Abstract
Background: Cutaneous T-cell lymphomas are a group of lymphoproliferative disorders that are characterized by dermal infiltration by mature, malignant T cells (Blood PMID: 30635287). Mycosis Fungoides (MF) is the most common form with presenting features of skin plaques, patches or tumors, typically following an indolent clinical course (J Am Acad Dermatol PMID: 16310068). Sézary Syndrome (SS) is the leukemic variant characterized by the presence of neoplastic, clonal T-cells with cerebriform nuclei (Sezary cells) involving the skin, lymph nodes, and peripheral blood (J Am Acad Dermatol PMID: 21145619). Current treatment for MF/SS includes skin-directed therapies for early stages, and systemic therapies for advanced stages of the disease. However, treatment rarely induces long-term remission or cure. Overall, MF/SS are rare disorders, which can lead to treatment and clinical outcome variability depending on the treatment center. Our study evaluates the outcomes of MF/SS patients treated at academic cancer programs (ACP) compared to those treated a community cancer programs (CCP).
Materials and Methods: We retrospectively reviewed cases of MF/SS diagnosed in the United States using the National Cancer Data Base between the years 2004 and 2022. Demographics and clinical and survival data were compared between patients treated at ACP and CCP. Kaplan-Meier and Cox proportional hazards models were utilized to compare overall survival (OS), adjusting for age, race/ethnicity, insurance type, distance from treating facility, and comorbidity score (Charlson-Deyo).
Results: A total of 14,650 MF/SS patients were retrospectively analyzed between the years 2004 and 2022, with 11,016 (75%) of patients receiving treatment at ACP and 1,745 (12%) at a CCP. Most patients were male (57% in ACP and 62% in CCP) and predominantly white (74% at ACP and 82% at CCP). ACP had higher proportions of both black (19% ACP vs 12% CCP, p < 0.001) and Hispanic patients (6% ACP vs 5% CCP, p < 0.001). Patients at ACP were younger at diagnosis (63 years) compared to CCP (67 years) (p < 0.001). A higher number of patients were uninsured at ACP (2.4%) compared to CCP (1.9%). Comparison of the great circle distance (CROWFLY), a variable measuring the distance between a patient's residence and hospital, revealed patients at ACP lived further from the hospital center (18 miles compared to 9 miles, p < 0.001). At ACP, patients were more likely to receive treatment (71% vs 66%, p < 0.001). Treatment was initiated quicker at ACP (31 days) than CCP (36 days) (p < 0.005) with associated faster systemic therapy initiation (38 days vs 45 days) (p = 0.002). More patients at CCP received radiation (23% vs 13%, p < 0.001). More patients were diagnosed with stage IV at CCP (9.1%) than ACP (7.8%) (p < 0.001). The 30- and 90- day mortality was similar across groups (30 day: ACP 88.5% vs CCP 87.7%) (90 day: ACP 88.5% vs CCP 87.7%). The adjusted median survival for MF/SS patients at ACP was higher (14 years vs 11 years, p < 0.001). Across all timepoints (2, 5, and 10 years), the KM-estimated OS was higher at ACP than CCP, with a 2-year survival of 88.7% vs 82.2%, a 5-year survival of 75% vs. 68%, and 10 year survival of 61% and 32%, respectively.
Conclusion: This data analysis of 14,650 patients with Mycosis Fungoides/Sézary Syndrome reveal significant differences in baseline characteristics and outcomes depending on treatment at an academic cancer program (ACP) or community cancer program (CCP). Patients at ACP were more racially diverse, more likely to be uninsured, and geographically lived further from treatment centers. Treatment initiation was more frequent and faster for systemic therapy at ACPs, while CCPs had higher rates of chemotherapy, stage IV diagnosis, and radiation therapy. Despite similar short-term mortality, ACP patients had longer adjusted median survival and significantly higher survival rates at 2, 5, and 10 years – with the10-year survival rate nearly double to that of CCP. These findings suggest early referral to ACP could lead to improved clinical outcomes in rare cancers such as MF/SS.
