T-cell Lymphoma Epidemiology: the Known and Unknown.

T-cell lymphoma, a collection of subtypes of Non-Hodgkin lymphoma, is a rare malignancy. The low prevalence of this disease has made it challenging to identify subtype-specific risk factors. Potential risk factors could enable us to identify high-risk patients and predict patient outcomes. Here, we report on the current epidemiologic and prognostic factors data associated with the individual subtypes both of peripheral T-cell lymphoma (PTCL) and cutaneous T-cell lymphoma (CTCL) found in large cohort and case studies. Additionally, with recent findings, as well as updates in the new World Health Organization (WHO) classification of lymphoid neoplasms, we consider what this could do to change our approach to this group of diseases.

Total skin electron therapy for cutaneous T-cell lymphoma using a modern dual-field rotational technique.

PURPOSE: To report our experience with rotational total skin electron irradiation (RTSEI) in cutaneous T-cell lymphoma (CTCL), and to examine response by disease stage and race. METHODS AND MATERIALS: We reviewed our outcomes for 68 CTCL patients who received RTSEI (≥ 30 Gy) from 2000 to 2013. Primary outcomes were complete clinical response (CCR), recurrence-free survival (RFS), and overall survival (OS). Using log-rank tests and Cox proportional hazards, OS and RFS were compared across tumor stages at time of RTSEI with further racial subgroup analysis. RESULTS: Median age at diagnosis and at time of radiation was 52 and 56 years, respectively. Median follow-up was 5.1 years, 49% were African American, and 49% were female. At time of treatment, 18, 37, and 13 patients were T stage 2, 3, and 4, respectively. At 6 weeks after RTSEI, overall CCR was 82% (88%, 83%, and 69% for T2, T3, and T4, respectively). Median RFS was 11 months for all patients and 14, 10, and 12 months for stage T2, T3, and T4, respectively. Tumor stage was not associated with RFS or CCR. Maintenance therapy after RTSEI was associated with improved RFS in both crude and multivariable analysis, controlling for T stage. Median OS was 76 months (91 and 59 months for T3 and T4, respectively). With the exception of improved OS in African Americans compared with whites at stage T2, race was not associated with CCR, RFS, or OS. CONCLUSIONS: These results represent the largest RTSEI clinical outcomes study in the modern era using a dual-field rotational technique. Our observed response rates match or improve upon the standard set by previous outcome studies using conventional TSEI techniques, despite a large percentage of advanced CTCL lesions in our cohort. We found that clinical response after RTSEI did not seem to be affected by T stage or race.

Cutaneous nonmycotic T- and natural killer/T-cell lymphomas: diagnostic challenges and dilemmas.

Mycosis fungoides is the prototype of primary cutaneous T-cell lymphoma and is more common in the West than in the East, whereas nonmycotic primary cutaneous T-cell lymphoma is more frequent than mycosis fungoides among Asians. Nonmycotic primary cutaneous T-cell lymphomas comprise several categories of neoplasms and might pose diagnostic challenges because of the rarity of these lesions and overlapping features among certain entities. The authors recommend diagnostic approaches including histopathological evaluation, immunohistochemical markers, and ancillary studies. Diagnostic dilemma in certain entities and cases with atypical clinicopathological features are discussed.

Age, race, sex, stage, and incidence of cutaneous lymphoma.

