Cases reported "Nose Neoplasms"

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1/11. Lymphoepithelioma-like carcinoma of the skin with apparent origin in the epidermis--a pattern or an entity? A case report.

    BACKGROUND: Lymphoepithelioma-like carcinoma (LELC) is prototypically represented by "undifferentiated" nasopharyngeal carcinoma, but it has also been described in many other anatomic locations, including the skin. In the last of these sites, primary LELC has been assumed in the past to show dermal adnexal differentiation. methods: The authors present a case wherein LELC of the skin (LELCS) instead appeared to be a morphologic manifestation of squamous carcinoma of the skin surface, as supported by the results of immunohistology and in situ hybridization. RESULTS: Like other examples of LELCS, it showed no evidence of integration of the Epstein-Barr viral genome, and its behavior was indolent. CONCLUSIONS: The heterogeneous nature of LELC as seen in different body sites is reviewed in this report, resulting in the conclusion that this tumor probably represents a morphologic pattern rather than a distinct clinicopathologic entity.
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2/11. Primary bony peripheral T-cell lymphoma mimicking nasal type NK/T-cell lymphoma: a case report.

    Primary bony lymphomas are rare, and nearly all are high-grade B-cell lymphomas. Natural killer (NK)/T-cell lymphomas are highly aggressive lymphomas of NK- or T-cell lineage with predominant extranodal presentation and are divided into nasal and nasal-type (extra-nasal). We report a primary bony peripheral T-cell lymphoma mimicking NK/T-cell lymphoma, nasal type. A 22-year-old Taiwanese male presented with a frontal skull bone mass noted for 3 weeks, and received craniectomy with tumor removal. His tumor showed extensive coagulative necrosis with angioinvasion by large lymphoma cells expressing CD2, CD8, CD16, CD43, CD45, CD45RO, CD56, T-cell intracellular antigen-1, and granzyme B, but not CD3, CD4, CD20, CD57, CD68, and betaF1. in situ hybridization for Epstein-Barr virus-encoded mRNA was negative. polymerase chain reaction study of formalin-fixed tissue showed clonal rearrangement of the T-cell receptor-gamma chain gene. The diagnosis was peripheral T-cell lymphoma, unspecified subtype. The initial stage was I(EA). His lymphoma was refractory to chemotherapy, and bony metastases developed in the right iliac bone 2 months later. He died of disease after 6 months without autopsy. We emphasize the importance of detailed immunohistochemical and gene rearrangement studies for the classification of malignant lymphomas via a very rare primary bony lymphoma of peripheral T-cell subtype.
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3/11. TCR gene-rearranged, extranodal NK/T-cell lymphoma, nasal type, presenting as gyrate patches.

    In the CD56 cutaneous nasal-type NK/T-cell lymphoma strongly associated with latent EBV infection, subcutaneous or dermal nodules are the most common skin findings, but great morphologic heterogeneity has been noted including papules, infiltrated plaques, and ulcerated tumors, and TCR genes are mostly germline. We describe a case of nasal and nasal-type NK/T-cell lymphoma featuring multiple erythematous polycyclic patches on the trunk, which is similar to patch stage mycosis fungoides or other cutaneous T cell lymphoma. Immunohistochemical study of a skin biopsy specimen revealed CD2 , CD3epsilon , CD56 , and CD45RO expression in the neoplastic cells. in situ hybridization using an anti-sense Epstein Barr virus early regions probe showed a positive reaction. However, clonal TCR beta gene rearrangement was found.
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4/11. Nasal natural killer/T cell lymphoma with cutaneous involvement: case report and Chinese literature review reported in china mainland.

    Nasal natural killer (NK)/T cell lymphoma is an Epstein-Barr virus (EBV) associated lymphoma that arises in the nasal area and aggressively invades surrounding tissues. Our patient was a 48-year-old male who had had nasal obstruction and nasal discharge for 2 years and infiltrating plaques and necrosis on his nasal dorsum for three months. He developed fever and fatigue two weeks before admission. biopsy from both skin and nasal mucosa revealed atypical medium-sized tumor cells infiltrating into the dermis. Immunohistochemical studies revealed that the tumor cells were UCHL-1, cytoplasmic CD3, CD56, TIA-1, and granzyme B positive, and CD8 and CD20 negative. in situ hybridization for EBV-dna was positive. Clonal TCRb and TCRg gene rearrangement were negative. The patient was treated with cyclophosphamide, vincristine, and prednisone (COP) and with local radiotherapy, but he died 20 days later. We reviewed the cases of nasal NK/T cell lymphoma reported in mainland china in the Chinese literature during the last 5 years.
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5/11. A case of nasal leiomyosarcoma: the first comparative genomic hybridization analysis.

