Primary Cutaneous Anaplastic Large Cell Lymphoma (PC-ALCL) is a CD30+ peripheral T-cell lymphoproliferative disorder without systemic involvement. It accounts for approximately 8% of cutaneous lymphoma cases. Most patients with PC-ALCL present with slow-growing, solitary or grouped skin nodules, and in some cases, regional lymph node involvement is observed.

A 61-year-old male patient presented to our clinic with swelling and edema of the right lower lip, along with a 57 cm draining ulcerative skin lesion in the suprasternal region. The patient had previously received six cycles of treatment for T-cell lymphoma at an outside center. A PET-CT scan identified a soft tissue lesion in the skin/subcutaneous tissue at the level of the right thyroid lobe and isthmus, measuring 57*15*52 mm, with a maximum SUV of 34. Biopsies taken from the lower lip and suprasternal skin were reported as primary cutaneous anaplastic large cell lymphoma. CD30 expression was found to be 95%. The patient was started on brentuximab vedotin along with the GDP protocol. After two cycles of treatment, improvement in the skin lesions was observed.

A 61-year-old male patient presented to our clinic with a complaint of a lesion on the anterior surface of the right tibia, measuring approximately 10*10 cm. The biopsy taken from the lesion was reported as CD30+ primary cutaneous anaplastic large cell lymphoma. A PET-CT scan revealed moderately hypermetabolic lymph nodes with thick cortices in the right iliac and inguinal lymphatic chains. Radiotherapy was applied to the area of the primary lesion and the regional lymph nodes, and improvement in the skin lesions was observed with treatment.

We aimed to discuss the outcomes of applying localized radiotherapy or systemic treatment to two patients with PC-ALCL who presented to our clinic: one with a relapse and the other with a new diagnosis. Brentuximab vedotin is effective in this disease. Since disease control could not be fully achieved with localized radiotherapy alone, we believe that the combination of systemic therapy and radiotherapy may be an important treatment option for these patients. Further large-scale case series are needed to guide treatment.