Otley, Clark C.

BACKGROUND Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma occurring mainly in the anogenital region. Traditional management with wide local excision has shown high recurrence rates, thus Mohs micrographic surgery (MMS) has emerged as a promising treatment option. OBJECTIVE To compare long-term outcomes after treatment with MMS or excision for primary EMPD. METHODS AND MATERIALS Retrospective cohort review was conducted for 207 patients with EMPD treated at Mayo Clinic in Rochester, MN, between 1961 and 2012. RESULTS Of the 25 patients treated with MMS, 19 primary tumors were included for outcome analysis, with an estimated 5-year recurrence-free survival rate of 91% (95% confidence interval [CI], 75–100) using Kaplan–Meier curve analysis. Of 158 patients treated with local excision, 124 were included for the analysis, with an estimated 5-year recurrence-free survival rate of 66% (95% CI, 56–78). The hazard ratio (HR) for association of treatment was 0.4 (95% CI, 0.10–1.65; p = .20). Estimated 5-year overall survival rates were 79% for MMS (95% CI, 61–100) and 68% for excision (95% CI, 59–78) (HR, 1.39 [95% CI, 0.69–2.82]; p = .36). CONCLUSION Although treatment of primary EMPD with MMS versus excision did not show statistical difference, MMS demonstrated favorable long-term outcomes and was associated with a higher recurrence-free survival rate.

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. OBJECTIVE To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). MATERIALS AND METHODS Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. RESULTS Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4.8 and 5.7 years, respectively. Twenty-eight patients (30.8%) with WLE had recurrence (mean, 4.4 years), whereas only 2 (3.0%) with MMS had recurrence (1.0 and 2.6 years). Recurrence-free survival rates at 1, 5, 10, and 15 years were significantly higher with MMS (p < .001). Mean preoperative lesion sizes were similar (5–6 cm) between the 2 groups, whereas mean (standard deviation) postoperative defect sizes were 10.7 (4.3) cm and 8.8 (5.5) cm for WLE and MMS, respectively (p = .004). Primary closure was used for 73% of MMS cases, whereas WLE more commonly used flaps, grafts, or other closures (52%). Two Mohs layers typically were required for margin control. CONCLUSION Surgical excision with meticulous histologic evaluation of all surgical margins is needed for DFSP treatment to achieve long-term high cure rates and low morbidity.

BACKGROUND Hidradenitis suppurativa (HS) is a progressive, recurrent inflammatory disorder. OBJECTIVE To assess long-term satisfaction and postoperative perceptions among patients who underwent surgical management of HS. MATERIALS AND METHODS A questionnaire was mailed to 499 HS surgical patients to assess surgical outcome, satisfaction, and quality of life. RESULTS Of the 499 questionnaires mailed, 113 were returned (22.6% response rate) and 2 were excluded for redundancy. Of the 111 respondents, 65 (58.6%) were female, 91 (82.0%) had Hurley Stage III disease, 88 (79.3%) were treated with excision and 23 (20.7%) with unroofing, 45 (40.5%) had perianal or perineal disease, and 41 (36.9%) had axillary disease. Most patients were satisfied or very satisfied with their surgical results (84.7%; 94 of 111), were glad they underwent surgery (96.3%; 105 of 109), and would recommend surgery to a friend or relative (82.6%; 90 of 109). Most patients were satisfied or very satisfied with the appearance of their healed wound (62.4%; 68 of 109). Retrospective mean quality of life increased significantly from 5 preoperatively to 8.4 postoperatively (p < .001). CONCLUSION Hidradenitis suppurativa surgical management was well regarded by patients and should be considered by future patients to limit the morbidity of HS.

