Introduction
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSMLPD) typically manifests as an indolent head/neck papule, frequently creating cosmetic concerns for patients. Optimal management is uncertain. Herein, we audit patient outcomes following 3 more commonly used management strategies: radiotherapy (RT), surgery and watchful-waiting. We also investigate the efficacy of ultra-low dose RT, which has not been previously studied in patients with PCSMLPD.
Methods
Eligibility required diagnosis of PCSMLPD, between 2007-2022, confirmed on central pathological review. RT responses were assessed: complete response (CR), less than CR, progressive disease (PD). Surgical excisions were classified: complete excision (CE) or incomplete excision (IE) using microscopic margins. Freedom from relapse (FFR) was compared between modalities.
Results
Of 41 eligible patients, 19 received RT first-line, 17 underwent excision (3 received adjuvant RT), and 5 chose watchful-waiting. Median follow-up was 37 (range, 6-167) months.
Overall, 24 patients received RT: 19 definitive first-line, 3 adjuvant first-line, 2 second-line. RT dose was 4Gy in 2 fractions in 10 (42%) patients, 20-40Gy in 14 (58%). Following RT, 100% achieved CR at 6 months, with no relapses. No toxicities were reported following 4Gy.
Patients receiving surgery first-line (n=14, 3 with positive margins) had CE and IE rates of 65% and 35%, respectively. 4 (23.5%) patients experienced relapse (2 local, 2 distant) at median 12 (range, 6-36) months post-excision.
4 of 5 patients managed with watchful-waiting experienced partial resolution post-biopsy; no complete resolution observed.
3-year FFR was 100% for RT-alone vs 61% for surgery (p=0.12).
Conclusion
RT is a successful, non-invasive option for PCSMLPD with 100% CR rate, no relapses, and numerically superior FFR compared to surgery, in this cohort. Low-dose RT for PCSMLPD (4Gy in 2 fractions) offers durable control, no acute toxicities and convenient 2-day treatment time. For patients without cosmetic concerns, watchful-waiting is a reasonable management strategy.