Lethal midline granuloma

Lethal midline granuloma (LMG) is an historical term for a condition in which necrotic and highly destructive lesions develop progressively in the middle of the face, principally the nose and palate. Many cases presented with ulcerations in or perforations of the palate.

Lethal midline granuloma
Other names	Extranodal NK/T-cell lymphoma, nasal type
Specialty	Oncology and hematology
Causes	Epstein–Barr virus

LMG was thought to be a manifestation of three^[1] or four^[2] different diseases: the well-characterized disease of granulomatosis with polyangiitis, the ill-defined disorders of polymorphic reticulosis or mid-line malignant reticulosis, and an incompletely defined form of non-Hodgkin lymphoma. Subsequent studies found that the cells infiltrating the midline tissues in cases of lethal midline granuloma that were not clearly diagnosed as granulomatosis with polyangiitis were: a) infected by the Epstein–Barr virus^[2] and b) consisted of malignant lymphocytes, usually NK cells or, rarely, cytotoxic T cells.^[3] The disease is therefore now regarded as a NK/T cell malignancy, is grouped with other Epstein–Barr virus–associated lymphoproliferative diseases^[4] and is classified by the World Health Organization (2017 update) as a manifestation of the well-defined disease, extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT).

ENKTCL-NT is a rare type of lymphoma that commonly involves the nasal cavity, oral cavity, and/or pharynx^[5] but less commonly can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues.^[6] Patients presenting with highly localized midline facial disease fit the historical definition of lethal midline granuloma. These cases, unlike other cases ENKTCL-NT that have more widespread disease, often show no or relatively little progression of their disease over long periods of time.^[7] Since cases of LMG that were manifestations of granulomatosis with polyangiitis, a vascular inflammatory but not malignant disease, the term lethal midline granuloma is considered confusing and obsolete.^[8]

References

1. Aozasa K, Ohsawa M, Tajima K, Sasaki R, Maeda H, Matsunaga T, Friedmann I (July 1989). "Nation-wide study of lethal mid-line granuloma in Japan: frequencies of wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions". International Journal of Cancer. 44 (1): 63–6. doi:10.1002/ijc.2910440112. PMID 2744899. S2CID 11005815.
2. Hartig G, Montone K, Wasik M, Chalian A, Hayden R (April 1996). "Nasal T-cell lymphoma and the lethal midline granuloma syndrome". Otolaryngology–Head and Neck Surgery. 114 (4): 653–6. doi:10.1016/s0194-5998(96)70264-4. PMID 8643282.
3. Matutes E (May 2018). "The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms". International Journal of Laboratory Hematology. 40 Suppl 1: 97–103. doi:10.1111/ijlh.12817. PMID 29741263.
4. Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein—Barr virus—associated lymphoid proliferations, a 2018 update". Human Pathology. 79: 18–41. doi:10.1016/j.humpath.2018.05.020. PMID 29885408. S2CID 47010934.
5. Yamaguchi M, Oguchi M, Suzuki R (September 2018). "Extranodal NK/T-cell lymphoma: Updates in biology and management strategies". Best Practice & Research. Clinical Haematology. 31 (3): 315–321. doi:10.1016/j.beha.2018.07.002. PMID 30213402. S2CID 52272644.
6. Park S, Ko YH (January 2014). "Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorders". The Journal of Dermatology. 41 (1): 29–39. doi:10.1111/1346-8138.12322. PMID 24438142. S2CID 42534926.
7. Hu B, Oki Y (2018). "Novel Immunotherapy Options for Extranodal NK/T-Cell Lymphoma". Frontiers in Oncology. 8: 139. doi:10.3389/fonc.2018.00139. PMC 5937056. PMID 29761078.
8. Keat Jin Lee (2003). Essential otolaryngology: head & neck surgery. McGraw-Hill Professional. p. 1052. ISBN 978-0-07-137322-7.

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