Cancer in Costello syndrome: a systematic review and meta-analysis.

BACKGROUND: Costello syndrome (CS) is a cancer-predisposition disorder caused by germline pathogenic variants in HRAS. We conducted a systematic review using case reports and case series to characterise cancer risk in CS. METHODS: We conducted a systematic review to identify CS cases to create a retrospective cohort. We tested genotype-phenotype correlations and calculated cumulative incidence and hazard rates (HR) for cancer and cancer-free death, standardised incidence rates (SIR) and survival after cancer. RESULTS: This study includes 234 publications reporting 621 patients from 35 countries. Over nine percent had cancer, including rhabdomyosarcoma, bladder, and neuroblastoma. The rate of cancer and death associated with p.Gly12Ser were lower when compared to all other variants (P < 0.05). Higher mortality for p.Gly12Cys, p.Gly12Asp, p.Gly12Val and p.Gly60Val and higher malignancy rate for p.Gly12Ala were confirmed (P < 0.05). Cumulative incidence by age 20 was 13% (cancer) and 11% (cancer-free death). HR (death) was 3-4% until age 3. Statistically significant SIRs were found for rhabdomyosarcoma (SIR = 1240), bladder (SIR = 1971), and neuroblastoma (SIR = 60). Survival after cancer appeared reduced. CONCLUSIONS: This is the largest investigation of cancer in CS to date. The high incidence and SIR values found to highlight the need for rigorous surveillance and evidence-based guidelines for this high-risk population.

Gender Disparities in Reimbursement Among Dermatologists and Dermatologic Surgeons.

BACKGROUND: Previous literature supports a disparity in income between male and female physicians across multiple specialties, even when controlling for variables such as working hours, maternity leave, and productivity. OBJECTIVE: To understand if income disparity exists between male and female general dermatologists (GDs), and in dermatologists who completed a dermatologic surgical fellowship. MATERIALS AND METHODS: The authors surveyed members of the American Academy of Dermatology, including 66 dermatologic surgeons (DSs) (34 male and 32 female DSs) and 252 GDs (119 male and 133 female GDs), on questions related to total annual income, demographics, current employment, and time spent providing patient care. A logarithmic ordinal regression model was used to analyze income and the effect of different variables. RESULTS: Male GDs were 2.46 times more likely than female GDs to be in a higher income category (95% confidence interval [CI]: 1.44-4.23). There was no significant difference between the incomes of male and female DSs (male-to-female odds ratio: 1.46, CI: -0.44 to 1.23). These findings did not change when variables of age, median patient visits, and hours worked were controlled for. CONCLUSION: Income gender inequality exists among GDs. However, this inequality does not seem to extend to DSs.

A case of dermatofibrosarcoma protuberans and reflectance confocal microscopy of a post-surgical skin graft.

Dermatofibrosarcoma protuberans (DFSP) is an overall rare malignancy yet is one of the most common cutaneous sarcomas. The diagnosis of DFSP is typically made following histopathologic examination of the lesion, classically revealing a storiform pattern of spindle cells with elongated nuclei infiltrating the dermis and subcutis. Surgical excision is the standard treatment. Local recurrence is estimated to occur in 20-50% of cases, thus frequent postsurgical monitoring is required. Noninvasive imaging modalities offer a potential alternative to multiple repeat biopsies. We report the first case where reflectance confocal microscopy accompanied clinical examination in monitoring for DFSP recurrence postsurgical excision.

Reflectance confocal microscopy evaluation of pigmented lesions on tattooed skin.

