Midline malignant reticulosis (so-called lethal midline granuloma)

Abstract

“Lethal midline granuloma” should be only a clinical term for rapidly evolving destructive lesions of the nose and the deep facial tissues. Such clinical presentation—after specific infections and carcinomas have been excluded—may occur in 3 different clinicopathologic entities: midline malignant reticulosis (polymorphic reticulosis), Wegener's granulomatosis, and malignant lymphoma of one of the accepted histologic types. One representative case of each of these is reported here, histologic criteria for differential diagnosis are outlined, and the literature is reviewed. Midline malignant reticulosis is defined as a lymphoproliferative disorder involving initially the nasal and the paranasal soft and bony parts and capable of later dissemination. Treatment should be based on the pathologic diagnosis made on viable biopsy tissue. Midline malignant reticulosis may have a better prognosis than malignant lymphoma of the nasal cavity when treated with tumoricidal doses of irradiation. On the other hand, Wegener's granulomatosis may respond to immunosuppresive drugs.

Non-healing granulomata of the upper respiratory tract.

“…My own studies of the available evidence and experience of a group of s-ich patients substantiates the view of Kassal et al 16 Provided diagnosis is early the prognosis of patients with such a "neoplastic change" remains better than when a malignant lymphoma occurs de novo in the nose. All nine patients reported by EicheP17 in 1966 survived at least five years after curative radiotherapy, and my own three patients all showed a favourable response.…”

“…Kassal et al 16 suggested that non-healing granuloma should be regarded as a form of maliznant midline reticulo'is. the disease usually remaining localized but eventually overcoming immunological control with possible visceral dissemination.…”

Serum Levels of Interleukin-9 Correlate with Negative Prognostic Factors in Extranodal NK/T-Cell Lymphoma

“…ENKTL was previously known as lethal midline granuloma because, macroscopically, the tumor looked like a necrotic granuloma, and the disease showed an aggressive and lethal course [30], [31]. Histologically, ENKTL is characterized by angiocentric and polymorphous lymphoreticular infiltrates, known as polymorphic reticulosis [31], [32].…”

Deciphering the role of Epstein-Barr virus in the pathogenesis of T and NK cell lymphoproliferations

“…A subsequent report in 1921 [88] described two patients with destructive nasal lesions, in whom syphilis was excluded as a diagnosis and no infectious organism could be identified. Further clinical and histological accounts of such a disease accumulated over the following decades [89-95]. Various terminologies for ENKTL have been used, including: lethal midline granuloma, rhinitis gangrenosa progressiva, polymorphic reticulosis, and malignant midline reticulosis.…”