Classification and recommended treatment options for folliculotropic mycosis fungoides

ABSTRACT

Introduction: Folliculotropic mycosis fungoides (FMF) is a cutaneous T cell lymphoma that is recognized as a distinct variant of mycosis fungoides (MF) due to its unique clinicopathologic features and its more aggressive disease course as compared to classic MF. Recent studies distinguished an indolent (early stage FMF) and a more aggressive (advanced stage FMF) subgroup, that require a different therapeutic approach.

Areas covered: This review provides an overview of the evolving concepts and recent developments in FMF and summarizes various treatment options for early and advanced stages of FMF including skin-directed therapies, immunomodulating agents, HDAC inhibitors, targeted anti-CD30 therapy and the role of chemotherapy and haematological stem cell transplantation.

Expert opinion: Recently defined prognostic subgroups show that early stage FMF patients have an indolent disease course and may benefit very well from skin-directed therapies, also used in early stage classic MF. Advanced skin limited FMF patients benefit most from radiotherapy based treatment modalities. Systemic chemotherapy is reserved for patients with extracutaneous dissemination or progressive disease that cannot be controlled with skin-directed or immunomodulating therapies.

KEYWORDS:

• Folliculotropic mycosis fungoides
• classification
• management

Article highlights

• Folliculotropic mycosis fungoides (FMF) is a cutaneous T cell lymphoma (CTCL) and a distinct variant of mycosis fungoides (MF) that is characterized by the infiltration of neoplastic T-cells into the hair follicles (folliculotropism) and the preferential localization of skin lesions in the head and neck area.

• Previous studies emphasized that FMF patients have a worse prognosis when compared to classic MF patients and should be treated more aggressively.

• Recent studies showed that not all patients with FMF have an aggressive disease course and distinguished an indolent (early-stage FMF) and a more aggressive (advanced-stage FMF) subgroup.

• Patients with early-stage FMF may benefit from skin-directed therapies, whereas in advanced-stage (skin-limited) FMF patients treatment with radiotherapy-based modalities is most effective.

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Declaration of Interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose

Additional information

Funding

This paper has been funded by LUMC Leiden University Medical Centre.