Mycosis fungoides with extracutaneous dissemination: A distinct clinicopathologic entity

Abstract

The clinical and histopathologic findings in 15 autopsied cases of mycosis fungoides with extracutaneous dissemination were reviewed. Mycosis fungoides involvement of the skin was present for an average of 22 months (range of 7 months to 6 years) from the time of diagnostic skin biopsy until the onset of clinical signs indicating systemic involvement. The clinical findings associated with dissemination of mycosis fungoides were fever, weight loss, generalized peripheral and hilar lymphadenopathy, palpable enlargement of the liver and spleen, and peripheral blood eosinophilia and lymphocytosis. The average survival from the development of these signs was 11 months (range of 3 to 18 months). At autopsy in all 15 cases cutaneous mycosis fungoides was associated with malignant lymphoma, mycosis fungoides type, involving lymphoid organs and viscera. The cellular infiltrates within extracutaneous sites of involvement were similar to those of the skin infiltrates and included variable numbers of pleomorphic mycosis cells admixed with many inflammatory cells. In none of the cases was there evidence of transition to a malignant lymphoma of a different cell type. Extracutaneous dissemination is a clinicopathologically distinct aspect of the natural history of mycosis fungoides and is not due to the development of a different type of malignant lymphoma.