Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis.

Extramammary Paget disease (EMPD) is a rare skin cancer of apocrine-rich skin that mimics common inflammatory and infectious dermatoses, leading to delays in diagnosis and increased patient morbidity. Better clinical recognition of this entity, multidisciplinary patient assessment, and deeper understanding of the underlying pathophysiology are essential to improve patient care and disease outcomes. It is important to distinguish primary intraepithelial/micro-invasive EMPD from invasive EMPD or cases with adenocarcinoma arising within EMPD. This 2-part continuing medical education series provides a complete picture of EMPD. Part 1 of this continuing medical education series reviews the epidemiology, oncogenesis, clinical and histopathologic presentation, workup, and prognosis of this rare cancer.

Extramammary Paget disease. Part II. Evidence-based approach to management.

Extramammary Paget disease is a rare cutaneous malignancy that most commonly affects the genitals, perianal area, and axilla of elderly patients. Delays in care often lead to high levels of disease burden for patients. Thus, evidence-based recommendations are paramount in mitigating morbidity and mortality for this unique patient population. This 2-part continuing medical education series provides a complete picture of extramammary Paget disease. Part 2 of this continuing medical education series focuses on the complex management of extramammary Paget disease including surgical and noninvasive therapies, as well as novel approaches for advanced disease.

Anatomic Location Influences Duration of Local Lidocaine Anesthesia in Dermatologic Surgery.

BACKGROUND: Although the onset and duration of local anesthetics are well-defined, how the anatomic site influences the duration of local anesthetics has not been well characterized in dermatology. OBJECTIVE: To define the duration of local anesthesia by anatomic site. MATERIALS AND METHODS: This was a prospective study. Adult healthy volunteers and patients undergoing Mohs micrographic surgery were invited to participate. The nose and the shin were chosen to represent highly and poorly vascularized anatomic sites, respectively. A total of 0.5 mL of buffered 1% lidocaine hydrochloride with 1:100,000 epinephrine was injected subcutaneously into each anatomic site of each participant. A pinprick test was used to assess adequate anesthesia until return of baseline sensation or visit completion. RESULTS: This study enrolled 25 participants. Time to return of sensation was significantly shorter on the nose compared with the shin ( p < .0001). On the nose, there was an association between male sex and shorter time to return of sensation. CONCLUSION: Time to return of sensation is significantly shorter on the nasal ala compared with the shin, suggesting that patients may regain sensation sooner on highly vascularized sites. Defining the duration of local anesthetics based on anatomic regions is important for treatment planning in dermatologic procedures.

Staging System Performance and Clinical Outcomes for Cutaneous Squamous Cell Carcinoma of the Ear: A Single-Center Retrospective Study.

BACKGROUND: Cutaneous squamous cell carcinoma (cSCC) of the ear is associated with poor outcomes. No studies have evaluated current staging system performance in this specific location. OBJECTIVE: Describe clinicopathologic characteristics and outcomes of ear cSCC and evaluate the performance of current staging systems. METHODS: Retrospective study including cases diagnosed and treated at a cancer center from January 2000 to December 2014. Demographic, clinical, and pathologic data were collected from clinical records. Biopsy slides were rereviewed and patients were staged according to the American Joint Committee on Cancer (AJCC) seventh, eighth, and Brigham Women's Hospital (BWH) staging. RESULTS: Of 125 patients, the mean age at diagnosis was 71.9 years (SD 12.5), with most men (89.6%, n = 112). Median follow-up was 22.3 months. Local recurrence and survival risk factors were similar to cSCC outside the ear. The Akaike's Information Criterion (AIC) estimates showed that the BWH system better predicted outcomes than the AJCC seventh, and the AJCC eighth, with AIC values of 189.9, 270.5, and 274.1, respectively. Limitations of the study include retrospective design, single center study, and no control group. CONCLUSION: Current staging systems perform well at stratifying risk in ear cSCC.

Ergonomic and psychosocial risk factors associated with work-related musculoskeletal disorders in Mohs histotechs.

