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1/12. Sialoblastoma: clinicopathological/immunohistochemical study.

    Sialoblastoma is an extremely rare salivary tumor diagnosed at birth or shortly thereafter with significant variability in histologic range and clinical course, so that for an individual case it may be difficult to predict the most appropriate therapy. We detail the case of a toddler noted to have a firm 1-2-cm mass in the left cheek at 21 months. Parotidectomy was performed at 26 months, revealing a sialoblastoma; the resection margins were positive. During the next 10 months, the mass recurred or persisted, necessitating numerous procedures. The tumor was composed of basaloid cells with fine chromatin and other more mature cuboidal epithelial cells. Ductules and solid organoid nests with some tendency toward peripheral pallisading were also noted. There was no perineural invasion; necrosis initially was sparse but increased over time. The mitotic rate also increased from 6 to 7/10 high-power fields in the first resection to 20/10 high-power fields in the last resection. Nuclear pleomorphism increased with time. The MiB1 proliferative index revealed a dramatic increase in the number of labeled nuclei: from 3 cells/10 high-power fields in the first specimen to 94 cells/10 high-power fields for the last specimen. Cytokeratin accentuated the ductal structures. S-100 showed a diffuse staining pattern, with darker staining of the spindled myoepithelial cells. The Her-2-neu protein showed moderate cytoplasmic staining, whereas the p53 showed only occasional labeling of nuclei. This is the first case of sialoblastoma with evidence of increasing anaplasia based on increasing proliferative capacity. Therefore, the distinction between benign and malignant sialoblastomas may not be as well defined as previously thought. The patient's prognosis is likely to be determined by the tumor grade as well as the stage at presentation and the extent of resection.
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2/12. Case report: Sinonasal teratocarcinosarcoma.

    Sinonasal teratocarcinosarcoma (SNTCS) is a rare, teratoma-like lesion of the nasal cavity and paranasal sinus. To the best of our knowledge, SNTCS is highly malignant. We report a case of SNTCS arising in the nasal cavity of a 71-year-old male who complained of nasal obstruction and epistaxis. In spite of intensive treatment, the tumor recurred three times and the patient died from a local extension into the anterior cranial fossa 7 years after initial onset. The resected tumors consisted of variegated components, such as epithelial elements, including cystic, ductal and glandular structures occasionally associated with squamous differentiation, neuroectodermal ones exhibiting neural rosette formation, and mesenchymal ones with prominent rhabdomyoblastic differentiation. Immunohistochemical and ultrastructural studies clearly demonstrated characteristic cellular differentiation of each component. These three principal elements were often topographically related and showed morphological transition with each other. These findings suggest the derivation of divergent components from common progenitor cells. Although the cellular atypia of the primary lesion was inconspicuous, the recurrent tumors became anaplastic and mitotically active. Histologic anaplasia may be somewhat related with aggressiveness of recurrent lesions. Appropriate sampling of specimens, and awareness of this rare teratoid tumor are important to make the correct diagnosis.
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3/12. Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression.

    OBJECTIVE AND IMPORTANCE: Rhabdoid meningioma (RM) is a relatively new, Grade III tumor entity according to the latest WHO classification. We report rhabdoid and partly papillary, highly anaplastic, intracerebral meningioma with diffuse leptomeningeal spread and distant SCF metastasis to the cervical cord. CLINICAL PRESENTATION: This 27-year-old female was admitted to the hospital with radiological findings suggestive of a primary brain tumor or a metastasis. After subtotal resection and during radiotherapy, follow-up MRI revealed recurrence, metastasis to meninges at the high cervical level, and diffuse basal leptomeningeal enhancement indicating infiltrating tumor. She died approximately 3 months after onset of symptoms. RESULTS: Histological examination revealed rhabdoid and papillary meningioma with high proliferation rate (80% of MIB1-positive cells), necrosis and extensive brain invasion. It was positive for vimentin and S-100 protein, showed focal epithelial membrane antigen expression and accumulation of intermediate filaments on ultrastructural examination. The recurrent tumor diffusely infiltrated leptomeninges and subarachnoid space. CONCLUSION: This is a rare example of mixed, rhabdoid and papillary variant of meningioma, located entirely within the brain parenchyma and accompanied by a fulminant clinical course. The combination of the histological anaplasia with the highest reported proliferation rate, and loss of the cohesion of neoplastic cells led to diffused infiltration of the leptomeninges and metastasis to the spinal cord.
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4/12. Intracranial astrocytoma: pathology, diagnosis and clinical presentation.

    Approximately 15,000 deaths a year occur in the united states from brain tumors. Astrocytomas, the most common primary tumor of the central nervous system, develop from astrocytes, the neuroglial support cell. Normal astrocytes can undergo anaplasia for unknown reasons and form brain tumors. Astrocytomas are graded from I to IV. As normal brain tissue is displaced by tumor cells, varying signs and symptoms develop, depending on tumor site and size. The goal of surgery, radiation, chemotherapy or a combination of these treatments is to decrease tumor size and improve quality of life and survival time. This article addresses astrocyte cell development, classification of astrocytomas, theories of tumor development, signs and symptoms of astrocytoma, diagnosis and treatment of the astrocytoma. The role of the nurse is explored and two case histories are presented.
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5/12. Case report 658: chondrosarcoma of the proximal phalanx of right fourth finger secondary to multiple hereditary exostoses (MHE).

