1/8. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/8. Epstein-Barr virus-associated intravascular large T-cell lymphoma presenting as acute renal failure in a patient with acquired immune deficiency syndrome.Intravascular lymphoma (IVL) is a rare neoplasm, recently included as a specific entity in the world health organization classification of lymphoid tumors. Most cases are of B-cell lineage; however, rare cases of T-cell phenotype have been reported. We report a human immunodeficiency virus (HIV)-positive patient who died of acute renal failure in whom IVL was identified at autopsy, predominantly involving the renal interstitial vessels. Immunohistochemical stains revealed a T-cell phenotype, which was confirmed by T-cell receptor gamma gene rearrangement studies. The lymphoma cells showed nuclear Epstein-Barr virus (EBV)-encoded rna transcripts by in situ hybridization, suggesting that EBV might be of etiologic importance in this tumor. The predominant involvement of kidney is unusual. With effective therapy, morbidity and mortality of hiv-1 infection has been substantially reduced, and survival times have been prolonged. However, the relative risk of secondary neoplasms, especially non-Hodgkin's lymphoma (NHL), has increased. Consequently, we conclude that unique types of NHL, such as this case of IVL, may be encountered more frequently in this patient population, and that NHL should be added to the list of differential diagnostic considerations in hiv-1-positive patients who develop acute renal failure.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/8. Primary renal synovial sarcoma with inferior vena cava and right atrium invasion.Primary renal synovial sarcoma is an uncommon and perhaps under-diagnosed disease. We report a case of renal tumor in a 19-year-old man. Clinically, the tumor mimicked renal cell carcinoma with renal vein, inferior vena cava and right atrium invasion. Histologically, the tumor consisted of monophasic, high-grade, spindle cell components. The diagnosis was validated by fluorescence in situ hybridization and reverse transcription-polymerase chain reaction, which demonstrated SYT-SSX translocation: a characteristic cytogenetic finding for synovial sarcoma. Our case shows that synovial sarcoma should be considered in the differential diagnosis of renal tumors, especially in adolescents and young adults. Proper molecular analysis should be undertaken to attain a definitive diagnosis.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/8. Pyothorax-associated lymphoma (PAL): a western case with marked angiocentricity and review of the literature.AIMS: To report a case of pyothorax-associated lymphoma in a non-immunocompromised 78-year-old man with a 45-year history of tuberculous pleuritis and left pleural effusion. Pyothorax-associated lymphoma is a high-grade non-Hodgkin's lymphoma occurring in 2% of patients with long-standing tuberculous pleuritis and pyothorax. Pyothorax-associated lymphoma is frequently Epstein-Barr virus (EBV)-associated, mainly reported in japan but exceedingly rare in western countries. methods AND RESULTS: histology revealed a high-grade, diffuse large B-cell lymphoma with immunoblastic and plasmacytoid features and marked angiocentricity with focal destruction of the vessel walls. immunohistochemistry revealed a post germinal B-cell phenotype. rna in-situ hybridization and molecular analysis showed a latent EBV infection and absence of human herpes virus-8 (HHV-8). CONCLUSIONS: Pyothorax-associated lymphoma represents a rare but distinctive type of diffuse large B-cell lymphoma, with characteristic clinico-epidemiological, immunohistological, and biological features.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/8. cavernous sinus and leptomeningeal metastases arising from a squamous cell carcinoma of the face: case report.OBJECTIVE AND IMPORTANCE: Invasion of trigeminal and facial perineural spaces is a recognized complication of cutaneous malignancies. Centripetal spread along the trigeminal nerve axis and into the cavernous sinus and the gasserian ganglion is rare. Metastasis to the leptomeninges and cauda equina has not been reported. We report a unique case of perineural spread and central dissemination from an epithelial squamous cell carcinoma (SCC) associated with a tumor biomarker. CLINICAL PRESENTATION: After excision of multiple cutaneous SCCs and basal cell carcinomas of the head and neck, a 70-year-old male patient developed successive, right-side, V1 and V2 trigeminal neuropathies and complete right cavernous sinus