Brewer, Jerry D.

AbstractBackgroundPatients with chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or non‐Hodgkin lymphoma (NHL) are at increased risk for the development of skin malignancies.ObjectivesThis study was conducted to estimate the incidences of rare skin malignancies in patients with CLL/SLL or NHL.MethodsPatients with a diagnosis of CLL/SLL or NHL recorded in the Surveillance, Epidemiology and End Results (SEER) database during 1992–2007 were identified. Diagnoses of specific skin malignancies were identified from SEER files.ResultsDuring 1992–2007, a total of 128,674 patients with first diagnoses of CLL/SLL or NHL were recorded in SEER; 4743 were excluded because follow‐up data were unavailable. Among the remaining 123,931 patients, 28,964 had CLL/SLL and 94,967 had NHL. Standardized incidence ratios (SIRs) for invasive malignant melanoma, Merkel cell carcinoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, Kaposi's sarcoma, and sebaceous carcinoma were 2.3, 8.2, 3.6, 2.5, 2.9, and 1.4, respectively, in CLL/SLL patients and 1.6, 3.2, 1.5, 1.3, 17.6, and 0.8, respectively, in NHL patients. When invasive melanoma was stratified by patient age and sex, the highest SIR (17.8) was found in men aged 0–49 years with CLL (P < 0.001).ConclusionsPatients with CLL/SLL or NHL have a higher risk for the subsequent development of rare skin cancers. Given the more aggressive nature of these malignancies in this setting, regular monitoring for the development and prompt treatment of cutaneous malignancy is prudent in patients with NHL and particularly in patients with CLL. Regular use of sun protection may decrease the morbidity associated with skin cancer in this immunosuppressed population.

Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous malignancy. The infectivity of Merkel cell polyomavirus (MCPyV), an apparent agent in MCC development, may be exacerbated with impaired immune responses. This paper reviews relevant data regarding the role of immunosuppression in the development of MCC and describes modes of immunodeficient states. Because of the inherently low incidence rate of MCC, several case studies and series are also briefly mentioned to provide a more comprehensive summary of MCC in the setting of immunosuppression. We describe immunosuppressed patients who have experienced excessive UV radiation, organ transplantation, human immunodeficiency virus infection/AIDS, autoimmune diseases, and lymphoproliferative disorders. Iatrogenic forms of immunosuppression are also highlighted. Studies that quantify risks consistently report that individuals with a history of solid organ transplantation, autoimmune diseases, AIDS, and/or lymphoproliferative diseases have a significantly elevated risk of developing MCC. Overall, immunocompromised patients also appear to have an early onset and more aggressive course of MCC, with poorer outcomes. Recommendations for multidisciplinary approaches are proposed to effectively prevent and manage MCC in these patients.

AbstractBackgroundBasosquamous carcinoma (BSC) and metatypical basal cell carcinoma (MBCC) are uncommon tumors poorly defined in the literature. Available studies suggest these tumors carry a greater risk of recurrence and metastases than basal cell carcinomas (BCCs) and, in some studies, squamous cell carcinomas. Formal treatment recommendations are not fully established.ObjectiveTo analyze BSC and MBCC separately, evaluate whether they are distinct tumor subtypes, and analyze Mohs micrographic surgery (MMS) efficacy for BSC and MBCC.MethodsRetrospective review of medical records and histologic specimens was conducted for 293 patients with 303 biopsy‐proven BSCs or MBCCs treated with MMS between 1996 and 2004. In total, 32 BSCs and 128 MBCCs were identified. Surgical and follow‐up data were analyzed.ResultsKaplan–Meier estimates of recurrence‐free survival after MMS were 100% at one year for both tumor subtypes and were 100% for BSC and 93.8% for MBCC at 5 years. Initial mean sizes were 1.5 cm for BSC and 1.3 cm for MBCC. Approximately 7% represented recurrent tumors at surgery. Of six patients with recurrences, none had known metastatic disease.LimitationsLimitations include retrospective design, analysis of only head and neck sites, and small sample sizes.ConclusionBSC and MBCC showed no significant distinguishing characteristics to separate them into two BCC subtypes. Reported recurrence rates for BSC and MBCC are 12–45% with wide local excision; estimated recurrence rates are 4.1% with MMS. Our study showed recurrence‐free survival of 95.1% at five years. Hence, MMS is effective in treating these BCC subtypes.

The authors documented a low compliance with guidelines to screen for skin malignancy in a community-dwelling cohort of patients with newly diagnosed chronic lymphocytic leukemia.

AbstractThe link between immunosuppression and skin cancer has been well described. The two most common situations involving immunosuppression‐associated skin cancer are solid organ transplantation and non‐Hodgkin lymphoma (NHL), including chronic lymphocytic leukemia (CLL). Patients with lymphoma are more likely to have development of a secondary malignancy, with skin cancer being the most common. The most common types of skin cancer in patients with NHL/CLL include melanoma, squamous cell carcinoma, basal cell carcinoma, and Merkel cell carcinoma. Many skin cancers demonstrate increased aggressiveness in patients with NHL/CLL and are associated with higher recurrence rates, increased regional metastasis, and death secondary to skin cancer metastases. This review delineates the current research regarding the relationship between NHL/CLL and cutaneous malignancy. Immunosuppressed patients with skin cancer should be treated promptly and aggressively to decrease recurrence and metastases. Regular skin self‐examinations, dermatologic examinations, sun‐protective habits, and education may prove beneficial in this high‐risk patient population.