Mohs histotechs are at high risk for work-related musculoskeletal disorders (WRMSDs) due to repetitive motions, forceful exertions, and fixed postures required for their work. Psychosocial factors such as perceptions of pressure to achieve deadlines may exacerbate risk of occupational injury. To assess the prevalence of and risk factors for WRMSD in Mohs histotechs, an online survey was created using literature review, expert opinion, and two validated instruments (the Nordic Musculoskeletal Questionnaire and the Oldenburg Burnout Inventory) and distributed to Mohs histotechs. 88% of respondents reported musculoskeletal problems in 2 or more of the 9 body sites assessed in the past year. Standing during specimen processing was correlated with having neck pain and upper back pain, while sitting was protective against lower back pain. Individuals that felt that they could not take breaks or slow their pace of work were more likely to report pain at multiple body sites. Total pain scores were significantly lower in those reporting extensive ergonomic training. There is a high prevalence of WRMSD in Mohs histotechs. Addressing poor posture as well as psychosocial risk factors could further reduce injury.

Blue Skin, Nail, and Scleral Pigmentation Associated with Minocycline.

Minocycline, a semisynthetic derivative of tetracycline that is used to treat various infectious and noninfectious conditions, can cause tissue hyperpigmentation. The skin, oral mucosa, sclera, and rarely the nails, can all be affected. The discoloration varies from blue, slate-gray, or brown, and it typically occurs in a dose-dependent fashion. The mechanism of hyperpigmentation, however, remains largely unknown. Herein, we present a case of gray-blue hyperpigmentation of the skin, sclera, and nails after long-term treatment with minocycline for acne.

Meningioma after radiotherapy for malignancy.

Complications of radiation exposure have gained importance with increasing cancer survivorship. Secondary malignancies have been associated with cranial radiation exposure. We present our experience with intracranial radiation-induced meningioma (RIM) and discuss the implications of its presentation and natural history for patient management. Patients diagnosed with meningioma who had received radiation therapy between 1960 and 2014 were identified. Records were retrospectively reviewed for details of radiation exposure, previous malignancies, meningioma subtypes, multiplicity and pathologic descriptions, treatment and follow-up. Thirty patients were diagnosed with RIM. Initial malignancies included acute lymphocytic leukemia (33.3%), medulloblastoma (26.7%) and glioma (16.7%) at a mean age of 8.1years (range 0.04-33years). The mean radiation dose was 34Gy (range 16-60Gy) and latency time to meningioma was 26years (range 8-51years). Twenty-one patients (70%) underwent surgery. Of these, 57.1% of tumors were World Health Organization (WHO) grade I while 42.9% were WHO II (atypical). The mean MIB-1 labeling index for patients with WHO I tumors was 5.44%, with 33.3% exhibiting at least 5% staining. Mean follow-up after meningioma diagnosis was 5.8years. Mortality was zero during the follow-up period. Meningioma is an important long-term complication of therapeutic radiation. While more aggressive pathology occurs more frequently in RIM than in sporadic meningioma, it remains unclear whether this translates into an effect on survival. Further study should be aimed at delineating the risks and benefits of routine surveillance for the development of secondary neoplasms after radiation therapy.

Outcomes of microdissection testicular sperm extraction in men with nonobstructive azoospermia due to maturation arrest.

OBJECTIVE: To evaluate sperm retrieval in men with nonobstructive azoospermia and maturation arrest (MA) undergoing microdissection testicular sperm extraction (micro-TESE). DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENT(S): Men with nonobstructive azoospermia and MA who underwent micro-TESE. INTERVENTION(S): Microdissection TESE. MAIN OUTCOME MEASURE(S): Sperm retrieval rate (SRR). RESULT(S): A total of 211 patients (13%) had a histologic finding of MA at the most advanced level. The overall SRR was 52%. A total of 146 patients were classified as having early MA (arrest at the primary spermatocyte stage), and 65 as having late MA (early spermatid stage). The SRR in men with early, vs. late, MA was 40% vs. 78%. Of the 211 men with MA, 51 had diffuse MA (100% of tubules showed MA). The SRR was significantly lower in men with diffuse vs. focal MA (35% vs. 57%). On multivariable analysis, late MA and higher follicle-stimulating hormone levels were positively associated with successful sperm retrieval. CONCLUSION(S): Sperm were successfully identified in up to one half of the men with MA after micro-TESE. Among men with MA, late MA seems to be the best predictor of successful sperm retrieval with micro-TESE.