Mycosis fungoides

Mycosis fungoides (MF) (also known as cutaneous T-cell lymphoma) is a type of malignant T-cell lymphoma that primarily involves the skin. 

Epidemiology

In the United States it is more common in males and in African american. In Europe it accounts for approximately 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 dermatitic skin lesions before the diagnosis is finally established. The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumours.

Pathology

Mycosis fungoides is an 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 extra cutaneous involvement includes

• lung ^7-8 : 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 ².

Radiographic features

Imaging features can vary depending on 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 ². 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

• WHO classification of neoplastic diseases of lymphoid tissues
• Sezary syndrome