    Leiomyosarcomas are rare in the head and neck region. The treatment of choice is surgical resection. We present a case of leiomyosarcoma in a 50-year-old female patient, arising from the inferior turbinate. The tumor was resected by a transnasal microendoscopic approach and no evidence for tumor recurrence was found during an endoscopic and radiologic follow-up of two years. cytogenetic analysis by means of comparative genomic hybridization revealed chromosomal gains at 4p13p15, 6p21p22, 7q22qter, 9q22qter and chromosomal losses at 10q22qter and 19p12p13.1. With augmented experience, the transnasal approach may be appropriate to resect circumscribed malignant tumors located in the nasal cavity.
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6/11. Nasal NK/T cell lymphoma in taiwan: a clinicopathologic study of 22 cases, with analysis of histologic subtypes, Epstein-Barr virus LMP-1 gene association, and treatment modalities.

    Nasal NK/T cell lymphoma is a distinctive type of extranodal lymphoma with an unique immunophenotype and a strong association with Epstein-Barr virus (EBV). It is one of the common extranodal lymphomas in taiwan. We studied 22 cases of nasal NK/T cell lymphoma to characterize their clinicopathologic features and to explore the possible differences between histologic subtypes and their clinical behavior as well as the prevalence of 30-base pair (bp) deleted latent membrane protein-1 (LMP-1) gene of the EBV. They consisted of 5 cases of small cell type (SC), 6 cases of medium-sized cell type (MC), 6 cases of large cell type (LC), and 5 cases of pleomorphic cell type (PC). Twelve patients were men and 10 were women (1.2 to 1), and their ages ranged from 34 to 75 years with a median age of 55.5 years. The median ages of the LC type and PC type were older than the other 2 types. No other clinical features differed significantly among the 4 subtypes. nasal obstruction was the most common initial presenting symptom. All but 1 case had stage IE disease at the time of diagnosis. Five cases developed extranasal involvement and skin was the most common site. No bone marrow involvement was detected. The majority of patients received local radiotherapy and chemotherapy. Local irradiation was more effective than chemotherapy alone. We achieved an overall survival of 63.6% at 5 years as estimated by the Kaplan-Meier analysis, which was better than other series. All cases displayed an immunophenotypic profile of CD3(epsilon) , CD20-, CD56 , and TIA-1 except that 1 case was CD3(epsilon)-. Fourteen of 22 cases (64%) expressed LMP-1. Nine cases of various cell types (41%) were also CD30 . Among the 4 histologic subtypes, the SC type differed from the other types by the absence of angiodestruction and necrosis, although angioinvasive growth was seen in 2 of them. Pseudoepitheliomatous hyperplasia was seen in only 3 cases of the SC type, and all 5 cases of the SC type were CD30-. No statistical difference in survival was found among the 4 histologic subtypes or between CD30 and CD30- cases. All 22 cases were positive for EBV by polymerase chain reaction and Epstein-Barr virus early rna (EBER) in-situ hybridization. A high prevalence rate of 86% (19/22) of the 30-base pair (bp) deleted LMP-1 gene was found, but 81.5% (22/27) of the EBV-positive control reactive lymphoid tissues also had the 30-bp deleted LMP-1 gene. Therefore, the high prevalence of the 30-bp deleted LMP-1 gene found in NK/T cell lymphoma could be due to the high prevalence of the deleted variant in this geographic region. However, it remains possible that the high prevalence of the deleted LMP-1 gene contributed to the increased incidence of EBV-associated nasal NK/T cell lymphoma in taiwan.
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7/11. Nasal NK/T cell lymphoma mimicking a squamous cell carcinoma: a case report.

    A 48 year old female presented with extensive ulceration of the nasal septum of 8 months duration. Investigations confirmed the local nature of the disease. A biopsy revealed large zones of ischemic necrosis and abnormal lymphoid cells invading vessel walls and glandular structures. Florid squamous metaplasia, and pseudoepitheliomatous hyperplasia of mucosal epithelium mimicked squamous cell carcinoma and necrotising sialometaplasia. immunohistochemistry and insitu hybridization confirmed the diagnosis of an EBV positive, Nasal NK/T cell lymphoma. A pubmed/medline search suggests that this is the first documented case from india.
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8/11. Polymorphic reticulosis: a malignant lymphoma of B-cell lineage.

