Although differentiation of
Wegener's granulomatosis from other destructive midline lesions is now clearly recognized by clinicians and pathologists,
confusion persists regarding the management of what has erroneously been called "
lethal midline granuloma." The experiences of 36 patients with destructive midline
granuloma supports the view that probably all are manifestations of
malignant lymphoma and should be treated as such. Although radical
dosage radiotherapy will control most local lesions, dissemination of
lymphoma may still occur despite absence of systemic disease at initial diagnostic work-up. Subsequent control with cytotoxic drugs is frequently unsuccessful, and it is suggested that
chemotherapy should be given routinely to every patient with primary nasal
lymphoma and possibly to those with
polymorphic reticulosis or
necrosis with atypical cellular exudate (NACE).