Introduction: PC4SMPTCL is a rare subtype of cutaneous T-cell lymphoma with generally indolent behavior but variable clinical outcomes. While most cases exhibit a favorable prognosis, some patients experience disease progression, recurrence, or systemic involvement. Objectives: This study aims to identify key prognostic factors that impact survival in patients with PC4SMPTCL. Methods: We compiled a pooled database of PC4SMPTCL cases, constructed Kaplan-Meier survival curves, and used the Cox proportional hazards model and Log-rank tests to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 219 patients were included in the analysis. The median age was 53, with no sexual preponderance. Seventy-nine percent were solitary lesions. The most common sites were the head and neck (60%). While 85% were alive at 29 years, the median EFS was 27 years. Advanced stage, any recurrence, and response less-than CR were detrimental to OS and EFS. Multiple and multifocal lesions, and lower extremity lesions had worse EFS. Surgery, radiation, and their combination had superior EFS to steroids. OS and EFS were not impacted by sex, age, number of recurrences, Ki67%, CD30+, TCR clonality, or spontaneous regression. Conclusions: PC4SMPTCL generally has a favorable prognosis, with prolonged survival in most cases. This pooled analysis identifies the clinical factors and treatment modalities that are major determinants of survival in this rare disease. These findings highlight key prognostic factors that can guide clinical management and risk stratification for PC4SMPTCL.
