Recommendations for treatment in Folliculotropic Mycosis Fungoides: Report of the Dutch Cutaneous Lymphoma Group

Summary

Background

Folliculotropic mycosis fungoides (FMF) is an aggressive variant of mycosis fungoides (MF) and generally less responsive to standard skin-directed therapies (SDTs). Recent studies distinguished an indolent (early-stage FMF) and a more aggressive (advanced-stage FMF) subgroup. The optimal treatment for both subgroups needs still to be defined.

Objectives

Evaluation of initial treatment results in patients with early- and advanced-stage FMF.

Methods

203 Patients (84 early-stage, 102 advanced-stage, 17 extracutaneous FMF), included in the Dutch Cutaneous Lymphoma Registry between 1985 and 2014 were studied. Type and results of initial treatment were retrieved from the Dutch Registry. Main outcomes were complete remission (CR), sustained complete remission (SCR), partial remission (>50% improvement; PR) and overall response (OR; CR+PR).

Results

Patients with early-stage FMF were treated with non-aggressive SDTs in 67 of 84 cases, resulting in CR and OR of 28% and 83% for monotherapy topical steroids, 0% and 83% for UVB and 30% and 88% for PUVA, respectively. In patients with advanced-stage FMF these SDTs were less effective (combined CR and OR: 10% and 52%, respectively). In patients with advanced-stage FMF local radiotherapy (CR 63%; OR: 100%), total skin electron beam irradiation (CR: 59%; OR: 100%) and PUVA combined with local radiotherapy (CR: 5%; OR: 75%) were most effective.

Conclusions

The results of the present study demonstrate that not all patients with FMF should be treated aggressively. Patients with early-stage FMF may benefit very well from standard SDTs also used in early-stage classic MF and have an excellent prognosis.

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