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Therapeutic Approach

Primary cutaneous anaplastic large cell lymphomas (PC-ALCL) represent 8% of all cutaneous T-cell lymphomas. PC-ALCL often follows an indolent course with an excellent prognosis, although relapses are common. Therapy is based on whether the patient presents with solitary or grouped lesions, multifocal lesions (20%), or PC-ALCL with extracutaneous, usually regional lymph node, involvement (10%). Surgical excision is used for solitary localized lesions only, while involved site radiation therapy (ISRT) is recommended for localized or grouped lesions. Systemic therapy, beginning with methotrexate, is given for multifocal lesions or PC-ALCL with extracutaneous disease. Relapses are treated with the same initial regimen unless refractory disease or intolerance develop. Treatment for refractory disease is not standardized; in these cases, other systemic therapies, including oral bexarotene and brentuximab vedotin, and referral to clinical trial should be considered. See Table 115-1.

Table 115-1Cutaneous Anaplastic Large Cell Lymphoma Treatment Table

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