Pediatric Melanoma Arising in a Background of Melanocytic Nevi.

Malignant melanoma is an aggressive neoplasm primarily involving the skin. They may arise de novo or from a premalignant melanocytic lesion. Melanomas are primarily known to occur in adults. Pediatric melanomas (PM) are rare and predominantly occur de novo following ultraviolet deoxyribonucleic acid (DNA) damage. They may also be associated with the presence of congenital melanocytic nevi. We report a rare case of a 6-year-old child with multiple melanocytic nevi subsequently diagnosed with melanoma of the back and metastatic disease.

Primary cutaneous SMARCA4-deficient undifferentiated malignant neoplasm: A rare case report and literature review.

Primary cutaneous SMARCA4-deficient undifferentiated malignant neoplasm (SD-UMN) is a rare and recently described entity characterized by the loss of expression of the SMARCA4 (BRG1) protein, which is involved in chromatin remodeling. SD-UMN presents a diagnostic challenge due to its rarity and unique histopathological and immunohistochemical features. In this report, we present a case of primary cutaneous SD-UMN in a 67-year-old man who presented with a rapidly growing, ulcerated, and bleeding nodule on his right cheek. Histopathological examination revealed a highly cellular dermal tumor consisting of pleomorphic epithelioid cells with prominent mitotic figures and necrosis, lacking any morphological evidence of differentiation. Immunohistochemical analysis showed a complete loss of SMARCA4 and SMARCA2 expression, while INI-1 expression remained intact. p53 was diffusely expressed, and p16 was completely absent. In addition, a range of markers, including high-molecular-weight cytokeratin, p63, SOX10, INSM1, MCPyV, NKX2.2, CD99, CDX2, CD56, ERG, NUT, desmin, androgen receptor, chromogranin, CD34, and CD43 were all negative. To date, only two cases of primary cutaneous SMARCA4-deficient undifferentiated tumors have been reported in the literature. Therefore, this case report adds to the limited body of knowledge on the clinical and histopathological features of this novel entity. The report highlights the importance of considering SD-UMN in the differential diagnosis of undifferentiated cutaneous tumors.

Atypical Presentation of Spitz Nevus: A Case Report.

Introduction: The Spitz nevus (SN) is an acquired melanocytic neoplasm composed of epithelioid and/or spindle cells, which tends to develop in childhood. In pediatric patients, it is usually located on the face and neck. Unusual locations have been found in the literature, such as the penis, mouth, and tongue, as well as 2 cases of ungual SN. Case Report: A 15-year-old male evaluated for dark brown-black longitudinal melanonychia that covered 40% of the nail, with pseudo-Hutchinson's sign, of 1 year of evolution. Discussion: The SN accounts for 1% of the melanocytic neoplasms. In the present paper, we show the third case of ungual SN never previously disclosed, which presents a zigzag pattern reported in the literature for its association with the pediatric population.

Painful schwannoma of scalp: A case report.

Schwannoma, also known as neurilemmoma, is a benign neoplasm of Schwann cells of the cranial or peripheral nerve sheath. Scalp involvement has been reported in 25% of patients with extracranial head and neck schwannomas, which can be misdiagnosed clinically as epidermal cyst or lipoma. In this article, we report a 32-year-old male presenting with a slow-growing painful subcutaneous mass on the left occipital scalps without any neurological symptoms. Pathological findings confirmed the diagnosis of schwannoma, and surgical removal resulted in the resolution of pain and lack of recurrence.

Reconstructive Procedures of the Auricular Concha after Cutaneous Oncologic Surgery: A Systematic Review.

