1/18. Successful treatment of disseminated nasal NK/T-cell lymphoma using double autologous peripheral blood stem cell transplantation.Nasal natural killer (NK)/T-cell lymphoma is a rare disease with an aggressive clinical course. prognosis is generally poor and the disease is invariably fatal after systemic dissemination. We report a patient with aggressive nasal NK/T-cell lymphoma who was resistant to therapy and developed systemic dissemination involving the intestine, skin, and stomach. Epstein-Barr virus (EBV) was detected by Southern blotting with EBV-terminal repeat probe and by in situ EBV-encoded small nuclear early region-1 hybridization. The patient was treated using double high-dose chemotherapy and autologous peripheral blood stem cell transplantation (auto-PBSCT). Pretransplant conditioning for the first auto-PBSCT was MCVC (high-dose ranimustine, carboplatin, etoposide [VP16], and cyclophosphamide), and for the second auto-PBSCT, modified ice (high-dose ifosfamide, VP16, and carboplatin). The patient obtained a complete remission and has been free of disease for 3.0 years since the second PBSCT. These observations suggest that double high-dose chemotherapy with PBSCT support may be effective in resistant nasal NK/T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/18. Epstein-Barr virus early ribonucleic acids as a diagnostic adjunct for relapsed metastatic tumors in patients with cured primary undifferentiated nasopharyngeal carcinoma.PURPOSE: Epstein-Barr virus (EBV) has been consistently shown to be associated with undifferentiated nasopharyngeal carcinoma (NPC). In this article, the authors attempt to detect Epstein-Barr virus in distant relapsed metastatic sites in undifferentiated NPC patients with cured primary cancer. MATERIALS AND methods: in situ hybridization (ISH) technique is a reliable method to detect EBV early RNAs (EBERs) within NPC cells. We used a nonisotopical ISH technique to examine the presence of EBERs in paraffin-embedded tissues obtained from 1 paired specimen of primary NPC and its metastatic counterpart at liver and 2 metastatic specimens of retroperitoneal lymph nodes and bone. RESULTS: All the primary lesions and the metastatic tumors of NPC with undifferentiated histology contained EBERs that could be clearly detected in the nuclei of cancer cells. CONCLUSIONS: This article shows that EBERs can be successfully detected in cells of the distant relapsed metastatic sites. These results suggest that this nonisotopical ISH method of EBERs can be potentially used to diagnose NPC patients developing distant relapsed metastatic lesions with cured primary cancer early. It can offer quick information as to institute suitable salvage chemotherapy for these patients.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/18. Epstein-Barr virus rna detection and glandular differentiation in nasopharyngeal carcinoma: report of 2 cases.Most tumors arising in the nasopharynx are either squamous cell carcinoma or so-called undifferentiated carcinoma of the nasopharyngeal type. Primary adenocarcinomas of the nasopharynx are rare, and glandular differentiation in undifferentiated carcinoma of the nasopharyngeal type has not been reported to date. We report 2 cases of undifferentiated carcinoma of the nasopharyngeal type that show distinct glandular differentiation by light microscopy, histochemistry, immunohistochemistry, and ultrastructure. Both tumors showed equal positivity for Epstein-Barr virus latent membrane protein and in situ hybridization for Epstein-Barr virus genome in the undifferentiated areas of the tumor and those featuring glandular differentiation.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/18. CD2- CD4 CD56 hematodermic/hematolymphoid malignancy.BACKGROUND: CD2- CD4 CD56 lymphoid malignancy has been only rarely reported the last 5 years. It is characterized by a high incidence of cutaneous involvement, cytologically agranular cells, aggressive clinical course, and negative Epstein-Barr virus (EBV) involvement. observation: We describe a Japanese patient with a unique hematolymphoid malignancy characterized by an involvement of skin, nasopharyngeal region, bone marrow, lymph node, and a CD4 CD43 CD56 CD2- CD3- CD8- and terminal deoxynucleotidyl transferase phenotype. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, a massive proliferation of atypical pleomorphic cells with medium-sized nuclei were observed throughout the dermis. No clonal rearrangement of T-cell receptor (TCR)-beta gene or immunoglobulin heavy chain J gene was found, and no positive identification of EBV by in situ hybridization for EBV-encoded small nuclear rna was found. The patient underwent high-dose chemotherapy with autografting of peripheral blood stem cells; however, the tumors quickly relapsed. CONCLUSION: We gathered data from 17 cases of lymphoid malignancy from the literature sharing immunophenotypic and genotypic features similar to those of our case, including CD2- CD4 CD56 and germline rearrangement of TCR. Although the cellular origin could not be decided, this malignancy was found to have 100% affinity for skin, a short course, and poor prognosis.