Current Concepts of the Lethal Midline Granuloma Syndrome

Abstract

"Lethal midline granuloma" is an unusual but devastating spectrum of lymphoproliferative disorders that is now thought to include four entities: idiopathic midline destructive disease, Wegener's granulomatosis, polymorphic reticulosis, and nasal lymphoma. These disorders must be considered in the differential diagnosis of any patient with chronic, nonspecific complaints localized to the upper airway. An aggressive diagnostic and therapeutic approach may significantly decrease morbidity and death resulting from these lesions.

Angiocentric T‐Cell Lymphoma of the Temporal Bone

“…At present, these lesions are placed into 4 categories that comprise the lethal midline granuloma syndrome: idiopathic midline destructive disease, Wegener's granulomatosis, polymorphic reticulosis, and non-Hodgkin's lymphoma. 6 Recent reports have provided evidence that PR may be an angiocentric lymphoma. First, the morphologic features of PR closely resemble the histologic features of angiocentric T-cell lymphomas found in other locations.…”

“…In the past they have been described pathologically as destructive granulation tissue, chronic inflammation, true neoplasms, and autoimmune phenomena. 6 Names have ranged from midline granuloma, granuloma gangreanescens, and idiopathic granuloma of the face, to malignant reticulosis, mutilating granuloma, and osteomyelitis necroticans. At present, these lesions are placed into 4 categories that comprise the lethal midline granuloma syndrome: idiopathic midline destructive disease, Wegener's granulomatosis, polymorphic reticulosis, and non-Hodgkin's lymphoma.…”

Orbital cellulitis or lymphoma? A diagnostic challenge

“…As an intense inflammatory appearance complicates the histologic picture with proliferation of histiocyes induced by the neoplastic process, repeat biopsies are often required. 7 Clinicians must have very good knowledge and take into account the clinical features, serology and immunohistochemical techniques in order to take a rational approach to diagnosis and therapy. Perhaps an aggressive disease of this nature should mandate a high degree of suspicion from the outset to exclude a neoplastic pathology.…”

“…The possibility of dissemination must always be entertained, and appropriate studies (including peripheral blood smear, liver-function tests, and CT scans of the skull, chest, and abdomen) should be carried out. 7 Finally, clinical alertness, along with adequate tissue sampling, is indispensable for correct diagnosis and treatment.…”

Vasculitis granulomatosa centrofacial: estudio de seis casos y propuesta para su clasificación

“…Con diagnósti-co de sinusitis crónica agudizada, recibió tratamiento con cefalosporina de primera generación con mejoría parcial de sus síntomas. Desde entonces empezó a presentar fiebre vesperal, escalofríos, diaforesis, pérdida de peso y, dos mesesdespués, toscon expectoración hemoptoica y deformidad progresiva del dorso nasal con nariz en silla de montar (figura 3). No existían antecedentes previos de importancia.…”