Mycosis fungoides (MF), also known as cutaneous T-cell lymphoma, is a type of malignant T-cell lymphoma that primarily involves the skin.
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.
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, and later to cutaneous tumours.
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 2,7,8:
- 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
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 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.
- 1. Tien RD, Brown M, Massey EW. CNS mycosis fungoides: CT and MR findings. J Comput Assist Tomogr. 16 (4): 529-33. - Pubmed citation
- 2. Lee HJ, Im JG, Goo JM et-al. Peripheral T-cell lymphoma: spectrum of imaging findings with clinical and pathologic features. Radiographics. 23 (1): 7-26. doi:10.1148/rg.231025018 - Pubmed citation
- 3. Law M, Teicher N, Zagzag D et-al. Dynamic contrast enhanced perfusion MRI in mycosis fungoides. J Magn Reson Imaging. 2003;18 (3): 364-7. doi:10.1002/jmri.10361 - Pubmed citation
- 4. Kulin PA, Marglin SI, Shuman WP et-al. Diagnostic imaging in the initial staging of mycosis fungoides and Sézary syndrome. Arch Dermatol. 1990;126 (7): 914-8. Arch Dermatol (link) - Pubmed citation
- 5. Tsai EY, Taur A, Espinosa L et-al. Staging accuracy in mycosis fungoides and sezary syndrome using integrated positron emission tomography and computed tomography. Arch Dermatol. 2006;142 (5): 577-84. doi:10.1001/archderm.142.5.577 - Pubmed citation
- 6. Shapeero LG, Young SW. Mycosis fungoides: manifestations on computed tomography. Radiology. 1983;148 (1): 202. Radiology (abstract) - Pubmed citation
- 7. Tamai K, Koyama T, Kondo T et-al. High-resolution computed tomography findings of diffuse pulmonary involvement by mycosis fungoides. J Thorac Imaging. 2007;22 (4): 366-8. doi:10.1097/RTI.0b013e3180592af8 - Pubmed citation
- 8. Ueda T, Hosoki N, Isobe K et-al. Diffuse pulmonary involvement by mycosis fungoides: high-resolution computed tomography and pathologic findings. J Thorac Imaging. 2002;17 (2): 157-9. J Thorac Imaging (link) - Pubmed citation
- overview of lymphoma
WHO classification of tumours of haematopoietic and lymphoid tissues
- Hodgkin lymphoma
- mature B-cell lymphoma
- mature T-cell and NK-cell lymphoma
- post-transplant lymphoproliferative/lymphoproliferation disorders
- location-specific lymphomas
- central nervous system
- head and neck lymphoma
- thoracic lymphoma
- gastrointestinal lymphoma
- hepatobiliary lymphoma
- genitourinary lymphoma
- musculoskeletal lymphoma
- cutaneous lymphoma
- lymphoma staging