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• W2891662025 abstract "Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma (CTCL). Studies on various types of CTCL have shown the prognostic significance of primary disease site (Su et al, 2017; Nguyen et al, 2018), while few studies have investigated the prognostic potential of primary disease site for MF. The primary aim of our study is to compare the survival outcomes of patients with MF among different primary disease sites, while controlling for confounders. The National Cancer Database (NCDB) was used to ascertain de-identified patients with MF using the International Classification of Disease – Oncology, third edition (ICD-O3) morphological code 9700 diagnosed between 2004 and 2014. Information extracted from the user participant file included demographics, clinical information, the first course of treatment received that contribute to the long-term control of the cancer, length of follow-up and overall survival (OS). Patients with no known primary disease site, follow-up, treatment or income data were excluded. A Kaplan–Meier estimate was used to evaluate OS, and differences in survival were examined by the log-rank test. Univariate and multivariate Cox proportional hazards regression was used to evaluate independent predictors associated with survival. Pairwise comparisons of different primary sites in OS were performed using propensity score matching to account for confounders (MatchIt package [https://gking.harvard.edu/matchit], R version 2.15.1 [https://www.r-project.org/]). Statistical significance was defined as P ≤ 0·05. Analysis was performed using IBM SPSS Statistics 22 (IBM Corporation, North Castle, NY, USA), and the figure was generated with GraphPad Prism 6 (GraphPad Software, La Jolla, CA, USA). Survival of the MF patient cohort relative to the expected survival in the age- and gender-matched general US population was analysed with the ‘strs' command in STATA/SE 13 (Stata Corporation, College Station, TX, USA) as detailed previously by Dickman et al (2015). US Census Bureau and the Centers for Disease Control and Prevention databases (www.census.gov; www.cdc.gov) were used to obtain information on the yearly age- and sex-matched segments of the US population. A total of 2428 patients with MF identified using the NCDB met the inclusion criteria. Trunk, upper limbs, lower limbs, head and neck and overlapping lesion of skin constituted 36·0% (n = 873), 12·2% (n = 297), 22·0% (n = 535), 10·1% (n = 244) and 19·7% (n = 479) of the entire cohort, with the mean age at diagnosis being 58·2, 57·3, 57·1, 60·5 and 58·5 years, respectively. The 5-year OS rate of the entire MF cohort was 84·8%. Significant differences in survival outcomes were present among different primary sites, with 5-year survival rates of 85·5%, 82·5%, 88·2%, 79·5%, and 78·3% for patients with disease localized to trunk, upper limbs, lower limbs, head and neck and overlapping lesion of skin (Fig 1). The 5- and 10-year survival relative to the expected survival of an age- and gender-matched US standard population was 90·9% (95% confidence interval [CI] 88·5–93·0%) and 87·8% (95% CI 82·4–92·9%), respectively. Stratified by primary disease sites, 5-year relative survivals to an age- and gender- matched US standard population were 93·0% (95% CI 89·2–96·3%), 87·0% (95% CI 79·6–93·0%), 97·0% (95% CI 92·0–101·0%), 88·5% (95% CI 80·0–95·4%) and 84·1% (95% CI 78·4–89·1%) for patients with disease localized to trunk, upper limbs, lower limbs, head and neck and overlapping lesion of skin. On multivariate Cox regression analysis, older age (P < 0·001), male gender (P < 0·001), African American race (P = 0·044), Charlson-Deyo score of 1 (P = 0·018) or >2 (P = 0·019), higher clinical stage (P < 0·001), the receipt of chemotherapy (P < 0·001) and immunotherapy (P = 0·048) were associated with shorter OS (Table 1). Academic facility type (P = 0·006) and private insurance status (P < 0·001) were associated with longer OS. Patients with disease localized to the trunk (Hazard ratio [HR] 1·378, 95% CI: 1·045–1·817, P = 0·023), upper limbs (HR 1·727, 95% CI: 1·234–1·417, P = 0·001) or overlapping lesion of skin (HR 1·545, 95% CI: 1·515–2·230074, P = 0·004) had shorter OS than those with disease localized to the lower limbs. After pair-wise propensity score matching based on age at diagnosis, gender, facility type, race, insurance status, Charlson-Deyo score, clinical stage and treatments received, patients with lesions localized to the lower limbs had significantly longer OS than those with lesions localized to the overlapping lesion of skin (P = 0·028). Furthermore, patients with lesion localized to the lower limbs demonstrated a trend of longer OS than patients with lesion localized to the upper limb (P = 0·175), head and neck (P = 0·124) and trunk (P = 0·122). Our results demonstrated that patients with disease located in the lower limbs had the longer OS than patients with disease located in head and neck, and upper limbs, trunk, while patients with disease located in the overlapping lesion of the skin had the worst survival, after adjusting for disease characteristics, socioeconomic factors and treatments received. In addition, age- and gender-matched relative survival data demonstrated that a component of mortality can be attributed to MF in the affected US population, and that the survival difference among different primary disease sites persisted relative to the expected survival of the general US population. The poorer OS for patients with lesions located in the overlapping lesion of the skin was possibly attributed to the diffuse nature of the disease, which has been shown to be an unfavourable prognostic factor of MF, resulting in difficult categorization of the primary site (Wilson et al, 2012; Nath et al, 2014; Desai et al, 2015). A recent study by Su et al (2017) of 4057 patients from the NCDB showed that primary cutaneous peripheral T-cell lymphoma patients with lesions localized to the lower extremity had significantly shorter OS when compared to those with disease localized to the head and neck, trunk, and upper extremity after adjusting for confounding factors. A prior study by Grange et al (1999) on non-MF/Sézary syndrome/lymphomatoid papulosis CTCL suggested that anatomical site was not a significant prognostic factor. The contradictory results between our study and other studies on CTCL are probably because the disease process of subtypes of CTCL, patient cohorts and factors included in the analysis are inherently different. Further studies are needed to investigate the underlying pathophysiology of different survival outcome by location in MF. None. The authors have no conflict of interest to declare. CS and HXB: designed research, performed research, interpreted data, wrote the paper, contributed to critical revisions/edits. RT: performed research, analysed data, wrote the paper. MG, GK, PJZ, RX and GZ: designed research, interpreted data, contributed to critical revisions/edits." @default.
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• W2891662025 date "2018-09-14" @default.
• W2891662025 modified "2023-10-17" @default.
• W2891662025 title "Disease site as a prognostic factor for mycosis fungoides: an analysis of 2428 cases from the <scp>US</scp> National Cancer Database" @default.
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• W2891662025 doi "https://doi.org/10.1111/bjh.15570" @default.
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