General
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• Formerly called angiocentric lymphoma, polymorphic reticulosis
• Commonly presents as lethal midline granuloma (destructive nasal or midline facial tumor)
• Common lymphoma subtype at this location in Asia (Ai Zheng 2007;26:1170, Hum Pathol 2004;35:86), Central and South America (Appl Immunohistochem Mol Morphol 2007;15:38)
• Rare in US; patients are often of Asian or Hispanic descent (Am J Surg Pathol 2000;24:1511)
• Highly associated with EBV
• 5 year survival of 50% (Cancer 2006;106:609); commonly relapses to skin or subcutaneous tissue

Case reports
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• 34 year old man whose post-radiotherapy lymphocytes were morphologically normal, but EBER1+ by ISH (Arch Pathol Lab Med 2002;126:602)
• 40 year woman with nasal sinus congestion (Case of the Week #101)
• 50 year old HIV+ African man (Arch Pathol Lab Med 2001;125:660)

Treatment
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• Radiotherapy, chemotherapy

Gross description
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• Usually perforated nasal septum, perforated palate, erosion of antral bone or ulceration of skin overlying nose or antrum

Micro description
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• Polymorphic infiltrate of lymphocytes (small to large), plasma cells, neutrophils and scattered atypical lymphoid cells with perinuclear clearing
• Frequent angiolymphatic invasion and necrosis, although vessel is usually infiltrated by atypical lymphocytes with no neutrophils and no fibrinoid necrosis present
• Often epitheliotropism
• Frequent histiocytes with erythrophagocytosis

Micro images
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Extensive necrosis

Pleomorphic tumor cells with necrosis

 

Tumor cells invade vessel walls without fibrinoid necrosis

Keratin negative, with positive internal control

CD20 negative

CD3+

 

CD56+

EBV+ (by ISH)

TIA+

 

Various images

Prior to radiotherapy

Post-radiotherapy cardiac involvement

Positive stains
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• CD2, CD3 (cytoplasmic), EBER-ISH (100%), CD56 (65%)
• p53 (50%), CD45RO, CD43, TIA1, granzyme B, perforin (35-100%), CD8 (20%), EBV LMP1 (48%)

Negative stains
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• CD3 (nuclear), CD16 (positive on histiocytes but not tumor cells), CD57
• CD79a (usually)

Molecular description
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• No T cell receptor gene rearrangement, no immunoglobulin light or heavy chain rearrangements

Molecular images
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Monoclonal proliferation of EBV+ cells

Differential diagnosis
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• Diffuse large B cell lymphoma: infiltrating T cells may obscure neoplastic B cells (Am J Surg Pathol 1999;23:1356)
• Florid HSV infection: HSV+, EBV-, no angioinvasion or angiodestruction, polyclonal (Mod Pathol 2003;16:166)
• Idiopathic midline destructive disease
• Wegener granulomatosis

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