UNLABELLED: The T- and B-cell cutaneous lymphomas (CLs) are relatively rare, and information regarding clinical presentation and differences among racial groups might be helpful in determining the best course of clinical care. Data from nearly 5000 patients with CL from the SEER (Surveillance, Epidemiology and End Results Program) registry were evaluated. Nonwhite racial groups present with mycosis fungoides (MF) at an earlier age compared with white, and African American (AA) have increased risk of presenting with higher T-stage compared with white patients. These findings have significant implications regarding need for earlier diagnosis and understanding the reasons for racial disparity in age and stage of presentation. BACKGROUND: The incidence of the T- and B-cell CLs has been well documented, but information pertaining to racial incidence by age, and by burden of disease (stage) have not been extensively documented. MATERIALS AND METHODS: The SEER 2004-2008 public use database was investigated. The relative incidence of CL in different races and age groups was examined. Univariate and multivariate stepwise logistic regression was performed for the likelihood of presenting at a higher stage. RESULTS: Of 4496 patients diagnosed with CL between 2004 and 2008; 1713 patients were diagnosed with MF, 1518 with non-MF cutaneous T-cell lymphoma, and 1265 patients with cutaneous B-cell lymphoma. For MF, there was a trend for females to be less likely to present with a higher T-stage (T3-T4) than males (odds ratio [OR], 0.73) on multivariate analysis (P = .06). For race, AA had a significantly increased risk of presenting with higher T-stage (T3-T4) MF (OR, 1.72) on multivariate analysis (P = .02), compared with white patients. For white, AA, Asian/Pacific Islander, and Native American/other/unknown, the mean age at diagnosis was 59.2, 51.5, 51.3, and 53.8. These groups presented at a significantly different age than white (P = .0001, 0.0001, and 0.0006). CONCLUSION: Nonwhite racial groups present with MF at an earlier age compared with white, and AA have increased risk of presenting with higher T-stage compared with white. These findings have significant implications regarding need for earlier diagnosis and understanding the reasons for racial disparity in age and stage of presentation.

Skin cancer in skin of color.

In general, skin cancer is uncommon in people of color when compared to Caucasians. When it does occur, it is often associated with increased morbidity and mortality. Differences in survival rates may be attributed to skin cancers being diagnosed at a more advanced stage, and socioeconomic factors such as lack of adequate insurance coverage and lack of transportation can function as barriers to timely diagnosis and early treatment. In addition to advanced stage at presentation, malignant skin lesions in skin of color often present in an atypical fashion. Because skin cancer prevention and screening practices historically have been lower among Hispanics, Blacks, and Asians, and given the changing demographics in the United States, interventions that are tailored to each of these groups will be needed. Public educational campaigns should be expanded to educate people of all skin types with emphasis on skin cancers occurring in areas not exposed to the sun (Byrd-Miles et al., 2007), since sunlight is not as important an etiologic factor in the pathogenesis of skin cancer in people of color. Dermatologists and primary care physicians should instruct their darker-skinned patients on how to perform routine skin self-examinations. Physicians should also encourage patients to ask their specialists such as their gynecologist, dentist, and ophthalmologist to look for abnormal pigmentation during routine exams. To reduce the burden of skin cancer, several prevention methods for all people have been strongly encouraged, including monthly self-examinations, daily use of SPF 30 or greater sunscreen, sunglasses with UV-absorbing lenses, and avoiding tanning booths (American Cancer Society, 2008) (see Table 7). In addition, recommendations for clinicians to promote the prevention of skin cancer in skin of color have also been made, including closely monitoring changing pigmented lesions on the palms and soles and hyperkeratotic or poorly healing ulcers in immunosuppressed patients (Halder & Bridgeman-Shah, 1995) (see Table 7).

Analysis of primary CD30+ cutaneous lymphoproliferative disease and survival from the Surveillance, Epidemiology, and End Results database.