Reconstruction of the auricular concha poses a challenge due to its difficult access and limited tissue flexibility; however, there are no recommendations in the literature on which reconstructive technique should be favored for this anatomical site. This systematic review intends to describe and compare the reconstructive techniques used in conchal bowl reconstruction following cutaneous oncologic surgery of this region, with regard to their complications and aesthetic results. In doing so, we aim to identify the best suited reconstructive procedure(s) for the conchal bowl. The six databases searched (PubMed, Scopus, Web of Science, Ovid, SciELO, and CENTRAL) yielded twelve eligible studies that explored the revolving door flap, split-thickness skin grafts (STSG), full-thickness skin grafts (FTSG), second intention healing, the preauricular translocation flap, subcutaneous pedicle grafts, and other local flaps. Qualitative synthesis of the results concluded that the revolving door flap could be the reconstructive procedure of choice for the auricular concha, following skin cancer excision. It has a low risk of necrosis, infection, and postoperative hemorrhage, as well as excellent aesthetic outcomes. STSG may be used as an alternative. Nonetheless, due to the low sample size and the high risk of bias in some studies, further investigations must be conducted on this subject.

Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature.

(1) Background: Endocrine Mucin-Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade, neuroendocrine-differentiated, cutaneous adnexal tumor, officially recognized by the World Health Organization (WHO) Skin Tumors Classification in 2018 as a separate entity and homologue of endocrine ductal carcinoma in situ (eDCIS)/solid papillary carcinoma of the breast. Although it is more frequent in the female sex, between 60 and 70 years old, in the peri-orbital region, EMPSGC has also been described in the male sex, in subjects under 60 and over 80, and in extra-eyelid localizations (cheek, temple, scalp), but also in extra-facial localizations (chest and scrotum). (2) Methods: We present the clinical case of a 71-year-old woman with an undated lesion of the scalp, which presented as a nodule, skin-colored, and 2.5 cm in maximum diameter. We also conduct a comprehensive literature review from 1997 to the end of 2022, consulting PubMed, Scopus, Web of Science (WoS), and Google Scholar using the following keywords: "Endocrine mucin-producing sweat gland carcinoma" and/or "EMPSGC" and/or "skin" and "cutaneous neoplasms". In addition, we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 253 patients were recorded; 146 were females (57.7%) and 107 were males (42.2%). The vast majority of the lesions were in the eyelids (peri-ocular region), and only a minority of cases involved the cheeks, supra-auricular, retro-auricular, and occipital region, with very rare cases in the scalp, to which the present is also added. (4) Conclusions: The morphological and immunophenotypical features are essential both for the correct diagnosis and to be able to classify this lesion among the corresponding eDCIS/solid papillary carcinoma of the breast, with neuroendocrine differentiation. Recent papers have attempted to shed light on the molecular features of EMPSGC, and much remains to be conducted in the attempt to subtype the molecular profiles of these entities. Future studies with large case series, and especially with molecular biology techniques, will be needed to further add information about EMPSGC and its relationship in the PCMC spectrum.

Implementation of artificial intelligence for the detection of cutaneous melanoma within a primary care setting: prevalence and types of skin cancer in outdoor enthusiasts.