    Whether the pathogenesis of polymorphic reticulosis is from T cells, B cells, or histiocytes has been controversial. In this study, the Southern blot hybridization technique was used to analyze immunoglobulin and T-cell receptor beta-chain genes and to perform the conventional surface marker analysis in two patients with polymorphic reticulosis. The immunophenotype demonstrated the presence of predominantly mature, activated t-lymphocytes, minimal B-cells, and no natural killer cells or monocytes/granulocytes. The mature T-cell phenotype could be due to either inflammatory infiltrates or neoplastic cells of peripheral T-cell type, because the two coexist in polymorphic reticulosis tumors. The value of surface marker examination is limited in the analysis of PMR tumors. However, genetic analysis revealed that only Ig genes were rearranged, with no rearrangement of the TCR beta gene. Rearrangement of immunoglobulin genes occurs in B-lineage lymphoid neoplasms and is thought to be a criterion for diagnosis of lymphoid neoplasms. Based on genetic analysis and clinicopathologic information, this study concluded that polymorphic reticulosis is a malignant lymphoma of B-cell lineage.
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9/11. Olfactory neuroblastoma: detection of genomic imbalances by comparative genomic hybridization.

    Olfactory neuroblastoma (esthesioneuroblastoma) is a very rare tumour of the olfactory mucosa. Morphological features and cytogenetic studies strongly suggest a neuro-ectodermal origin. Up to now, cytogenetic studies are inconsistent. Some of them have proposed that the tumour belongs to the pPNET family. In the present study we describe genomic imbalances in olfactory neuroblastoma in a 46-year-old woman by using the molecular cytogenetic technique--comparative genomic hybridization (CGH)--in order to define the spectrum of genetic abnormalities in the tumour. The anatomical location and morphological findings were the basis for the diagnosis of esthesionearoblastoma. Immunohistochemical reactions for NSE, synaptophysin, chromogranin a, HNK-1/Leu-7 and S-100 revealed a characteristic immunophenotype. The CGH analysis showed multiple changes including dna overrepresentations of chromosomes 4, 8, 11 and 14, partial dna gains of the long arms of chromosomes 1 and 17, deletions of the entire chromosomes 16, 18, 19 and X, and partial losses of chromosomes 5q and 17p. This study represents an early utilisation of the CGH technique in olfactory neuroblastoma and demonstrates that the tumour carries complex chromosomal aberrations.
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10/11. Characterization of a novel human natural killer-cell line (NK-YS) established from natural killer cell lymphoma/leukemia associated with Epstein-Barr virus infection.

    A novel cell line was established from a patient with a leukemic-state nasal angiocentric natural killer (NK) cell lymphoma with systemic skin infiltration. The morphology of the leukemic cells was large-granular-lymphocyte (LGL), and their immunophenotype was CD2 , CD3-, CD5 , CD7 , CD16-, CD56 , and CD57-. The presence of Epstein-Barr viral (EBV) genome was shown in specimens from the patient's nose, skin, and peripheral blood by in situ hybridization using an EBV-encoded small rna-1 probe or by Southern blotting using a terminal-repeat probe of the EBV genome. Leukemic cells were cocultured with a mouse stromal cell line (SPY3-2) in the presence of 100 U/mL recombinant human interleukin-2 and a novel stromal cell-independent cell line, NK-YS, was established. The NK-YS cells showed LGL morphology and expressed surface CD2, CD5, CD7, CD25, CD56, and CD95. The NK-YS cells retained cytotoxicity against K562 and jurkat cells. A Southern blotting using a terminal-repeat probe of EBV showed that NK-YS and fresh leukemic cells had a clonal EBV genome, whereas the T-cell receptor beta and gamma chain genes of NK-YS were not rearranged. In an immunocytochemical analysis, the NK-YS cells showed a type-II latent infection of EBV. The NK-YS cells preserved the original characteristics of NK cell lymphoma/leukemia and will be a useful tool for the study of biological characteristics of EBV-associated nasal angiocentric NK cell lymphoma/leukemia.
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