Mycosis Fungoides – Symptoms, Stages and Treatment
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, a rare skin cancer that progresses slowly and can affect the skin, lymph nodes, and internal organs.
Wissenswertes über "Mycosis Fungoides"
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, a rare skin cancer that progresses slowly and can affect the skin, lymph nodes, and internal organs.
What is Mycosis Fungoides?
Mycosis fungoides is the most common form of primary cutaneous T-cell lymphoma (CTCL), a rare group of non-Hodgkin lymphomas that originate in the skin. Despite its name, it is not a fungal infection -- the term was coined historically due to the mushroom-like appearance of some skin tumors. The disease predominantly affects older adults and typically follows a very slow (chronic indolent) course over many years or even decades.
Causes and Risk Factors
The exact cause of mycosis fungoides is not yet fully understood. It is believed to involve the malignant transformation of mature CD4-positive T-lymphocytes that home to the skin. Factors that may contribute include:
- Chronic antigenic stimulation of the skin (e.g., from infections or inflammation)
- Genetic mutations within T-cells
- Possible viral involvement (discussed but not proven)
- Environmental exposures such as pesticides or industrial chemicals
Men are affected approximately twice as often as women, and the median age at diagnosis is around 55 to 60 years.
Stages and Symptoms
Mycosis fungoides classically progresses through three main stages, often over many years:
1. Patch Stage
Flat, reddish-brown, often scaly skin patches that closely resemble eczema or psoriasis. These patches are commonly found on non-sun-exposed areas such as the buttocks and trunk. Itching is frequent and can be the predominant complaint.
2. Plaque Stage
Skin lesions become thicker and raised, developing into plaques with a reddish-violet color. Itching may intensify, and diagnosis becomes more straightforward in this stage.
3. Tumor Stage
Raised, mushroom-shaped nodules and tumors appear, which may ulcerate (break open). In this advanced stage, lymph nodes and internal organs may become involved.
A particularly aggressive variant is Sezary syndrome, in which malignant T-cells are detectable in the blood and widespread reddening of the entire skin surface (erythroderma) is present.
Diagnosis
Diagnosing mycosis fungoides can be challenging, as early-stage disease closely resembles common inflammatory skin conditions. The following investigations are typically used:
- Skin biopsy: A tissue sample is taken from affected skin areas for histological and immunohistochemical examination -- this is the most important diagnostic step.
- Immunohistochemistry: Detection of typical T-cell markers (e.g., CD3, CD4, CD8) and loss of CD7 or CD26.
- T-cell receptor clonality analysis: Molecular genetic detection of a clonal T-cell population.
- Blood count and differential: Examination for Sezary cells in the peripheral blood.
- Imaging: CT, PET-CT, or MRI to assess lymph node or organ involvement in advanced stages.
Staging is performed using the TNMB system (Tumor, Node, Metastasis, Blood), specifically developed for cutaneous lymphomas.
Treatment
Treatment depends on the stage of the disease. Since mycosis fungoides often remains in an early stage for many years, quality of life is a central consideration:
Skin-Directed Therapies (Early Stages)
- Topical corticosteroids: Anti-inflammatory creams or ointments
- Topical retinoids: e.g., bexarotene gel
- Mechlorethamine gel (nitrogen mustard)
- PUVA therapy: Combination of psoralen (a photosensitizing agent) and UVA irradiation
- Narrowband UVB phototherapy: Particularly suitable for early patch-stage disease
- Local radiotherapy with electron beam or X-rays
Systemic Therapies (Advanced Stages)
- Bexarotene (systemic): A retinoid X receptor agonist
- Interferon-alpha
- Histone deacetylase (HDAC) inhibitors: e.g., vorinostat, romidepsin
- Mogamulizumab: A monoclonal antibody targeting CCR4
- Brentuximab vedotin: An antibody-drug conjugate for CD30-positive cases
- Chemotherapy (e.g., gemcitabine, liposomal doxorubicin) in late-stage disease
- Allogeneic stem cell transplantation: Potentially curative in selected younger patients with advanced disease
Prognosis
The prognosis strongly depends on the stage at the time of diagnosis. Most patients with early-stage disease have a nearly normal life expectancy. In advanced tumor-stage disease or with organ involvement, the prognosis is considerably worse. A cure is rarely achievable in early stages, but the disease can often be controlled for many years with appropriate treatment.
References
- Willemze R. et al. - EORTC classification for primary cutaneous lymphomas: a proposal from the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer. Blood. 2019.
- Willemze R. et al. - Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines. Annals of Oncology. 2018.
- National Comprehensive Cancer Network (NCCN) - Clinical Practice Guidelines in Oncology: Primary Cutaneous Lymphomas. Version 2024.
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