Introduction: Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL) is a rare subtype of primary cutaneous T-cell lymphoma. Due to its rarity and clinical overlap with other CD30-positive lymphoproliferative disorders, the prognostic impact of various clinical and pathological factors is not fully understood. Objectives: This exploratory analysis aims to identify demographic patterns and determinants of survival in this rare malignancy. Methods: We compiled a pooled database of cases that satisfy the diagnostic criteria for pcALCL. Kaplan-Meier survival curves were constructed. Cox proportional hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 189 patients with confirmed pcALCL were identified. The median age was 55, with slight male preponderance. Seventy-two percent were ALK-. The median OS and Time to First Recurrence (TTFR) were 156 and 36 months, respectively. HIV+, immunosuppression, ALK-, multiple lesions, ulceration, response-less than CR, and recurrence were detrimental to OS. Time to first recurrence correlated positively with OS. OS was not impacted by sex, age, time to diagnosis, EBV status, stage, or small variant histology. Lymph node involvement and multiple lesions had worse TTFR. Compared to no treatment, OS was increasingly better with Radiation, Chemotherapy, surgery, and combined modalities (p=0.002). Conclusions: This pooled analysis identifies the clinical, immunohistochemical, and treatment modalities that are major determinants of OS in this rare disease. These findings provide valuable insights into the prognostic determinants and optimal management strategies for pcALCL.