The evaluation of pigmented lesions on tattooed skin poses a diagnostic challenge for dermatologists, as a nevus may be partially or completely obscured by tattoo pigment. Because of incidences of melanoma arising from tattooed skin, the current gold standard is to biopsy these lesions. Reflectance confocal microscopy (RCM) is a noninvasive imaging modality used in the diagnosis and management of skin diseases that may allow for diagnosis, while preserving the tattoo design. Retrospective chart review was conducted to identify pigmented lesions on or near tattooed skin that were evaluated with RCM. Confocal characteristics and diagnoses were recorded and analyzed. Nineteen lesions from 15 patients were retrospectively reviewed. Tattoo pigment did not hinder evaluation and diagnosis of pigmented lesions on RCM. About 94.7% of lesions were diagnosed as benign melanocytic nevi by an expert confocal reader. One lesion was confocally diagnosed as melanocytic nevus with atypia but was found to be an inflamed melanocytic nevus on histology. Tattoo pigment particles were differentiated from other hyper-refractile entities by an expert confocal reader based on size, morphology, and clinical correlation. RCM may provide a solution to the diagnostic challenge of pigmented lesions on or near tattooed skin.

The growth of teledermatology: Expanding to reach the underserved.

The regulation of telemedicine in the United States is evolving, with new legislation expanding reimbursement and cross-state licensing capabilities. As telemedicine grows, communities with limited access to traditional dermatologic care may find a solution in teledermatology. A search of the medical literature and online health care law resources published within the past decade was performed to assess the current status of telemedicine availability, health record integration and security, reimbursement policy, and licensure requirements in the United States, with a focus on teledermatology. The majority of states have implemented policies requiring private insurance coverage. Medicaid reimburses some form of telemedicine in all states but restricts which modalities can be used and by which specialties. Medicare places the heaviest limitations on telemedicine coverage. Twenty-four states and Guam are members of the Interstate Medical Licensure Compact (IMLC), and 27 states offer alternative cross-state practice options. With the advent of publicly and privately funded programs, volunteer efforts, and mobile applications, teledermatology is more readily available to rural and underserved communities.

Life of a Wound: Serial Documentation of Wound Healing After Shave Removal Using Reflectance Confocal Microscopy

Reflectance Confocal Microscopy (RCM) is a new noninvasive skin imaging modality that is comparable to traditional histopathology. We present serial in vivo RCM imaging of an atypical nevus after shave excision over a 1-month period. Findings on RCM images are consistent with the inflammatory, proliferative, and remodeling phases of wound healing, and RCM may serve as a new tool to study wound healing in vivo over time. J Drugs Dermatol. 2019;18(5):472-474.

Feasibility, safety and clinical outcomes of cardiophrenic lymph node resection in advanced ovarian cancer.

OBJECTIVES: Surgical resection of enlarged cardiophrenic lymph nodes (CPLNs) in primary treatment of advanced ovarian cancer has not been widely studied. We report on a cohort of patients undergoing CPLN resection during primary cytoreductive surgery (CRS), examining its feasibility, safety, and potential impact on clinical outcomes. METHODS: We identified all patients undergoing primary CRS/CPLN resection for Stages IIIB-IV high-grade epithelial ovarian cancer at our institution from 1/2001-12/2013. Clinical and pathological data were collected. Statistical tests were performed. RESULTS: 54 patients underwent CPLN resection. All had enlarged CPLNs on preoperative imaging. Median diameter of an enlarged CPLN: 1.3cm (range 0.6-2.9). Median patient age: 59y (range 41-74). 48 (88.9%) underwent transdiaphragmatic resection; 6 (11.1%) underwent video-assisted thoracic surgery. A median of 3 nodes (range 1-23) were resected. A median of 2 nodes (range 0-22) were positive for metastasis. 51/54 (94.4%) had positive nodes. 51 (94.4%) had chest tube placement; median time to removal: 4d (range 2-12). 44 (81.4%) had peritoneal carcinomatosis. 19 (35%) experienced major postoperative complications; 4 of these (7%) were surgery-related. Median time to adjuvant chemotherapy: 40d (range 19-205). All patients were optimally cytoreduced, 30 (55.6%) without visible residual disease. Median progression-free survival: 17.2mos (95% CI 12.6-21.8); median overall survival: 70.1mos (95% CI 51.2-89.0). CONCLUSIONS: Enlarged CPLNs can be identified on preoperative imaging and may indicate metastases. Resection can identify extra-abdominal disease, confirm Stage IV disease, obtain optimal cytoreduction. In the proper setting it is feasible, safe, and does not delay chemotherapy. In select patients, it may improve survival.

