Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease.

OBJECTIVES: To identify the rates of associated and occult cancers in patients with extramammary Paget disease (EMPD) discovered using cancer screening methods at a tertiary medical center; to propose evidence-based cancer screening guidelines at the time of diagnosis of EMPD; and to clarify terminology associating EMPD with underlying malignancies. PATIENTS AND METHODS: A retrospective review of patients with histologically confirmed EMPD presenting for care at our institution between January 1, 1992, and December 31, 2015, was performed. Both male and female patients were included. Descriptive analysis was performed. RESULTS: A total of 161 patients met the inclusion criteria. Most (59.6%) were female patients, and the mean age at the time of EMPD diagnosis was 70.8±10.1 years. Most (82%) of the 161 patients had at least 1 cancer screening test performed, though screening practices varied widely. Of those screened for an underlying malignancy, 17 distant, noncontiguous malignancies were identified in 15 patients (11.4%), with prostate (n=5), urinary tract (n=5), and breast (n=2) malignancies found most frequently. Most malignancies were identified by urine cytology, mammography, and prostate-specific antigen blood test. Of all patients, 37 (23.0%) had an underlying contiguous malignancy identified by pathology. CONCLUSION: All patients diagnosed with EMPD should undergo cancer screening. At minimum, evaluation should include age-appropriate screening and the addition of urine cytology, mammography, and prostate-specific antigen blood test-if not already performed-may be of particular use. An algorithm for evaluation of patients with newly diagnosed EMPD is proposed.

Factors Influencing Squamous Cell Carcinoma In Situ Recurrence and Implications for Treatment Choice.

BACKGROUND: Numerous treatment modalities have been reported for squamous cell carcinoma in situ (SCCIS). Risk factors for recurrence have not been systematically reviewed. OBJECTIVE: To systematically review and summarize the data on risk factors that contribute to recurrence of SCCIS. MATERIALS AND METHODS: A PubMed search was completed using the terms "SCCIS," "Bowen's disease," "Bowen's disease and recurrence," and "Bowen's disease and Mohs." These sources were cross-referenced for the terms "treatment," "management," "therapy," "recurrence," and "margins." Studies were selected on the basis of relevance and applicable treatments. RESULTS: Immunosuppression was the only variable with a statistically signficant association with progression or recurrence of SCCIS. Although there were no data directly correlating subclinical lateral extension or invasive squamous cell carcinoma within SCCIS with recurrence, evidence supports both of these as common features of SCCIS. Other potential recurrence risk factors for which there are limited supporting data included tumor size, depth of follicular extension, and location. CONCLUSION: Immunosuppression was the only risk factor associated with increased risk of tumor recurrence. Subclinical tumor extension and occult invasive squamous cell carcinoma are relatively common features that theoretically could increase recurrence risk. These factors should be considered when deciding upon treatment for SCCIS. Further study is required to quantify variables that influence recurrence and to identify optimal treatment options.

A matter of margins: Surgical and pathologic risk factors for recurrence in extramammary Paget's disease.

OBJECTIVES: To determine surgical and pathologic variables associated with recurrence in extramammary Paget's disease (EMPD). METHODS: Medical records of patients seeking care for EMPD from 1/1992-9/2015 were reviewed. Follow-up was restricted to 5years following primary surgery. Recurrence-free survival (RFS) was estimated using the Kaplan-Meier method. Risk factors were evaluated for an association with recurrence and positive margins, respectively, using Cox proportional hazards regression and logistic regression. RESULTS: Of 154 patients, 90 (58.4%) were female and 65 (41.6%) were male. Treatment consisted of wide local excision (WLE, includes WLE or radical vulvectomy, 77.3%), Mohs micrographic surgery (MMS, 19.5%), and abdominoperineal resection (3.2%). RFS at 1, 3, and 5years was 84.5% (95% confidence interval (CI), 78.2-91.4%), 66.1% (95% CI, 57.5-75.9%), and 56.1% (95% CI, 46.9-67.1%), respectively. Positive surgical margins were univariately associated with higher risk of recurrence (HR 3.55, 95% CI 1.74, 7.24). Margin status significantly correlated with procedure type (33.3% vs. 3.4% had positive margins with WLE vs. MMS, p=0.01). Among patients with negative margins, there was a 2.5 fold increased risk of recurrence after WLE compared to MMS (95% CI, 0.57-10.9, p=n.s.). CONCLUSION: Inclusion of males allowed us to examine the influence of a different surgical approach (MMS) on margin status and recurrence rates in EMPD. In contrast to prior studies including solely vulvar EMPD, we observed strong association between margin status and recurrence risk. Risk of positive margins was significantly higher after WLE compared to MMS. MMS should be explored to improve outcomes in gynecologic patients with EMPD.

Incidence and Trends of Basal Cell Carcinoma and Cutaneous Squamous Cell Carcinoma: A Population-Based Study in Olmsted County, Minnesota, 2000 to 2010.

OBJECTIVE: To determine population-based incidence estimates of basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (cSCC). PATIENTS AND METHODS: We reviewed the medical records of a population-based cohort diagnosed with nonmelanoma skin cancer between January 2, 2000, and December 31, 2010. The age- and sex-adjusted incidence rates were calculated and compared with estimates from previous periods. RESULTS: The age-adjusted BCC incidence (cases per 100,000 person-years) was 360.0 (95% CI, 342.5-377.4) in men and 292.9 (95% CI, 278.6-307.1) in women. The age-adjusted cSCC incidence (cases per 100,000 person-years) was 207.5 (95% CI, 193.9-221.1) in men and 128.8 (95% CI, 119.4-138.2) in women. From years 1976 to 1984 to years 2000 to 2010, the age- and sex-adjusted incidence (cases per 100,000 person-years) of BCC increased from 222.0 (95% CI, 204.5-239.5) to 321.2 (95% CI, 310.3-332.2) and that of cSCC from 61.8 (95% CI, 52.3-71.4) to 162.5 (95% CI, 154.6-170.3). Over time, the anatomical distribution of BCC shifted from the head and neck to the torso and that of cSCC shifted from the head and neck to the extremities. CONCLUSION: The incidences of BCC and cSCC are increasing, with a disproportionate increase in cSCC relative to BCC. There is also a disproportionate increase in the incidence of both tumors in women, as well as a shift of anatomical distributions.

