Malignant histiocytosis (histiocytic sarcoma). A (the?) major cause of the 'midline granuloma syndrome'.

Five out of eight consecutive cases with initial symptoms of a 'midline granuloma' were identified as malignant histiocytosis (histiocytic sarcoma) which within 5 months to 4 years led to generalization and death. The three remaining cases also fulfilled the morphological criteria of this type of neoplasia, though these patients are still alive 1/2 to 8 years after diagnosis, possibly as a result of local radiotherapy. The age of the individuals ranged from 18 to 71 years and there was a male preponderance of 7:1. The histiocytic nature of the atypical cells was primarily documented by intense activity of NaF-inhibitable non-specific esterase, of acid phosphatase and of beta-glucuronidase as demonstrated in cryostat sections of formaldehyde-saccharose-fixed fresh biopsy specimens and by the detection of alpha-1-antichymotrypsin, alpha-1-antitrypsin, and lysozyme antigens, in that order of constancy (immunohistochemical examination of formaldehyde-fixed paraffin sections, using the avidin-biotin-peroxidase complex method). There was among the reported cases a considerable heterogeneity with regard to these 'markers'. We conclude that malignant histiocytosis is a (the?) major cause of the 'midline granuloma syndrome'.