Mycosis fungoides
Mycosis fungoides (MF) is a type of malignant cutaneous primary T-cell lymphoma.
Epidemiology
In the United States, it is more common in males and African Americans. In Europe, it accounts for ~6% of all non-Hodgkin lymphomas. It is rare in Asian countries.
Clinical presentation
It is usually initially seen by dermatologists with patients presenting with skin lesions. Patients often have a history of several years of eczematous or dermatitis skin lesions before the diagnosis is finally established. The skin lesions then progress from the patch stage to the plaque stage, and later to cutaneous tumors.
Pathology
Mycosis fungoides is initially an indolent lymphoma but in its later stages can cause peripheral lymphadenopathy and can finally progress to widespread extracutaneous visceral/internal organ involvement.
Examples of extracutaneous involvement include :
- lung: pulmonary involvement with mycosis fungoides
- central nervous system: CNS mycosis fungoides
- from autopsy series, CNS involvement of MF can be seen in 14% of cases
Radiographic features
Imaging features can vary depending on the type of organ involvement. If the diagnosis is given on the requisition form, specifically look for skin thickening or cutaneous lesions.
Treatment and prognosis
The prognosis heavily depends on the stage if disease where extracutaneous involvement causes a dramatic change in the 5-year survival rate, which drops from 90% with skin disease to 50% with nodal involvement and to near 0% with systemic spread .
Patients have been treated with radiation therapy, topical glucocorticoids, topical nitrogen mustard, phototherapy, psoralen with ultraviolet A, electron beam radiation, interferon, and systemic cytotoxic therapy. Unfortunately, most of these treatments tend to be palliative.
See also
Siehe auch:
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