BACKGROUND: Postthymic/peripheral T-cell malignancy shows significant histopathologic and clinical diversity, even in its prognosis, and the correlations remain to be debated. METHODS: The clinicopathologic features of 212 Japanese patients with these neoplasms were investigated. RESULTS: There were 131 male and 81 female patients, whose ages ranged from 2 to 90 years (mean, 51.7 years). Lymphadenopathy was the most frequent clinical presentation, and the patients also had frequent skin lesions, hyperimmunoglobulinemia, hypercalcemia, and a rapid clinical course. Furthermore, the differences in the histologic features of each subcategory reflected the clinical pictures. The immunophenotypic analysis was indispensable in establishing a correct diagnosis, and the high-grade tumors often showed loss of pan-T antigens. CONCLUSIONS: The histopathologic classification proposed by Suchi et al., which has been incorporated into the updated Kiel classification, showed a good prognostic correlation.
BACKGROUND: Postthymic/peripheral T-cell malignancy shows significant histopathologic and clinical diversity, even in its prognosis, and the correlations remain to be debated. METHODS: The clinicopathologic features of 212 Japanese patients with these neoplasms were investigated. RESULTS: There were 131 male and 81 female patients, whose ages ranged from 2 to 90 years (mean, 51.7 years). Lymphadenopathy was the most frequent clinical presentation, and the patients also had frequent skin lesions, hyperimmunoglobulinemia, hypercalcemia, and a rapid clinical course. Furthermore, the differences in the histologic features of each subcategory reflected the clinical pictures. The immunophenotypic analysis was indispensable in establishing a correct diagnosis, and the high-grade tumors often showed loss of pan-T antigens. CONCLUSIONS: The histopathologic classification proposed by Suchi et al., which has been incorporated into the updated Kiel classification, showed a good prognostic correlation.