Incidence and Clinical Features of Rare Cutaneous Malignancies in Olmsted County, Minnesota, 2000 to 2010.

BACKGROUND: The incidence of rare cutaneous malignancies is unknown. Current estimates of rare cutaneous malignancy incidences are based on broad epidemiologic data or single institution experiences, not population-based data. OBJECTIVE: To determine the incidence of several rare nonmelanoma skin cancers. MATERIALS AND METHODS: The authors conducted a retrospective chart review of a population-based cohort between the years 2000 and 2010. Residents of Olmsted County, Minnesota, who were diagnosed with a biopsy-proven nonmelanoma skin cancer-excluding basal cell carcinoma and squamous cell carcinoma-were included in this study. The primary outcome was tumor incidence. Additionally, the authors extracted patient demographics, tumor characteristics, treatment modalities, and outcomes. RESULTS: The age-adjusted and sex-adjusted incidences per 100,000 persons of multiple rare cutaneous malignancies were: atypical fibroxanthoma (1.8), sebaceous carcinoma (0.8), dermatofibrosarcoma protuberans (0.4), microcystic adnexal carcinoma (0.7), eccrine carcinoma (0.4), eccrine porocarcinoma (0.2), and leiomyosarcoma (0.2). CONCLUSION: The authors report population-based incidences and clinical characteristics for these rare cutaneous malignancies. The immune status and smoking status of patients and the treatment and outcomes of these tumors are reported. Additional studies in a broader population are needed to further define the epidemiology and outcomes of these malignancies.

Staging for cutaneous squamous cell carcinoma as a predictor of sentinel lymph node biopsy results: meta-analysis of American Joint Committee on Cancer criteria and a proposed alternative system.

IMPORTANCE: The appropriate clinical setting for the application of sentinel lymph node biopsy (SLNB) in the management of cutaneous squamous cell carcinoma (cSCC) is not well characterized. Numerous case reports and case series examine SLNB findings in patients who were considered to have high-risk cSCC, but no randomized clinical trials have been performed. OBJECTIVE: To analyze which stages in the American Joint Committee on Cancer (AJCC) criteria and a recently proposed alternative staging system are most closely associated with positive SLNB findings in nonanogenital cSCC. DESIGN, SETTING, AND PARTICIPANTS: Medical literature review and case data extraction from private and institutional practices to identify patients with nonanogenital cSCC who underwent SLNB. Patients were eligible if sufficient tumor characteristics were available to classify tumors according to AJCC staging criteria and a proposed alternative staging system. One hundred thirty patients had sufficient data for AJCC staging, whereas 117 had sufficient data for the alternative system. EXPOSURE: Nonanogenital cSCC and SLNB. MAIN OUTCOMES AND MEASURES: Positive SLNB findings by cSCC stage, quantified as the number and percentage of positive nodes. RESULTS: A positive SLN was identified in 12.3% of all patients. All cSCCs with positive SLNs were greater than 2 cm in diameter. The AJCC criteria identifed positive SLNB findings in 0 of 9 T1 lesions (0%), 13 of 116 T2 lesions (11.2%), and 3 of 5 T4 lesions (60.0%). No T3 lesions were identified. The alternative staging system identified positive SNLB findings in 0 of 9 T1 lesions (0%), 6 of 85 T2a lesions (7.1%), 5 of 17 T2b lesions (29.4%), and 3 of 6 T3 lesions (50.0%). Rates of positive SLNB findings in patients with T2b lesions were statistically higher than those with T2a lesions (P = .02, Fisher exact test) in the alternative staging system. CONCLUSIONS AND RELEVANCE: Our findings suggest that most cSCCs associated with positive SLNB findings occur in T2 lesions (in both staging systems) that are greater than 2 cm in diameter. The alternative staging system appears to more precisely delineate high-risk lesions in the T2b category that may warrant consideration of SLNB. Future prospective studies are necessary to validate the relationship between tumor stage and positive SLNB findings and to identify the optimal staging system.

Solar keratoses: photodynamic therapy, cryotherapy, 5-fluorouracil, imiquimod, diclofenac, or what? Facts and controversies.

Actinic keratosis is a common dermatologic condition that may regress, remain stable, or progress to squamous cell carcinoma. Some question whether all actinic keratoses should be routinely treated, whereas others contend that the unpredictable natural history of this disease necessitates treatment to prevent malignant transformation. Available treatments include photodynamic therapy, cryotherapy, 5-fluorouracil, imiquimod, and diclofenac. Each of these options has its advantages and disadvantages, although they all have a place in the management of actinic keratosis. An overview of these treatment modalities is presented, as are the controversies surrounding the treatment of actinic keratosis.

Management of unusual cutaneous malignancies: atypical fibroxanthoma, malignant fibrous histiocytoma, sebaceous carcinoma, extramammary Paget disease.

Atypical fibroxanthoma, malignant fibrous histiocytoma, sebaceous carcinoma, and extramammary Paget disease are rare cutaneous tumors. Their recognition and diagnosis are critical in decreasing long-term morbidity and mortality. Surgical excision is the treatment of choice for these tumors, and Mohs micrographic surgery has been shown to be as favorable or better than wide local excision in providing long-term clearance rates.