Lethal midline granuloma

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

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.

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]

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Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, and/or pharynx At these sites, the disease often takes the form of massive, necrotic, and extremely disfiguring lesions. However, ENKTCL-NT can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. ENKTCL-NT mainly affects adults; it is relatively common in Asia and to lesser extents Mexico, Central America, and South America but is rare in Europe and North America. In Korea, ENKTCL-NT often involves the skin and is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.

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Epstein–Barr virus–associated lymphoproliferative diseases are a group of disorders in which one or more types of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells, are infected with the Epstein–Barr virus (EBV). This causes the infected cells to divide excessively, and is associated with the development of various non-cancerous, pre-cancerous, and cancerous lymphoproliferative disorders (LPDs). These LPDs include the well-known disorder occurring during the initial infection with the EBV, infectious mononucleosis, and the large number of subsequent disorders that may occur thereafter. The virus is usually involved in the development and/or progression of these LPDs although in some cases it may be an "innocent" bystander, i.e. present in, but not contributing to, the disease.

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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. 1 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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