Incidence Trends of Primary Cutaneous T-Cell Lymphoma in the US From 2000 to 2018

Abstract

Recent incidence patterns of cutaneous T-cell lymphoma (CTCL) in the US are not well described. We sought to describe recent incidence trends by tumor subtype, sex, age, race and ethnicity, socioeconomic status (SES), and geography.Methods | Incidence data were derived from 18 populationbased registries of the Surveillance, Epidemiology, and End Results (SEER) Program for 2000 to 2018. We included histologically confirmed cases of first primary CTCL with malignant behavior and primary site of involvement skin (C44.0-C44.9). Patient characteristics included sex, age, race and ethnicity, and geographic region (metropolitan vs nonmetropolitan counties). 1 Area-level SES information was available from 2000 to 2016 and was categorized into quintile; Q1 lowest, Q5 highest. 2 We used the 2000 US standard population to calculate age-adjusted annual incidence rates (IR) per million people. Annual percent change (APC) was calculated by using the weighted least squares method. Statistical calculations used SEER*Stat statistical software (version 8.3.9.2) in January 2022. The Stanford institutional review board deemed this study exempt from review and waived the requirement for patient informed consent because only deidentified data were used.Results | We identified 14 942 new cases of CTCL from 2000 to 2018. Table 1 shows the number of cases, IR, and APC of IR of CTCL by International Statistical Classification of Diseases and Related Health Problems for Oncology (ICD-O) diagnosis. Mycosis fungoides (MF) was the most common diagnosis, followed by primary CTCL (PCTCL) and primary cutaneous ana-

Sezary Syndrome: A Case Report and Review of Current Therapeutics

“…Cutaneous T-cell lymphomas (CTCLs) are a rare group of lymphomas that primarily involve the skin. Mycosis fungoides (MF) is the most common type of CTCL accounting for approximately 57% of CTCLs [ 1 ]. MF presents with localized or widespread skin lesions (patch, plaque, or tumor) and in some cases, lymph nodes, blood, and viscera may also be involved [ 2 ].…”

“…Sezary syndrome (SS) is a rare type of CTCL characterized by erythroderma, generalized lymphadenopathy, and clonally related malignant T-cells in the skin and peripheral blood [ 2 ]. The incidence of SS/MF increases with age with the majority of cases over 40 years of age [ 1 , 2 ]. In the United States, the incidence is higher in men, non-Hispanic black patients, individuals in high socioeconomic status quintiles, and people living in metropolitan counties [ 1 ].…”

Prognostic Factors and Survival Outcomes Among Patients With Mycosis Fungoides in China

“…Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), accounting for nearly 50% of all primary cutaneous lymphomas . An increase in MF incidence has been observed in recent years . As an indolent disease, most patients with MF are diagnosed at an early stage, characterized by a slow progression over years, and a favorable prognosis.…”

“…1 An increase in MF incidence has been observed in recent years. [2][3][4] As an indolent disease, most patients with MF are diagnosed at an early stage, characterized by a slow progression over years, and a favorable prognosis. However, 9% to 34% of patients progress to advanced stages that are associated increased mortality.…”

Cutaneous T cell lymphoma atlas reveals malignant Th2 cells supported by a B cell-rich microenvironment

“…Cutaneous T-cell lymphoma (CTCL) is a rare disease 1 and a subgroup of non-Hodgkin lymphomas, with mycosis fungoides (MF) being the most common type with an incidence of 5.42 per million in the United States 2 . Early-stage MF (stages I-IIA 3 ) typically presents in the skin with patches and plaques, which can be mistaken for benign inflammatory conditions such as atopic dermatitis (AD) and psoriasis, posing a challenge for clinical and histological diagnosis 4,5 .…”