15 November 2007 – Case of the Week #101
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We continue to add new books to our Books pages, including these:
Pancreatic Cytopathology (Essentials in Cytopathology)
By David C Chhieng and Edward B Stelow
2007 (1st ed), 204 pages, 172 illus, $60 list
This volume will fulfill the need for an easy-to-use and authoritative synopsis of pancreas cytopathology. This volume, part of the Essentials in Cytopathology book series, fits into the lab coat pocket and is ideal for portability and quick reference.
By Susan King Strasinger
2008 (5th ed), 320 pages, 375 illus, $55 list
This thoroughly updated 5th Edition provides you with concise and carefully structured full-color instruction in the handling and analysis of non-blood body fluids.
By Jacques Wallach
2006 (8th ed), 1200 pages, 109 illus, $70 list
"Arranged by organ system, the book summarizes the available tests for most diseases, explains what the results mean, and discusses differential diagnoses. This thoroughly updated edition includes additional tests based on molecular biology, new data on the use of genetic tests, and many new diagnostic laboratory tests in chemistry.
We thank Dr. Ankur Sangoi, Stanford University, Stanford, California (USA) for contributing this case. To contribute a Case of the Week, please email info@PathologyOutlines.com with the clinical history, your diagnosis and microscopic images in JPG, GIF or TIFF format (send as attachments in any size, we will shrink if necessary). Please include any other images (gross, immunostains, etc.) that may be helpful or interesting. We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US dollars) for your time after we send out the case. Please only send cases with a definitive diagnosis, and preferably cases that are out of the ordinary.
Case of the Week #101
A 40 year woman with nasal sinus congestion had a CT scan, which showed a mass. Endoscopy showed friable nasal tissue. A biopsy was obtained.
What is your diagnosis?
NK / T cell lymphoma, nasal type
The low power images show large areas of necrosis. Higher power images show an angiocentric pattern of pleomorphic tumor cells with hyperchromatic nuclei and occasional perinuclear clearing. There is prominent angiodestruction, although the vessel walls lack neutrophils or fibrinoid necrosis.
The tumor cells are negative for a cytokeratin cocktail, although some residual epithelial cells/vessels are positive. Most tumor cells are immunoreactive for the T cell marker CD3 (cytoplasmic staining), but negative for the B cell marker CD20. There is strong staining for the NK marker CD56, Epstein Barr Virus (by in situ hybridization) and TIA, a marker of activated cytotoxic T cells.
NK / T cell lymphomas (nasal type) were previously called angiocentric lymphoma or polymorphic reticulosis. They commonly present as a destructive nasal or midline facial tumor. In the US they are rare, and usually affect patients of Asian or Hispanic descent (AJSP 2000;24:1511). Outside the US, they are more common in Asia (Ai Zheng 2007;26:1170, Hum Path 2004;35:86), Central and South America (Appl Immunohistochem Mol Morphol 2007;15:38). Non-nasal NK/T cell lymphomas are similar, and often present in the GI tract, skin and testis.
The tumor cells are positive for T cell markers CD2 and CD3 (cytoplasmic but not nuclear staining), as well as CD56, an NK marker. They are almost always EBV positive by immunostaining or in situ hybridization. They are also positive for cytotoxic T cell markers TIA, granzyme and occasionally perforin. However, they are negative for CD16 and CD57, and there is usually no T cell receptor gene rearrangement.
The differential diagnosis includes diffuse large B cell lymphoma (the infiltrating T cells may obscure the neoplastic B cells, AJSP 1999;23:1356) and florid herpes simplex virus of the nasopharynx (CD56+, HSV+, no angioinvasion or angiodestruction, EBV-, polyclonal, Mod Path 2003;16:166).
Treatment includes radiation therapy and chemotherapy, but the overall 5 year survival is only about 50% (Cancer 2006;106:609)
Nat Pernick, M.D., President
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