    A case of grade 1 chondrosarcoma of the phalanx of a finger complicating MHE is presented. Although the cytological features did not include anaplasia, other histological features, the presence of MHE, and recurrence of the tumor after local excision aided in making the correct diagnosis. This case emphasizes the importance of correlating the histological features with the radiological and clinical findings of all bone tumors, and especially cartilaginous lesions.
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6/12. Pleomorphic (anaplastic) neuroblastoma.

    Four very unusual cases of neuroblastoma in children are reported. Clinically, they had a similar presentation: advanced disease at onset, diffuse abdominal spread, poor treatment response, and rapid progression. Pathologically, the four cases were characterized by a striking degree of cellular and nuclear pleomorphism and anaplasia with bizarre and monstrous expressions. Although some authors have referred to the immature ganglion cells found in neuroblastoma as "bizarre ganglion cells," they usually mean the enlarged, maturing neuroblasts that are typical of ganglioneuroblastoma but far from the extreme anaplasia seen in our cases. A correlation between the clinical course and the histological picture could be hypothetically suggested, but additional evidence and confirmation is needed. These cellular aberrations might represent an unsuccessful and impaired attempt at maturation. Independent from prognosis, the definition of anaplastic neuroblastoma is a useful conceptual specification in the study of this tumor and of giant cell and pleomorphic malignant neoplasms of childhood.
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7/12. Malignant transformation of benign gliomas during interstitial irradiation.

    Interstitial curietherapy with 125-iodine is an effective therapeutic option in the treatment of low grade gliomas. Four cases with astrocytoma grade II are presented, where tumour growth characteristics have changed to anaplasia during interstitial irradiation after a primary period of tumour regression. Anaplastic transformation could be due to a radiation effect or an insufficient therapeutic influence of interstitial irradiation on natural tumour progression of glioma growth due to genetic events.
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8/12. Aggressive oligodendroglioma predicted by chromosome 10 restriction fragment length polymorphism analysis. Case study.

    Oligodendrogliomas are indolent brain tumors with mean postoperative survival of about 5 years. However, the range of postoperative survivals is wide, suggesting that these tumors are heterogeneous in their biologic behavior. Using restriction fragment length polymorphism (RFLP) analysis, we studied a case of an oligodendroglioma with loss of chromosome 10 sequences, a finding that has only been reported in glioblastoma multiforme and anaplastic astrocytomas. Four and a half months after the initial surgery the patient returned with a recurrent tumor having classic radiologic and pathologic features of glioblastoma multiforme. Loss of chromosome 10 alleles in oligodendroglioma may be predictive of aggressive biologic behavior, even in the absence of recognized histopathologic characteristics of anaplasia, and may enable us to select more appropriate treatments for this group of patients.
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9/12. Malignant clear cell hidradenoma.

    We report the case of a 77-year-old man with a recurrent malignant clear cell hidradenoma. Establishing histopathological criteria of malignancy for these tumors can be difficult as nuclear anaplasia may be slight to moderate or even absent; however, in contrast to the benign form, malignant clear cell hidradenoma tends to invade the surrounding tissue. As these tumors can show a high rate of local recurrence (50%) and may even metastasize to lymph nodes, bone or visceral organs, wide surgical excision should be performed after the initial diagnosis.
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10/12. Clear cell meningioma: report of a spinal case.

    A sacrococcygeal case of clear cell meningioma in a 38-year-old man is reported. Compared to the seven spinal clear cell meningiomas described so far, this case demonstrated the highest recurrency rate. Multiple recurrences were most likely due to the extensive infiltrative growth pattern of the tumor hindering complete microscopic surgical resection. Histologic parameters were not relevant to predict recurrences, since cellular anaplasia was lacking and growth fraction was low in the first operation and all subsequent recurrences. In view of what is now known of clear cell meningiomas, the tumor showed all histologic and immunohistochemical features which are typical for this tumor entity. However, the tumor was first diagnosed by capable pathologists and neuropathologists as fibroma-like mesenchymal tumor, metastatic renal carcinoma, chordoma, chondroma untypical osteosarcoma, and microcystic meningioma. Correct diagnosis was made by electron microscopy which has revealed comparable findings to those of other rare electron microscopical cases reported so far; high content of cytoplasmic glycogen diffusely distributed throughout the cytoplasm or aggregated within vacuoles, intermediate filaments, desmosomes, interdigitation of cell membranes and large amounts of collagen fibers, some of which were of the giant amianthoid type. Since meningiomatous features of clear cell meningiomas are not obvious light microscopically and the tumors may be confused with nonmeningiomatous neoplasms, electron microscopical investigation is highly recommended in each case of suspected clear cell meningioma.
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