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Pathology - Mycosis fungoides
A low-grade T-cell lymphoma characterized by variably epidermotropic skin-homing T-lymphocytes.
Epidemiology: The most prevalent type of primary cutaneous lymphoma, however it remains a rare condition, impacting 0.3 individuals per 100,000 yearly. • Typically an affliction of maturity, however it may sporadically impact children.
Aetiology: Unknown. Genetics • The course of the disease correlates with chromosomal abnormalities, especially those involving chromosomes 8 and 17.
Presentation • Defined by the progressive emergence of patches, plaques, and tumors on non-sun-exposed skin, mainly in the regions of the buttocks and trunk.
Patches are several extensive (> 10mm) flat erythematous scaly sores.
• Plaques are raised lesions that may arise inside existing patches or develop independently.
• Tumour nodules and, at times, erythroderma may subsequently arise. • Advanced illness may affect the bone marrow, lymph nodes, and visceral organs.
Histopathology The patch stage has a modest upper dermal T-cell infiltrate accompanied by variable epidermotropism. The early stages of the disease are frequently challenging to detect due to overlapping characteristics with many inflammatory disorders. • The plaque stage has a more pronounced, band-like infiltration of T-cells with increased epidermotropism. Aggregates of neoplastic cells within the epidermis are commonly observed (Pautrier microabscesses). The nuclear atypia of the lymphocytes is more pronounced. The tumor stage indicates a more widespread skin infiltrate that may migrate into the subcutaneous fat. Epidermotropism may be diminished. Immunophenotype: Most instances exhibit a T-helper cell phenotype, specifically CD3 + CD4 + CD8–.
Prognosis: The likelihood of progression and mortality is associated with the illness stage at presentation. • The 10-year survival rates are elevated (85–95%) in patch and plaque stage disease, decreasing to 40% in tumor stage, and to 20% with nodal involvement.
A low-grade T-cell lymphoma characterized by variably epidermotropic skin-homing T-lymphocytes.
Epidemiology: The most prevalent type of primary cutaneous lymphoma, however it remains a rare condition, impacting 0.3 individuals per 100,000 yearly. • Typically an affliction of maturity, however it may sporadically impact children.
Aetiology: Unknown. Genetics • The course of the disease correlates with chromosomal abnormalities, especially those involving chromosomes 8 and 17.
Presentation • Defined by the progressive emergence of patches, plaques, and tumors on non-sun-exposed skin, mainly in the regions of the buttocks and trunk.
Patches are several extensive (> 10mm) flat erythematous scaly sores.
• Plaques are raised lesions that may arise inside existing patches or develop independently.
• Tumour nodules and, at times, erythroderma may subsequently arise. • Advanced illness may affect the bone marrow, lymph nodes, and visceral organs.
Histopathology The patch stage has a modest upper dermal T-cell infiltrate accompanied by variable epidermotropism. The early stages of the disease are frequently challenging to detect due to overlapping characteristics with many inflammatory disorders. • The plaque stage has a more pronounced, band-like infiltration of T-cells with increased epidermotropism. Aggregates of neoplastic cells within the epidermis are commonly observed (Pautrier microabscesses). The nuclear atypia of the lymphocytes is more pronounced. The tumor stage indicates a more widespread skin infiltrate that may migrate into the subcutaneous fat. Epidermotropism may be diminished. Immunophenotype: Most instances exhibit a T-helper cell phenotype, specifically CD3 + CD4 + CD8–.
Prognosis: The likelihood of progression and mortality is associated with the illness stage at presentation. • The 10-year survival rates are elevated (85–95%) in patch and plaque stage disease, decreasing to 40% in tumor stage, and to 20% with nodal involvement.
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