A clinicopathologic study of malignant lymphomas of the nose, paranasal sinuses, and hard palate, including cases of lethal midline granuloma.

Malignant lymphomas of the nose, paranasal sinuses, and hard palate show marked clinicopathologic, immunologic, and prognostic diversity. The clinical features and pathologic spectrum of these lesions were studied in 20 cases (11 female and 9 male cases) with a mean age of 51 years at initial presentation. Malignant lymphomas of the large cell type were most frequently encountered (11/20). The next largest category was malignant lymphoma, diffuse, mixed small and large cell type (six of 20). Two thirds, 13 of 20 cases, had morphologic features suggestive of peripheral T-cell lymphomas. Necrosis, an angiocentric growth pattern, and epitheliotropism were found in nine, eight, and three cases, respectively. Of ten cases immunophenotyped on fresh-frozen or fixed, paraffin-embedded tissue sections, eight had a T-cell phenotype and two had a B-cell phenotype. Of 17 patients with sufficient follow-up data, ten are alive (median follow-up 33 months) and seven are dead (median survival 12 months). Patients with clinical Stages IE and IIE did not have a superior 5-year survival to those with more advanced disease. Histologic type also did not correlate with survival but this may be due to the aggressive histologic grade of the majority of cases and the retrospective nature of this study. The authors conclude that, despite the overall high-grade histologic type, the pathologic spectrum of malignant lymphomas involving this anatomic region is broad. Furthermore, some cases do not fit well into the National Cancer Institute (NCI) Working Formulation but more closely resemble the histologic features of peripheral T-cell lymphomas described in Japan.