancer survivors who had been irradiated to the cervical region, subclinical thyroid disease, of potential clinical significance, was detected by (99m)Tc TcO(4)(-) in about 20%. Children may be more commonly affected. Although the cost effectiveness of screening will require a larger sample number, we propose a surveillance schema for this patient population.
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keywords = cancer
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15/231. Medulloblastomas with extensive posttherapy neuronal maturation. Report of two cases.

    The authors report on two patients with classic medulloblastoma, each of whom underwent extensive therapy-associated neuronal maturation. The first patient presented at 3 months of age with hydrocephalus caused by a 5-cm tumor in the cerebellar vermis. He underwent a gross-total resection of a desmoplastic medulloblastoma. No mature elements were identified. Despite adjuvant chemotherapy, a 1.5-cm recurrent tumor developed 6 months later. Sections from the subtotally resected tumor demonstrated exclusively mature neuronal elements, ranging from neurocytes to ganglion cells. Four months later, a second recurrent tumor was resected. The specimen collected this time demonstrated classic medulloblastoma morphological characteristics. The patient was subsequently treated with radiation therapy, which seemed to have an effect; however, the tumor eventually progressed and the patient died. The second patient presented at 3 years of age with a midline medulloblastoma and was treated with subtotal resection, radiation therapy, and chemotherapy. Although the tumor remained stable on radiographic imaging, a second resection was performed 8 years later to alleviate hydrocephalus. Histological examination revealed predominantly small mature neurons with scattered ganglion cells and extensive calcification. No adjuvant therapy was given and the patient is alive and well as of his last follow-up examination. The mature neuronal neoplasms resected in both patients demonstrated negligible proliferative indices and stained appropriately with neuronal immunohistochemical markers. The smaller neuronal population resembled those of a central neurocytoma and medullocytoma/cerebellar neurocytoma. Analogous to neuroblastoma, our cases suggest that adjuvant therapy can induce extensive or complete neuronal maturation in medulloblastoma. Additional cases must be studied to determine the prognostic significance of this rare phenomenon.
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16/231. Subglottic hemangioma associated with cutaneous and cerebellar hemangiomas detected by MRI: report of one case.

    Subglottic hemangioma (SGH) is a benign neoplasm that may cause severe and life-threatening respiratory obstruction in infants. However, patients usually present with inspiratory stridor in the first few months of life and may be mistakenly diagnosed as recurrent or persistent croup. Definitive diagnosis is made by image studies, endoscopic examination and biopsy or all. We report a 2-month-old female infant of SGH with initial clinical manifestations of dyspnea and inspiratory stridor co-existing with cutaneous and cerebellar hemangiomas. Clinicians must be alert the possibility of SGH when associated with cutaneous hemangioma. This patient has received oral steroid treatment for more than two months with improvement of the airway obstruction. Although purplish patch lesions over left side of face, eyelid, cheek, and peri-oral regions regressed, the size of the SGH on the followed MRI was slightly enlarged. The diagnosis and various treatments of SGH are discussed and reviewed in this paper.
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17/231. Adjuvant immunotherapy for malignant brain tumors in infants and children.

    Immune deficiency of immunocompetent cells or of humoral factors are essential causes of tumor growth. The authors have investigated the transfer of immunocompetent cells - allogeneic bone marrow cell transfusion and white blood cell intracranial infusion - for the treatment of 11 malignant gliomas in infants and children as an adjuvant to surgery, radiation and/or chemotherapy. Ten cases, from 3 months to 11 years, received bone marrow cell transfusion. Two medulloblastomas and 3 pontine gliomas are dead. Five cases are alive and well 37-65 months following surgery. Among these two posterior fossa neoplasms, a medulloblastoma and a glioblastoma have survived 46 and 65 months, respectively. One cerebral glioblastoma received allogeneic white blood cells infused locally into the tumor bed: it recurred 1 year following surgery, chemotherapy, and immunotherapy. Cytolysis of the tumor cells by sensitized lymphoid cells were demonstrated in this case. The role of immunotherapy should be limited at the present time to adjuvant therapy until its effect on tumor growth is statistically confirmed. The results so far are promising, and improvement of the immunological approach in treating malignant brain tumors is under way.
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18/231. Pleomorphic xanthoastrocytoma of the cerebellum.

    We report a clinicopathologic case of a pure cerebellar pleomorphic xanthoastrocytoma occurring in a 68-year-old male patient. The occurrence of pleomorphic xanthoastrocytoma outside the cerebral hemispheres is exceedingly rare. In the cerebellum only five cases have been reported so far, four of which are composite pleomorphic xanthoastrocytoma-gangliogliomas. This observation reinforces the argument that pleomorphic xanthoastrocytomas should be included in the differential diagnosis of cerebellar neoplasms.
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19/231. Combined pleomorphic xanthoastrocytoma-ganglioglioma of the cerebellum.

    Combined pleomorphic xanthoastrocytoma-ganglioma is a rare neoplasm, occurring in patients younger than 30 years. The clinical course of these tumors is difficult to predict because of their rarity. We report a case of combined pleomorphic xanthoastrocytoma-ganglioma that, in addition to the patient's age, is unusual in several respects. The lesion was located in the cerebellar vermis of a 60-year-old man and was radiographically solid. Histologically, there was an admixture of markedly pleomorphic astrocytic cells and neoplastic ganglion cells, with permeation of the overlying leptomeninges and surrounding cerebellum. In addition, there was focal capillary endothelial proliferation. There was no necrosis, and mitotic activity was rare at 1 mitotic figure per 40 high-power fields. The patient underwent a near gross total resection and postoperative radiotherapy and remains well through 16 months of follow-up.
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20/231. Supratentorial hemangioblastoma in a patient with breast cancer. A case report.

    patients with breast cancer may develop cerebral metastasis. Radio- and chemotherapy are advocated as an alternative to surgery in such patients. A woman operated on for breast cancer 2 years earlier developed a cerebral lesion. A definite preoperative diagnosis of the lesion was not possible on the basis of CT and MRI findings. The lesion proved to be a supratentorial hemangioblastoma. Neurosurgical treatment is recommended for patients with breast cancer who present a cerebral lesion, since a correct diagnosis may only be possible in the operating theater.
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