Cases reported "Nose Neoplasms"

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1/98. Nasal and nasal-type natural killer/T-cell lymphoma.

    Nasal and nasal-type natural killer (NK)/T-cell lymphomas follow an aggressive course and have a poor prognosis. Recent pathologic studies suggest that the disease is a malignant proliferation of NK cells, which often express CD56. An association with the Epstein-Barr virus has also been reported. skin involvement occurred in each of the 3 patients studied. radiation therapy provided some benefit to the patients in the early stages. Conventional chemotherapies were not effective. To overcome this multiple-drug resistance of the tumor cells, cyclosporine and high-dose chemotherapy was combined with peripheral-blood stem-cell transplantation. The average life span from the onset of the disease for our patients was 9.6 months. Further improvement in the management of nasal and nasal-type NK/T-cell lymphomas is necessary.
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ranking = 1
keywords = lymphoma
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2/98. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

    Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.
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ranking = 2
keywords = lymphoma
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3/98. Primary splenic tuberculosis in a patient with nasal angiocentric lymphoma: mimicking metastatic tumor on abdominal CT.

    Tuberculosis may be difficult to diagnose when it presents in an uncommon extrapulmonary site. The authors report a case of splenic tuberculosis mimicking metastatic tumor on computed tomography in a 60-year-old woman who had been treated with combination chemotherapy for nasal angiocentric lymphoma. Diagnostic splenectomy revealed multiple necrotic masses in the spleen, which were consistent with caseating granulomas microscopically. diagnosis was confirmed by positive cultures in Lowenstein medium, which grew typical mycobacterium tuberculosis organisms. Following splenectomy, the patient was also treated with a triple-drug antituberculosis regimen with no recurrence of her symptoms.
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ranking = 0.83333333333333
keywords = lymphoma
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4/98. Primary CD56 nasal-type T/natural killer-cell subcutaneous panniculitic lymphoma: presentation as haemophagocytic syndrome.

    Natural killer (NK) cells are large granular lymphocytes that mediate cytotoxic reactions which are not restricted by the major histocompatibility complex. In recent years it has become apparent that a minor proportion of malignant lymphomas expresses an NK-cell phenotype defined by its reactivity with the CD56 antibody. Primary purely cutaneous CD56 lymphomas have rarely been reported. They share a generally aggressive course and are highly associated with Epstein-Barr virus. We describe a patient with a primary cutaneous nasal-type T/NK-cell lymphoma that presented as a haemophagocytic syndrome and showed an aggressive clinical course.
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ranking = 1.1666666666667
keywords = lymphoma
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5/98. MALT lymphoma of nasal mucosa treated with antibiotics.

    Mucosa associated lymphoid tissue (MALT) lymphomas occur in sites other than the gastrointestinal tract and in the early stages respond to treatment with antibiotics. We report a rare case of nasal mucosal MALT lymphoma which responded to treatment with antibiotics and has since then remained in remission.
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keywords = lymphoma
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6/98. Nasal non-Hodgkin's lymphoma causing mucocele of the maxillary antrum.

    The authors report a case of an antral mucocele secondary to obstruction of the antral ostium by a non-Hodgkin's lymphoma. Antral mucoceles are rare, and this is the first report of one such caused by a nasal lymphoma.
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keywords = lymphoma
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7/98. Nasal natural killer cell lymphoma in a post-renal transplant patient.

    Posttransplant lymphoproliferative disorders in organ allograft recipients are most commonly of B-cell origin and only occasionally of T-cell origin. We present here a case of nasal natural killer cell lymphoma associated with Epstein-Barr virus that occurred in a recipient of a renal transplant 4 years posttransplantation. Immunohistochemically, the lymphoma cells showed CD2-, surface CD3-, cytoplasmic CD3E , CD56 , CD57-, CD16-, and CD43 phenotype. Analyses of T-cell receptor beta and gamma genes showed germ line configurations. EBER-1 was detectable in the lymphoma cells. The patient was diagnosed as having natural killer cell lymphoma and was treated with six courses of combination chemotherapy for non-Hodgkin's lymphoma He has been in remission for more than 3 years thereafter. To the best of our knowledge, this is the first report of a posttransplant NK cell lymphoma associated with Epstein-Barr virus.
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ranking = 1.6666666666667
keywords = lymphoma
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8/98. Nasal and nasal type CD56 natural killer cell/T-cell lymphoma: a case with rapid progression to bone marrow involvement.

    We describe herein a case of CD56 natural killer cell lymphoma (NKCL) that showed unusual clinical manifestations and a fulminant course of disease. A 70-year-old male patient presented with rapidly growing skin lesions and fever; a nasal lymphoma was subsequently detected and bone marrow involvement developed within a few weeks. Although the time relationship is not clear, bone marrow involvement could be dissemination from localized disease of nasal and nasal type NKCL. As seen in our case, the course can be excessively aggressive and fulminant even when it first appeared as a localized disease, and CD56 positivity, which is a specific characteristic of NKCL, may serve as a factor showing a poor prognosis for a malignant lymphoma.
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ranking = 1.1666666666667
keywords = lymphoma
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9/98. Nasal NK/T cell lymphoma presenting as transverse myelopathy.

    A case of nasal NK/T cell lymphoma with central nervous system (CNS) involvement is reported. A 56-year-old man presented with eyelid edema and transverse myelopathy. cerebrospinal fluid examination revealed atypical lymphoid cells with azurophilic granules, which were positive for CD2, CD8, and CD56, and negative for CD3 and CD5 by flow cytometry. Because a tumor mass was found involving the ethmoid and maxillary sinuses, CNS involvement was considered to have resulted from local invasion by the nasal lymphoma. In spite of intensive chemotherapy including intrathecal infusion, the patient died 6 months after the initial diagnosis. autopsy revealed that lymphoma cells were positive for cytotoxic molecules, granzyme B and TIA-1, and EB virus-encoded rna-1 (EBER-1), and they showed no rearrangement of TCR-beta, -gamma, or -delta genes, suggesting an NK-cell origin of the lymphoma cells. They showed an angiocentric and angiodestructive pattern in the subarachnoid space, focally extending to the cerebral cortex and cranial and spinal nerve roots. Marked demyelination was found in the lateral and posterior funiculi of the spinal cord. Thus, the pathogenesis of this spinal demyelination might be attributed to ischemia secondary to angiocentric and angiodestructive infiltration by lymphoma cells.
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ranking = 1.5
keywords = lymphoma
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10/98. Effects of cancer therapy on dental and maxillofacial development in children: report of case.

    Malignant lymphoma is one of the most common hematological diseases of children. The prognosis is fairly good with multimodal cancer therapy. We reported a boy with Burkitt's lymphoma in the nasal cavity who received chemotherapy and irradiation of the head and neck area at four years of age. During seven years of follow-up, we studied the developmental effects of cancer therapy, including general growth, maxillofacial bones, and dentition. Compared with boys of matching age, the development of his entire body and maxillofacial bones was delayed. In the irradiated areas, the roots of teeth were short or poorly developed and the root apices showed premature closure. After the patient was in remission from the tumor in his early childhood, the long-term effects of cancer therapy on dental and maxillofacial development are worth our further evaluation and follow-up.
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ranking = 0.33333333333333
keywords = lymphoma
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