BACKGROUND Hidradenitis suppurativa is a progressive, recurrent inflammatory disease. Surgical management is potentially curative with limited efficacy data. OBJECTIVE To evaluate hidradenitis surgical patients. METHODS Retrospective review of outcomes of 590 consecutive surgically treated patients. RESULTS Most patients were white (91.0% [435/478]), men (337 [57.1%]), smokers (57.7% [297/515]) with Hurley Stage III disease (476 [80.7%]). Procedure types were excision (405 [68.6%]), unroofing (168 [28.5%]), and drainage (17 [2.9%]) treating disease of perianal/perineum (294 [49.8%]), axilla (124 [21.0%]), gluteal cleft (76 [12.9%]), inframammary (12 [2.0%]), and multiple surgical sites (84 [14.2%]). Postoperative complications occurred in 15 patients (2.5%) and one-fourth (144 [24.4%]) suffered postoperative recurrence, which necessitated reoperation in one-tenth (69 [11.7%]) of patients. Recurrence risk was increased by younger age (hazard ratio [HR], 0.8; 95% confidence interval [CI], 0.7–0.9), multiple surgical sites (HR, 1.6; 95% CI, 1.1–2.5), and drainage-type procedures (HR, 3.5; 95% CI, 1.2–10.7). Operative location, disease severity, gender, and operative extent did not influence recurrence rate. CONCLUSION Excision and unroofing procedures were effective treatments with infrequent complications and low recurrence rates. Well-planned surgical treatment aiming to remove or unroof the area of intractable hidradenitis suppurativa was highly effective in the management of this challenging disease.

AbstractBackgroundHailey–Hailey disease (HHD) is an autosomal dominant genodermatosis that leads to skin breakdown and blister formation, usually in intertriginous areas. Laser ablation is a known surgical treatment for HHD.ObjectivesWe report outcomes in a series of patients with HHD treated with carbon dioxide (CO2) laser ablation.MethodsA retrospective chart review of data for 13 patients who underwent CO2 laser ablation for HHD was conducted. Questionnaires assessing effectiveness, pain, healing time, recurrence, new disease, complications, additional ablation, improvement in quality of life (QoL), and overall satisfaction were completed by all patients.ResultsAll patients had been recalcitrant to previous treatments prior to CO2 laser ablation. Anatomic sites treated and anesthesia techniques for the procedure varied. The mean ± standard deviation (SD) score for the effectiveness of CO2 laser ablation for HHD was 9.3 ± 0.9 (maximum: 10). All patients reported improved QoL. No patient had recurring disease within the treatment field. Five patients underwent additional CO2 laser treatments at new sites. One patient reported postoperative infection. No other complications were identified. The mean ± SD score for overall satisfaction with CO2 laser ablation was 8.9 ± 1.1.ConclusionsTreatment with CO2 laser ablation is very effective and can lead to prolonged or permanent remission in most HHD patients. Patients are highly satisfied with the results and report a substantial improvement in QoL.

Purpose Less than 20% of patients with melanoma who undergo sentinel lymph node (SLN) biopsy based on American Society of Clinical Oncology/Society of Surgical Oncology recommendations are SLN positive. We present a multi-institutional study to discover new molecular risk factors associated with SLN positivity in thin and intermediate-thickness melanoma. Patients and Methods Gene clusters with functional roles in melanoma metastasis were discovered by next-generation sequencing and validated by quantitative polymerase chain reaction using a discovery set of 73 benign nevi, 76 primary cutaneous melanoma, and 11 in-transit melanoma metastases. We then used polymerase chain reaction to quantify gene expression in a model development cohort of 360 consecutive thin and intermediate-thickness melanomas and a validation cohort of 146 melanomas. Outcome of interest was SLN biopsy metastasis within 90 days of melanoma diagnosis. Logic and logistic regression analyses were used to develop a model for the likelihood of SLN metastasis from molecular, clinical, and histologic variables. Results ITGB3, LAMB1, PLAT, and TP53 expression were associated with SLN metastasis. The predictive ability of a model that included these molecular variables in combination with clinicopathologic variables (patient age, Breslow depth, and tumor ulceration) was significantly greater than a model that only considered clinicopathologic variables and also performed well in the validation cohort (area under the curve, 0.93; 95% CI, 0.87 to 0.97; false-positive and false-negative rates of 22% and 0%, respectively, using a 10% cutoff for predicted SLN metastasis risk). Conclusion The addition of cell adhesion–linked gene expression variables to clinicopathologic variables improves the identification of patients with SLN metastases within 90 days of melanoma diagnosis.