Clinical Course And Prognosis

Primary cutaneous CD30+ ALCL has a favorable prognosis, with 5-year survival rates of 90% (4,14). Spontaneous regression and age less than 60 years are associated with a better prognosis, while extracutaneous disease and age greater than 60 years have a worse outlook. Cytomorphology (anaplastic or pleomorphic and immunoblastic) seems not to be a prognostic factor (14). Recurrences are observed

Primary cutaneous anaplastic large cell lymphoma

Clinical features

Solitary, rapidly growing, ulcerated nodule Histological features

Cohesive sheets of anaplastic tumor cells Sparse reactive inflammatory infiltrate Immunophenotype

CD2+, CD3+, CD5+, CD4+, CD45RO+, CD30+, ALK-Molecular biology

Clonal TCR rearrangement in majority of cases Treatment

Excision, ionizing radiation, methotrexate, chemotherapy in disseminated forms Clinical course and prognosis

Favorable (> 90% 5-year survival rate)

in up to 40% of the cases (4) and do not require different treatment than initial lesions (15). Extracutaneous spread occurs in 10% of the patients, in particular in those with multiple grouped or multifocal tumor lesions. Regional lymph nodes may be involved, but the survival rate is similar to that of patients with skin lesions only (4). Fatal outcome is observed in up to 12% of the patients. Up to 40% of CD30+ ALCLs show spontaneous regression (16). If complete tumor regression occurs, no further treatment is necessary; usually the rapid tumor growth in the first weeks makes therapeutic intervention necessary.

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