Definition
Lethal midline granuloma (LMG) or midline malignant polymorphic reticulosis is characterized by progressive, unrelenting ulceration and necrosis of the midline facial tissues.
LMG is associated with Epstein-Barr virus. Despite considerable controversy, three clinicopathologic entities remain identified: Wegener’s granulomatosis, LMG, and polymorphic reticulosis (PMR). A review of the literature suggests that cases described as idiopathic midline destructive disease and PMR are an evolutionary spectrum from almost benign to fatal malignant lymphoma. Wegener’s granulomatosis is an epithelioid necrotizing granulomatosis with vasculitis of small vessels. Systemic involvement of the kidneys and lungs is common.
PMR is an unusual disorder, characterized by atypical mixed lymphoid infiltration of the submucosa with necrosis, sometimes extending to bone or cartilage. PMR has been considered a lymphoproliferative disorder; most, if not all, cases are peripheral T-cell lymphomas....
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References
Fauci AS, Hayes BF, Costa J et al (1982) Lymphomatoid granulomatosis: prospective clinical and therapeutic experience over 10 years. N Engl J Med 306:68–74
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Perez, C.A., Thorstad, W.L. (2013). Lethal Midline Granuloma. In: Brady, L.W., Yaeger, T.E. (eds) Encyclopedia of Radiation Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-85516-3_557
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DOI: https://doi.org/10.1007/978-3-540-85516-3_557
Publisher Name: Springer, Berlin, Heidelberg
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