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/18. Nasal natural killer lymphoma associated with Epstein-Barr virus in a patient infected with human immunodeficiency virus.Nasal natural killer (NK) lymphoma associated with Epstein-Barr virus (EBV) is a rare lymphoma that has not yet been reported in patients with human immunodeficiency virus (hiv). This report describes the first case, to our knowledge, of nasal NK cell lymphoma in an hiv-positive patient. A 50-year-old African man presented with an obstructive nasopharyngeal tumor, leading to the diagnosis of hiv infection. Nasal biopsy specimens showed NK cell lymphoma, confirmed on nasal tissues by morphologic, immunohistochemical, and polymerase chain reaction studies using a denaturing gradient gel electrophoresis technique that showed no T-cell receptor gamma rearrangement. The EBV was detected by in situ hybridization. The patient received chemotherapy but died from infection. To our knowledge, this is the first reported case of nasal NK cell lymphoma associated with EBV in an hiv patient. Involvement of EBV in hiv non-B-cell lymphomas may represent a further manifestation of opportunistic EBV infection arising in these patients.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/18. Development of follicular dendritic cell sarcoma in hyaline-vascular Castleman's disease of the nasopharynx: tracing its evolution by sequential biopsies.AIMS: Hyaline-vascular Castleman's disease (HVCD) and follicular dendritic cell (FDC) sarcoma occurring in the nasopharynx are both extremely rare. We report the first case of transformation of the former into the latter as documented by sequential biopsies. The steps involved in the transformation were described in detail and the possible role of p53 studied. methods AND RESULTS: The patient presented at the age of 23 years with nasopharyngeal HVCD. Hyaline- vascular Castleman's disease with FDC overgrowth was diagnosed in a recurrence 8 years later, and a frank FDC sarcoma developed at the same site 11 years after initial presentation. The patient remained disease-free 3 years after excision and adjuvant chemotherapy. The FDC sarcoma comprised swirling fascicles of spindly cells with indistinct cell borders. The tumour cells expressed the FDC markers CD21, CD35 and CNA.42 and in-situ hybridization for Epstein-Barr virus-encoded RNAs was negative. Over-expression of p53 protein was observed in the FDC sarcoma and an increased number of weakly p53-positive spindly cells could also be demonstrated in the HVCD specimen. This finding suggested a possible role of p53 in the evolution from HVCD to FDC sarcoma. Critical analysis of the literature shows that, among the 13 reported cases of FDC sarcoma associated with Castleman's disease, possible progression from the latter to the former is documented in only two cases. CONCLUSIONS: The sequential changes observed in the current case provide further evidence to strengthen the role of HVCD as a possible precursor of FDC sarcoma. There is a possible role of p53 in the transformation process but confirmation by future studies is needed.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/18. Upper respiratory tract carcinoma with chromosomal translocation 15;19: evidence for a distinct disease entity of young patients with a rapidly fatal course.BACKGROUND: carcinoma of the upper respiratory tract is rare in childhood, and cytogenetic aberrations have not been characterized in this population. The chromosomal translocation 15;19 has been reported four times previously. All patients were young and had tumors arising in the thorax. The three reports that provide clinical follow-up all describe superior vena cava syndrome and death soon after presentation. All tumors were diagnosed as carcinoma (three undifferentiated, one mucoepidermoid), and the authors suggested thymus, lung, or germ cell origin. methods: The authors investigated the clinical and pathologic findings in two patients with poorly differentiated carcinoma showing evidence of t(15;19). This included a 13-year-old girl with a rapidly growing epiglottic mass, leading to superior vena cava syndrome and death and a 12-year-old girl with an aggressive nasopharyngeal mass showing intracranial extension. RESULTS: The laryngeal tumor was poorly differentiated, with vesicular nuclei, prominent nucleoli, extensive necrosis, and a lymphoplasmacytic infiltrate; cells were positive for cytokeratin and negative for lymphoma, melanoma, germ cell, and endocrine markers. Electron microscopy showed rare intermediate junctions and basal lamina. The nasopharyngeal tumor was poorly differentiated with areas of obvious squamous