Mycosis fungoides: developments in incidence, treatment and survival

Abstract

Background Prior studies have demonstrated improved disease-specific survival of mycosis fungoides (MF) patients over the last 50 years. Objective To analyse patterns of survival and incidence from 1973 to 2016 and determine whether apparent improvements in MF-specific survival are due to lead-time bias rather than improvements in treatment. Methods We performed an analysis of 10 155 patients diagnosed with MF from 1973 to 2016 in the United States cancer registries of SEER-18. We also performed a literature review of papers including stage data for unselected populations of MF patients prior to 2000. Results Incidence of MF increased from 3.0 per million person-years in the 1970s to 5.9 in the 2010s. For all cohorts, non-Hodgkin lymphoma (including MF) was the leading cause of death. Survival analysis demonstrated marked improvement in disease-specific and overall survival from the 1970s to 2010s. Based on systematic review of the literature, 32%-73% of patients diagnosed prior to 2000 were diagnosed with early-stage disease, as opposed to 81% of patients in the SEER 2000-2016 cohort (P < 0.035 for all cohorts). Conclusions Although there have been improvements in MF-related survival over the last 50 years, these may reflect improvements in our ability to diagnose early-stage disease rather than improved treatment.

Association of marital status with stage and survival in patients with mycosis fungoides: A population‐based study

“…Mycosis fungoides (MF), characterized by a monoclonal proliferation of CD4‐positive T cells, is the most common type of cutaneous T‐cell lymphoma (CTCL) and constitutes almost 50% of all primary cutaneous lymphomas. 1 According to a newly published study, the incidence of MF increased from 3.0 per million person‐years in the 1970s to 5.9 in the 2010s, 2 with a higher incidence in Blacks. 3 …”

“…It has been reported that the survival rates are associated with the stages and patients presenting with an early stage at diagnosis have a better prognosis. 2 , 8 , 9 , 10 Therefore, early diagnosis is essential to improve the prognosis of patients with MF.…”

Pigmented purpuric dermatoses versus purpuric mycosis fungoides: Clinicopathologic similarities and new insights into dermoscopic features

“…Mycoses fungoides is a rare cutaneous lymphoma, and even fewer cases are present in its purpuric variant (PMF) 13 . Some authors have suggested that PPD may precede mycosis fungoides over the years 7,14 ; nevertheless, mycosis fungoides may initially manifest with purpuric eruptions as in PMF 15 .…”

“…Mycoses fungoides is a rare cutaneous lymphoma, and even fewer cases are present in its purpuric variant (PMF). 13 Some authors have suggested that PPD may precede mycosis fungoides over the years 7,14 ; nevertheless, mycosis fungoides may initially manifest with purpuric eruptions as in PMF. 15 Clinical and histopathological features of PPD and PMF mimic each other considerably and make the differentiation of these entities challenging.…”

Evaluation of Melanocyte Loss in Mycosis Fungoides Using SOX10 Immunohistochemistry

“…Clonal T-cell receptor (TCR) gamma gene rearrangements, that may be determined by PCR-based amplification and gel electrophoresis, support the diagnosis [ 2 ]. MF usually follows an indolent course but may progress from a patch stage to plaque to tumor and disseminated disease [ 3 ]. There is generally good clinicopathologic correlation for these stages of disease and they display distinct findings histologically [ 4 ].…”