Abstract
Introduction Primary cutaneous CD30+ T-cell lymphoma (CD30+ PCTCL) is a very rare entity, accounting for about 30% of all cutaneous T-cell lymphomas and consists of primary cutaneous anaplastic large cell lymphoma (PCALCL) and lymphomatoid papulosis (LyP). Large-scale studies describing the natural history of this entity are lacking.
Materials and Methods The National Cancer Database (NCDB) was queried for patients diagnosed with CD30+ PCTCL between 2004 and 2018. All patients aged 18 years or older diagnosed with CD30+ PCTCL were included, and we excluded patients who lost to follow-up (unknown vital status of last contact). Kaplan-Meier and multivariate Cox regression were used in the analyses.
Results The total cohort consisted of 2017 patients with CD30+ PCTCL. The specific subtype, PCALCL vs LyP, was not available. 1168 (58%) were males, 1684 (84%) Caucasian, and 232 (12%) African American, 1317 (65%) had confirmed early-stage disease (stage I and II) and 226 (11%) had confirmed advanced stage disease (stage III and IV). The median age at diagnosis was 64 years (range 18-90) and the median overall survival (OS) for the whole cohort was 166 months (95% CI 150.5-181). Compared to patients with early-stage disease, patients with advanced stage were more likely to be African American (16.8% vs 9.8% P=0.005), have B-symptoms (21.7% vs 4.3%, P<0.001), have high IPI score (2.2% vs. 0.3%, P <0.001) and to be female (50.4% vs 39.9%, P=0.003). Patients with early-stage disease had better median OS compared to patients with advanced stage disease (178 vs. 35 months, P<0.001). There was no significant difference between patients with early and advance stage disease in terms of age, facility type where they received treatment, insurance status, or Charlson-Deyo score.
In patients with early-stage disease, there was no statistically significant difference in the median OS between patients who received radiation only (N=144) compared to patients who had surgical resection alone (N=255) (not reached in both groups, P=0.676) with 5-year OS of 83% in both groups.
In patients with advanced stage disease, there was no statistically significant difference in median OS between patients who received chemotherapy (N=131) and patients who did not receive chemotherapy (N=90) (30 vs. 43 months, P=786). Also, among patients who received chemotherapy, there was no statistically significant difference in the median OS between patients who received single agent chemotherapy and patients who received multiagent chemotherapy (35 vs 30 months, P=0.934).
Conclusion This large real-world analysis indicates that CD30+ PCTCL predominantly affects elderly patients and is associated with excellent long-term survival. Intensive therapies such as multi-agent chemotherapy are not associated with survival benefit. Surgical excision and radiation treatments are equally efficacious in early-stage disease. We suggest that overtreatment with intensive therapies should be avoided, and conservative therapeutic approach is warranted in most patients. Further research is warranted to determine well-defined indications for intensive therapies.
Disclosures
Tun:Gossamer Bio, Inc.: Consultancy, Research Funding.
Author notes
Asterisk with author names denotes non-ASH members.