differentiation observed histologically, immunophenotypically, and ultrastructurally. Cytogenetic and fluorescent in situ hybridization studies were consistent with t(15;19)(q13;p13.1) in both cases. Both children received chemo- and radiotherapy. The first child died of disease after 36 weeks; autopsy revealed tumor in the larynx with spread to the skin/subcutis (neck and thorax) and lymph nodes (cervical, subcarinal, and pulmonary hilar). The second child developed widespread bony metastases and died of disease after 13 weeks. CONCLUSIONS: In conjunction with previous reports, the authors' findings show that t(15;19) is part of a distinct clinicopathologic entity characterized by young age, midline carcinoma of the neck or upper thorax, and a rapidly fatal course. female gender and superior vena cava syndrome are common. The histogenesis of these distinctive tumors is unknown. The authors' findings suggest origin in the upper airway, perhaps from submucosal glands.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/18. Nasal natural killer cell/T-cell lymphoma showing cellular morphology mimicking normal lymphocytes.We report the autopsy case of a 34-year-old Japanese man with a nasal natural killer (NK)-cell/T-cell lymphoma. The patient developed the disease at 32 years of age, and a biopsy of the nasopharynx revealed pleomorphic lymphoma cell proliferation. radiotherapy was performed, but the patient eventually died of respiratory failure. After radiotherapy, no histologic evidence of malignancy was obtained with biopsy materials featuring lymphocytic infiltration. autopsy studies, including in situ hybridization for Epstein-Barr virus-encoded rna, revealed generalized infiltration of normal lymphocyte-like, UCHL-1-positive, and Epstein-Barr virus-encoded rna-positive lymphoma cells. Monoclonal proliferation of the Epstein-Barr virus-carrying cells was verified by means of Southern blot analysis. Retrospectively, we concluded that the normal lymphocyte-like presentation of the lymphoma cells, probably influenced by radiotherapy, prevented pathologists from recognizing the lymphoma. The utility of in situ hybridization for Epstein-Barr virus-encoded rna in identification of tumor cells is emphasized with respect to the present case.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
9/18. Nasopharyngeal carcinoma metastasis to the pituitary gland: a case report and literature review.While nasopharyngeal carcinoma (NPC) commonly invades the skull base, true central nervous system metastasis is a rare phenomenon. We report a case of NPC metastasis to the pituitary gland and review the literature for similar events. Eight months after his definitive radiation therapy, our patient presented with symptoms of optic chiasm compression and panhypopituitarism. Medical imaging revealed a pituitary mass but demonstrated no evidence of skull base erosion or direct intracranial extension. Subsequent biopsy of the pituitary lesion was confirmed as NPC in origin with Epstein-Barr virus-encoded rna in-situ hybridization studies. The patient was treated with high dose chemotherapy followed by autologous stem cell transplant, which produced short-term symptomatic relief and at least a 7 month survival.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
10/18. Coexpression of NOR1 and SIX3 proteins in extraskeletal myxoid chondrosarcomas without detectable NR4A3 fusion genes.Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal tumor cytogenetically characterized by reciprocal translocations, such as t(9;22)(q22;q12) and t(9;17)(q22;q11), which result in EWSR1/NR4A3 and TAF15/NR4A3 fusion genes (alias EWS/NOR1, TAF2N/NOR1), respectively. NOR1 is an orphan nuclear receptor and acts as a transcription factor that can bind to its putative coactivator, SIX3. Although the NOR1 fusion protein has been implicated in oncogenesis of EMC, a small fraction of EMC lacks detectable rearrangements of the NR4A3 gene or 9q22. We report a case of EMC with no detectable NR4A3 gene alterations, as assessed with various molecular techniques including reverse transcription-polymerase chain reaction (RT-PCR), Southern blotting, interphase fluorescence in situ hybridization, and PCR single-strand conformation polymorphism-but with coexpression of native NOR1 and SIX3. In our survey of another 18 EMCs, we identified one more case expressing both NOR1 and SIX3 but lacking NR4A3 fusion. Fourteen tumors with detectable NR4A3 fusion genes (EWSR1-NR4A3; TAF15-NR4A3) expressed neither native NOR1 nor SIX3. SIX3 expression is normally confined specifically to the developing eye and fetal forebrain, although the expression of NR4A3 is largely ubiquitous. Our data suggest that aberrant coexpression of NOR1 and SIX3 is a potential alternative mechanism underlying the development of EMC.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
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