images in clinical medicineT h e n e w e ng l a n d j o u r na l o f m e dic i n e n engl j med 367;22 nejm.org november 29, 2012 2133A 63-year-old man presented with a 6-month history of fatigue, weight loss, and gingival bleeding. Physical examination suggested the presence of a massively enlarged spleen, a finding confirmed on a reconstructed coronal computed tomographic image of the abdomen (Panel A, arrowheads). A complete blood count revealed thrombocytopenia (platelet count, 27,000 per microliter; reference range, 150,000 to 400,000) and lymphocytosis (lymphocyte count, 8900 per microliter; reference range, 1000 to 4800). Microscopical examination of a bone marrow aspirate revealed abnormal lymphocytes with abundant light-blue cytoplasm, round nuclei, fine chromatin, and irregular projections of the cell membrane (Panel B, arrows). Similarly abnormal lymphocytes were also evident on microscopical examination of a peripheral-blood smear. Immunohistochemical staining was positive for tartrate-resistant acid phosphatase. Immunophenotyping by means of flow cytometry was positive for CD19, CD20, CD11c, CD25, and CD103 and negative for CD5 and CD10. These findings confirmed a diagnosis of hairy-cell leukemia. Named for the microscopical appearance of its malignant cells, this uncommon, indolent B-cell non-Hodgkin's lymphoma often presents with cytopenias, systemic symptoms, and massive splenomegaly. This patient had a complete remission after treatment with the purine analogue cladribine. He remained in remission at his last follow-up, at 9 months, with normal peripheral-blood counts, a normal bone marrow aspirate, and no evidence of splenomegaly.

Radiation therapy (RT) is the standard of care for cases of superior vena cava (SVC) syndrome secondary to metastatic adenopathy. Histologies vary in radiosensitivity and response time, making alternative therapies such as chemotherapy and/or intravenous stenting preferable alternative options for certain diagnoses. Metastatic urothelial carcinoma is a particularly rare cause of SVC syndrome with only 3 cases reported in the literature. Consequently, optimal management remains challenging, particularly in cases of high tumor burden. Here we present a case of highly advanced metastatic urothelial cancer with SVC syndrome and tracheal compression. The patient started urgent RT but expired midway through her treatment course due to systemic progression of disease, requiring SVC and tracheal stenting. The authors review the literature including discussion of the few other known cases of SVC syndrome due to urothelial carcinoma and a review of this histology's response to RT. This experience suggests, that in cases of SVC syndrome with widespread advanced disease, stenting and chemotherapy with or without RT may be the most important initial treatment plan, depending on goals of care.

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