The Tam formula: A pilot study for a new treatment for melasma.

BACKGROUND: Melasma is a common hyperpigmentation disorder with numerous, but often unsatisfactory treatment options. AIMS: A pilot study to evaluate the efficacy and safety of a novel topical combination of 12% hydroquinone, 6% kojic acid, and 5% vitamin C cream for melasma. PATIENTS AND METHODS: A pilot study of 6 women with melasma was conducted at an academic dermatology department and a private dermatology practice to evaluate the efficacy of a topical combination of 12% hydroquinone, 6% kojic acid, and 5% vitamin C cream, entitled the "Tam Formula." Two blinded evaluators calculated Melasma Area and Severity Index (MASI) Scores before and after treatment to evaluate change from baseline, and statistical analysis was performed. RESULTS: Treatment with this combination topical cream resulted in an average 63.77 ± 22.10 percent reduction in MASI scores. CONCLUSIONS: While there is a need for further investigation, this pilot study indicates the Tam Formula may provide an alternative treatment option for melasma.

Gender Disparities in Income Among Board-Certified Dermatologists.

Although there is evidence that gender-based disparities exist in salary, academic rank, and other factors in several areas in medicine, limited data exist on differences between male and female dermatologists. Existing studies have focused on academic dermatologists, not including the vast majority of dermatologists who work in solo and group private practices. A cross-sectional self-reported survey eliciting total annual income and other factors was performed in the fall of 2018 in the United States. A total of 397 board-certified dermatologists (MDs/DOs) participated in this study, including 53.63% female and 46.37% male respondents. A statistically significant difference existed within total annual income between male and female dermatologists (P<.0001). Several factors were identified that demonstrated statistically significant differences between male and female dermatologists, including productivity, practice area of focus, type of fellowship training, and faculty rank. However, despite controlling for these variations, gender remained a statistically significant predictor of income on both univariate and multivariate regression analyses (P=.0002/P<.0001), indicating that a gender-based income disparity exists in the field of dermatology that cannot be explained by other factors.

Back to the future: integrating technology to improve patient-provider interactions.

The adoption of electronic medical records (EMRs) resulted in both improvement and deterioration of different aspects of patient-provider interactions. We envision further integration of current and future technology to optimize patient-provider interactions, which we present using a hypothetical patient encounter.

Noninvasive imaging tools in dermatology.

The diagnosis of skin disease relies on visual inspection, often followed by biopsy and histopathologic examination, which remains the gold standard in diagnosis. New imaging tools, including dermoscopy, reflectance confocal microscopy (RCM), and optical coherence tomography (OCT), can provide noninvasive diagnoses while sparing unnecessary biopsies. We discuss dermoscopy, RCM, and OCT, and compare cost, clinical integration, reimbursement, and accuracy of these imaging modalities.

"Deep" vertical Mohs and closure with a tumor-free flap for dermatofibrosarcoma protuberans: A case report.

Dermatofibrosarcoma protuberans is an uncommon low-grade malignant tumor that can invade locally and rarely metastasize. Dermatofibrosarcoma protuberans has a high rate of local recurrence due to incomplete excision, especially in deep tissues. Morbidity is often related to multiple local recurrences and removal of excessive tissue with large, complex repairs. We present a case of incompletely excised dermatofibrosarcoma protuberans after initial wide local excision. We subsequently employed a "deep" vertical Mohs micrographic surgical technique to remove the remaining tumor while creating a flap with the tumor-free superficial portion to preserve tissue and avoid a complicated repair. The patient is tumor-free for 7 years.