Background: There is enthusiasm for implementing artificial intelligence (AI) to assist clinicians detect skin cancer. Performance metrics of AI from dermoscopic images have been promising, with studies documenting sensitivity and specificity values equal to or superior to specialists for the detection of malignant melanomas (MM). Early detection rates would particularly benefit Australia, which has the worlds highest incidence of MM per capita. The detection of skin cancer may be delayed due to late screening or the inherent difficulty in diagnosing early skin cancers which often have a paucity of clinical features and may blend into sun damaged skin. Individuals who participate in outdoor sports and recreation experience high levels of intermittent ultraviolet radiation (UVR), which is associated with the development of skin cancer, including MM. This research aimed to assess the prevalence of skin cancer in individuals who regularly participate in activities outdoors and to report the performance parameters of a commercially available AI-powered software to assess the predictive risk of MM development. Methods: Cross-sectional study design incorporating a survey, total body skin cancer screening and AI-embedded software capable of predictive scoring of queried MM. Results: A total of 423 participants consisting of surfers (n = 108), swimmers (n = 60) and walkers/runners (n = 255) participated. Point prevalence for MM was highest for surfers (6.48%), followed by walkers/runners (4.3%) and swimmers (3.33%) respectively. When compared to the general Australian population, surfers had the highest odds ratio (OR) for MM (OR 119.8), followed by walkers/runners (OR 79.74), and swimmers (OR 61.61) rounded out the populations. Surfers and swimmers reported comparatively lower lifetime hours of sun exposure (5,594 and 5,686, respectively) but more significant amounts of activity within peak ultraviolet index compared with walkers/runners (9,554 h). A total of 48 suspicious pigmented lesions made up of histopathology-confirmed MM (n = 15) and benign lesions (n = 33) were identified. The performance of the AI from this clinical population was found to have a sensitivity of 53.33%, specificity of 54.44% and accuracy of 54.17%. Conclusions: Rates of both keratinocyte carcinomas and MM were notably higher in aquatic and land-based enthusiasts compared to the general Australian population. These findings further highlight the clinical importance of sun-safe protection measures and regular skin screening in individuals who spend significant time outdoors. The use of AI in the early identification of MM is promising. However, the lower-than-expected performance metrics of the AI software used in this study indicated reservations should be held before recommending this particular version of this AI software as a reliable adjunct for clinicians in skin imaging diagnostics in patients with potentially sun damaged skin.

Store-and-Forward Teledermatology for Assessing Skin Cancer in 2023: Literature Review.

BACKGROUND: The role of teledermatology for skin lesion assessment has been a recent development, particularly, since the COVID-19 pandemic has impacted the ability to assess patients in person. The growing number of studies relating to this area reflects the evolving interest. OBJECTIVE: This literature review aims to analyze the available research on store-and-forward teledermatology for skin lesion assessment. METHODS: MEDLINE was searched for papers from January 2010 to November 2021. Papers were searched for assessment of time management, effectiveness, and image quality. RESULTS: The reported effectiveness of store-and-forward teledermatology for skin lesion assessment produces heterogeneous results likely due to significant procedure variations. Most studies show high accuracy and diagnostic concordance of teledermatology compared to in-person dermatologist assessment and histopathology. This is improved through the use of teledermoscopy. Most literature shows that teledermatology reduces time to advice and definitive treatment compared to outpatient clinic assessment. CONCLUSIONS: Overall, teledermatology offers a comparable standard of effectiveness to in-person assessment. It can save significant time in expediting advice and management. Image quality and inclusion of dermoscopy have a considerable bearing on the overall effectiveness.

Solitary fibrofolliculoma on the nasal septum: a case report.

Fibrofolliculoma is a benign, perifollicular, connective tissue tumor that usually arises in the form of multiple lesions; it is rarely seen as a solitary lesion. The lesions are clinically asymptomatic, 2 to 4 mm skin-colored, soft dome-shaped papules. Here, we report a patient who visited our hospital with a palpable lesion on the nasal septum. The lesion did not cause pain upon palpation, and nasal endoscopy confirmed an irregular wart-like lesion measuring 6 × 6 mm in the left anterior nasal septum near the columella. Other otolaryngology findings were normal, and there were no similar lesions in other parts of the body. None of the patient's family members were known to have had such lesions. An excisional biopsy was performed on the mass for removal of the lesion, and histological examination confirmed the lesion as fibrofolliculoma. We report the first case of solitary fibrofolliculoma in the nasal septum in a healthy 62-year-old woman along with a review of the relevant literature.

Diagnostic performance of augmented intelligence with 2D and 3D total body photography and convolutional neural networks in a high-risk population for melanoma under real-world conditions: A new era of skin cancer screening?

