Abstract
Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare form of non-Hodgkins lymphoma. Current frontline treatments for pcALCL include surgical resection, anthracycline-based chemotherapy, and/or radiation therapy (RT) depending on disease severity. While brentuximab vedotin (BV) has been used for refractory/relapsed cases, it recently received Food and Drug Administration (FDA) approval for use in combination with chemotherapy for peripheral T-cell lymphomas. In this case report, we utilized a combined modality therapy of RT and BV for a limited stage aggressive pcALCL presentation for which routine management is contraindicated. A 59-year-old man with a history of peripheral vascular disease (PVD) presented with an aggressive pcALCL involving the left inferior eyelid and small ipsilateral level II hypermetabolic lymph nodes at stage IIE. Due to the patient's history of PVD, the tumor's rapid growth, possible lymph node involvement, and eye proximity, BV was chosen as the initial chemotherapy treatment followed by RT. Complete metabolic resolution of the primary cutaneous lesion and lymphadenopathy was reached after BV treatment alone; complete clinical response of the primary tumor was reached following radiation therapy. Relapse occurred within 7 months. Salvage cyclophosphamide, vincristine, etoposide, and prednisone were not effective. Retreatment with BV + RT is currently being used to treat the new lesions. Our case illustrates that a combination of BV and RT can be a safe and effective initial treatment in patients with limited stage pcALCL who cannot tolerate anthracycline-based chemotherapy. Our patient had a complete response but ultimately relapsed; thus larger clinical trials are needed to better understand early-stage disease.