PURPOSE: Primary CD30+ cutaneous lymphoproliferative disease (PCLPD) is a spectrum of indolent cutaneous T-cell lymphomas. The primary intention of the analysis of the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database was to report epidemiologic information and overall survival of patients with PCLPD. METHODS: We investigated the SEER database from 1973 to 2004 and performed univariable and multivariable survival analysis. RESULTS: A total of 268 cases of PCLPD were recorded from 1973 to 2004. Median age at diagnosis was 61 years (range, 5 to 98 years). Among cases, 58% were male, and 42% female. Race distribution was 87% white, 7% black, and 4% Asian/Pacific Islander. A total of 157 patients had primary, localized PCLPD. For the total population (N = 268), overall survival at 3 years was 81% (95% CI, 74% to 87%). Population-matched relative survival at 3 years was 87% (SE, 3.6%). Disease-specific survival at 5 years was 92% (95% CI, 86% to 95%). Head and neck skin site predicted for inferior overall survival in patients with primary, localized PCLPD on univariable analysis (hazard ratio [HR] = 4.4; P = .008; 95% CI, 1.5 to 13.2), and was suggestive of decreased overall survival on multivariate analysis (HR = 3.0; P = .06; 95% CI, 0.95 to 9.7). CONCLUSION: Localized PCLPDs are rare diseases with an excellent overall survival and occur more frequently in whites and in men. Head and neck skin primary site may be associated with poorer survival. CONCLUSIONS regarding subsets demonstrating association with survival should be taken with caution, given the small number of deaths analyzed.

Incidence of cutaneous T-cell lymphoma in the United States, 1973-2002.

OBJECTIVE: To describe incidence trends for cutaneous T-cell lymphoma (CTCL) in the United States. DESIGN: Population-based study. SETTING: Data were obtained from 13 population-based cancer registries of the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute from 1973 through 2002. PARTICIPANTS: A total of 4783 cases of CTCL were identified for the period 1973 through 2002. MAIN OUTCOME MEASURE: Diagnosis of CTCL. RESULTS: The overall annual age-adjusted incidence of CTCL was 6.4 per million persons. Annual incidence increased by 2.9 x 10(-6) per decade over the study period. Incidence was higher among blacks (9.0 x 10(-6)) than among whites (6.1 x 10(-6)) and was higher among men (8.7 x 10(-6)) than among women (4.6 x 10(-6)). The racial differences in incidence decreased with age, while the sex differences increased with age and decreased over time. Substantial geographic variation in incidence was found. Incidence was correlated with high physician density, high family income, high percentage of population with a bachelor's degree or higher, and high home values. Changes in International Classification of Diseases for Oncology (ICD-O) morphologic definitions have resulted in the redistribution of the cases of CTCL among specific subclassifications. CONCLUSIONS: The continued rise in incidence of CTCL is substantial, and the cause of this increase is unknown. The racial, ethnic, sex, and geographic differences in incidence may be of etiologic importance. Changes in ICD-O definitions have made it difficult to evaluate incidence trends for subclassifications of CTCL such as mycosis fungoides. In addition, these changes resulted in the creation of ambiguous histologic codes, which may have caused coding errors. These errors along with the lack of independent verification are limitations of our study. An epidemiological investigation using population-based data is important to better understand this disorder.

Skin cancer in African Americans.

Skin cancer is the most common type of malignancy in the United States. Incidence within the African American population remains relatively low, but data is limited for this racial group, making accurate determination of incidence and mortality difficult. Factors implicated as causative in the pathogenesis of cutaneous malignancy in African Americans include, but are by no means limited to, sunlight, albinism, burn scars, X-rays, preexisting pigmented lesions, chronic inflammation, and chronic discoid lupus erythematosus. Anatomic distribution of lesions may be similar to that seen in whites for basal cell carcinoma but not for other skin cancers. For squamous cell carcinoma, melanoma, and cutaneous T-cell lymphoma, African Americans do not as well in terms of mortality as do whites. This difference probably is due either to the fact that African Americans have more advanced stages of disease at diagnosis than do whites or, in some cases, because the course of the disease is more aggressive in African Americans for reasons yet unknown. There is a need for heightened awareness of skin cancer in African Americans by patients and physicians. Emphasis should be on education and early diagnosis with the primary goal in mind being the reduction of incidence of and mortality due to skin cancer in African Americans. In addition, because of environmental factors, African Americans will be exposed to more solar ultraviolet radiation in the future. Strategies should be developed for public education to keep this exposure to low levels in this racial group.