syndrome during a 5-year period. Concurrently, the right face became paralyzed. Left facial paresis developed during the latter half of this period. Two months before admission, subacute left lower-extremity radicular weakness resulted in falls. Serial magnetic resonance imaging scans obtained in the previous 4 years were unrevealing. At the time of admission, enhancing masses were found in the 1) right cavernous sinus and dura, foramina ovale and rotundum, and Meckel's cave, 2) right subtemporal region and orbital rectus muscles, and 3) cauda equina. cerebrospinal fluid analysis demonstrated mild pleocytosis and rare carcinoma cells. INTERVENTION: biopsy of the right cavernous sinus mass confirmed moderately differentiated, metastatic SCC. Immunohistochemical staining and fluorescence in situ hybridization revealed epidermal growth factor receptor overexpression and genomic amplification. CONCLUSION: The indolent progression of cranial nerve palsy among patients with resected cutaneous SCCs of the head and neck must raise clinical suspicion of perineural spread, even in the absence of radiological changes. Biomarkers predicting aggressive SCC behavior, illustrated here by epidermal growth factor receptor amplification and central invasion, have the potential to guide early therapy.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/8. Cutaneous intravascular NK-cell lymphoma: report of a rare variant associated with Epstein-Barr virus.Intravascular lymphoma (IVL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin and brain. Except a few cases of T-cell lineage, most of the reported cases were large B-cell lymphomas. We encountered a case of cutaneous IVL in a 71-year-old woman presenting with multiple erythematous patches and nodules on her trunk and extremities. The intravascular large cells showed an immunophenotype of CD3epsilon( );, CD5(-), CD20(-), CD30(-), CD56( ), and TIA-1( ). The lymphoma cells were also positive for Epstein-Barr virus by Epstein-Barr virus-encoded rna in situ hybridization test and the T-cell receptor gene was germline. This IVL differs from nasal type NK/T-cell lymphoma only by its intravascular nature. Only 3 cases of intravascular NK-cell lymphoma have been reported before. Because this variant is extremely rare, our case is documented and compared with the 3 previously reported cases.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/8. Intravascular large B-cell lymphoma associated with a near-tetraploid karyotype, rearrangement of BCL6, and a t(11;14)(q13;q32).Chromosome analysis of a patient with intravascular large B-cell lymphoma (IVL) revealed a complex, near-tetraploid karyotype with 83 chromosomes. Abnormalities included a t(11;14)(q13;q32), which was confirmed with both interphase fluorescence in situ hybridization (FISH) using an IGH/cyclin d1 dual-color, dual-fusion probe set and cyclin d1 immunohistochemical analysis. Abnormality of 3q was also evident. interphase FISH analysis with a dual-color, break-apart probe set confirmed rearrangement of BCL6. To our knowledge, this is the first report of these abnormalities in IVL.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/8. Primary intravascular synovial sarcoma: a disease of young adult women? Report of a case diagnosed by aspiration biopsy and review of the literature.Intravascular synovial sarcoma (IVSS) is an extremely rare tumor with only four well-documented cases in the English literature. All tumors were located in large veins of the lower extremities or trunk in young women except for one case occurring in a 54-yr-old woman. We report here an additional case of IVSS arising from the superior vena cava in a 32-yr-old woman who presented with a cervical mass and superior vena cava syndrome. A fine-needle aspiration biopsy (FNAB) was performed and showed a malignant biphasic tumor with spindle cell and epithelioid components. The tumor cells were negative for CD31, CD34, factor viii, desmin, smooth muscle actin, and S-100 protein, and had positive staining for vimentin and cytokeratin (AE1/AE3) predominantly in the spindle and epithelial components, respectively. A diagnosis of synovial sarcoma was made and confirmed in a subsequent transvascular biopsy demonstrating chromosomal translocation t(X, 18) by fluorescence in situ hybridization using a dual color, break-apart-style probe for SYT. Although clinically similar to previously reported IVSS, this is the first case arising in large veins of the upper portion of the trunk and diagnosed by FNAB.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |