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PubMed · 446817

[Lethal midline granuloma. A case report].

Abstract

In their study of a case of lethal midline-granuloma, the authors describe the relations between this affection, Wegener's granulomatosis and lymphoma. The case under review illustrates the difficulty in accurately defining the nosologic picture of the disease. Anatomo-pathological examinations present two possibilities: one being malignant granuloma of the Stewart type, the other that of lymphoma. From its subsequent evolution, we may eventually learn whether this is purely a local illness or represents the onset of a systemic disease.

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BibTeXRIS

A Simonis-Blumenfrucht, C Mestdagh, F Lustman, J Wanet-Rouard, D Balikdjian. 1979. [Lethal midline granuloma. A case report].. https://pubmed.ncbi.nlm.nih.gov/446817/

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Extranodal NK/T-cell lymphoma, nasal type.

Extranodal NK/T-cell lymphoma, nasal type (ENKTCL), previously known as lethal midline granuloma is a distinct clinico-pathological entity associated with Epstein-Barr virus that typically causes destruction of the midface, palatal and orbital walls. In addition, ENKTCL can involve the skin, soft tissue, testes, gastrointestinal and upper respiratory tract. ENKTCL neoplastic cells express some T-cell associated antigens, most commonly CD2 and cytoplasmic CD3epsilon and, in favour of an NK-cell origin, CD56. Early stage disease may respond to radiotherapy alone, however late stage disease does not respond well to any available therapies. Overall, patients with ENKTCL have a cumulative probability of survival at 5 years ranging from 37.9% to 45.3%.

Granuloma, Lethal Midline↗