We describe a rare case of adult T-cell leukemia characterized by an expansion of CD4+ CD8+ double-positive lymphocytes associated with human T-lymphotropic virus type 1 (HTLV-1) and a complex karyotype in a 43-year-old Caribbean male who was initially admitted to our hospital with significant lethargy, visual disturbances, dysphagia, right facial palsy and numbness in both feet for 3 days. He was found to have severe hypercalcemia (15.6 mg/dl). Peripheral blood smear showed multilobulated clover-shaped nuclei. Bone marrow and CSF flow cytometries revealed abnormal monoclonal expansion of T cells positive for CD4, CD5, CD8 and CD25 but negative for CD7, CD20, CD56, CD68 and terminal deoxynucleotidyl transferase. The polymerase chain reaction analysis showed a distinct band of the T-cell receptor γ gene, revealing T-cell clonal integration of the proviral DNA of HTLV-1, thus confirming the diagnosis of acute adult T-cell leukemia/lymphoma. Cytogenetic study revealed a male karyotype with monosomy 12, unbalanced translocation 5q and 13q and additional material on 5q, 7q, 14q and 17q. The patient underwent prednisone (EPOCH) chemotherapy followed by autologous transplantation with BEAM regimen. Although patients with a rare mixed CD4+ CD8+ immunophenotype usually present with an aggressive clinical course and have a poor prognosis, our patient was able to survive for 2.5 years.
Bladder cancer usually spreads via the lymphatic and hematogenous routes, the most common sites of metastases of urinary bladder cancers being the regional lymph nodes, liver, lung, bone, peritoneum, pleura, kidney, adrenal gland and intestines. Generalized lymph node metastasis of transitional cell cancer of the bladder is extremely rare. According to our literature search, there has been no case report of transitional cell cancer of the bladder that manifests as an extensive large lymph node metastasis involving the intraparotid, supraclavicular thoracic inlet, axillary and regional abdominal and pelvic lymph nodes without bone or visceral organs involved. Such a presentation could be mistaken as malignant lymphoma and the importance of a biopsy of the lymph nodes is emphasized. The clinical course of rapid progression of the disease and the presence of wild-type p53 with rapid response to chemotherapy and a short remission may represent a unique case, which is discussed here.
Head and neck squamous cell cancers (HNSCCs) represent 4 to 5% of all solid malignancies. Despite improvements in diagnostic techniques, 60% of patients will present with locally advanced HNSCCs with a median survival of about 12 months and 5-year overall survival of approximately 10-40%. Recent clinical trials have altered the treatment landscape by refining existing forms of radiation, incorporation of IMRT, choice of chemotherapeutic agents, introduction of biological and targeted therapy, immunotherapy and gene therapy. Cetuximab, a monoclonal antibody directed against the human epidermal growth factor receptor (EGFR), has recently been approved in combination with RT in patients with locally advanced HNSCCs. Antiangiogenic therapies and tyrosine kinase inhibitors (gefitinib and erlotinib) have also shown promise in the clinical trials. Vandetanib, an antagonist of both vascular endothelial growth factor receptor (VEGFR) and the EGFR is currently being tested in phase II trial. New patents on hypoxia-inducible factor 1 alpha, mesenchymal-epithelial transition factor, insulin-like growth factor or the PI3K/AKT/mTOR pathway, farnesyl transferase inhibitors have shown promise in the management of HNSCCs. Nevertheless, identification of predictive biomarkers of resistance or sensitivity to these therapies remains a fundamental challenge in the optimal selection of patients most likely to benefit from them. However, increase in efficacy comes at the cost of increased toxicity. The current review focuses on insight into recent patents and updates on the clinical trials using new investigational agents in the management for HNSCCs.
Sporadic Burkitt lymphoma (BL) accounts for 1% to 2% of lymphomas in adults. The abdomen is the most common site of involvement. The 2008 World Health Organization Classification of Tumors of Haematopoietic and Lymphoid Tissues introduced a new category of high-grade B-cell lymphomas: an entity with overlapping features of both diffuse large B-cell lymphoma and Burkitt lymphoma (DLBCL/BL). These lymphomas reportedly have poor overall survival rates. We present a case of an elderly adult atypical sporadic BL with jaw and bone marrow involvement. The tumor had the typical characteristic of BL including atypical monotonous lymphoid infiltrate with a starry-sky appearance and a high Ki67 proliferate index of 95%. MYC/IgH rearrangement was seen in 93.5% of the cells with 92% of cells also positive for IgH/Bcl-2, a feature of double-hit lymphoma. This atypical case of adult sporadic BL had features as classified of DLBCL/BL. Though high intensity regimen is used in BL and atypical BL, elderly patients tend to not tolerate these regimens well. Our patient was treated with bendamustine and bituximab given herelderly age and poor cardiac status. The patient responded well to chemotherapy and remains in complete remission two years after diagnosis up to the date of this report. The atypical presentation and excellent response to bendamustine plus rituximab has not been previously reported.
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