BACKGROUND: Convolutional neural networks (CNNs) have outperformed dermatologists in classifying pigmented skin lesions under artificial conditions. We investigated, for the first time, the performance of three-dimensional (3D) and two-dimensional (2D) CNNs and dermatologists in the early detection of melanoma in a real-world setting. METHODS: In this prospective study, 1690 melanocytic lesions in 143 patients with high-risk criteria for melanoma were evaluated by dermatologists, 2D-FotoFinder-ATBM and 3D-Vectra WB360 total body photography (TBP). Excision was based on the dermatologists' dichotomous decision, an elevated CNN risk score (study-specific malignancy cut-off: FotoFinder >0.5, Vectra >5.0) and/or the second dermatologist's assessment with CNN support. The diagnostic accuracy of the 2D and 3D CNN classification was compared with that of the dermatologists and the augmented intelligence based on histopathology and dermatologists' assessment. Secondary end-points included reproducibility of risk scores and naevus counts per patient by medical staff (gold standard) compared to automated 3D and 2D TBP CNN counts. RESULTS: The sensitivity, specificity, and receiver operating characteristics area under the curve (ROC-AUC) for risk-score-assessments compared to histopathology of 3D-CNN with 95% confidence intervals (CI) were 90.0%, 64.6% and 0.92 (CI 0.85-1.00), respectively. While dermatologists and augmented intelligence achieved the same sensitivity (90%) and comparable classification ROC-AUC (0.91 [CI 0.80-1.00], 0.88 [CI 0.77-1.00]) with 3D-CNN, their specificity was superior (92.3% and 86.2%, respectively). The 2D-CNN (sensitivity: 70%, specificity: 40%, ROC-AUC: 0.68 [CI 0.46-0.90]) was outperformed by 3D CNN and dermatologists. The 3D-CNN showed a higher correlation coefficient for repeated measurements of 246 lesions (R = 0.89) than the 2D-CNN (R = 0.79). The mean naevus count per patient varied significantly (gold standard: 210 lesions; 3D-CNN: 469; 2D-CNN: 1324; p < 0.0001). CONCLUSIONS: Our study emphasises the importance of validating the classification of CNNs in real life. The novel 3D-CNN device outperformed the 2D-CNN and achieved comparable sensitivity with dermatologists. The low specificity of CNNs and the lack of automated counting of TBP nevi currently limit the use of augmented intelligence in clinical practice.

Dermatofibrosarcoma Protuberans of the Scalp Mimicking Trichilemmal Cyst: A Case Report.

We present a case of a 47-year-old female with a swelling on her scalp that was at first thought to be trichilemmal cysts. After two years, she returned to her general practitioner with a larger scalp mass. Following a biopsy, histological analysis revealed dermatofibrosarcoma protuberans (DFSP). She then had the tumor completely removed, resulting in clean margins.

Integrated Safety Analysis on Skin Cancers among Patients with Psoriasis Receiving Ixekizumab in Clinical Trials.

INTRODUCTION: Limited data exist on skin cancer risk in patients with psoriasis using biologics. Here, we report treatment-emergent adverse events (TEAEs) of skin cancer in patients treated with ixekizumab from psoriasis clinical trials. METHODS: Integrated safety databases from 17 clinical trials of adults with moderate-to-severe psoriasis treated with ≥ 1 dose of ixekizumab for ≤ 5 years were used to analyze exposure-adjusted incidence rates (IRs) per 100 patient-years of exposure (PYE) and clinically characterize dermatologist-adjudicated skin cancer TEAEs. RESULTS: Of 6892 patients, 58 presented with ≥ 1 skin cancer TEAE (IR 0.3) with IRs remaining stable with longer ixekizumab exposure. Non-melanoma skin cancer (NMSC) was the most common event (IR 0.3) affecting 55 patients; of those, 44 had basal cell carcinoma (IR 0.2) and 16 had squamous cell carcinoma (IR 0.1). Two treatment-emergent melanoma events were identified; neither were classified as serious AEs. CONCLUSIONS: Incidence of skin neoplasms in patients with psoriasis treated with ixekizumab for ≤ 5 years was low, and among those events, NMSC was most common. Limitations included that longer exposure may be required to confirm risk of skin cancer and that the study exclusion criteria of several studies, which excluded patients with skin cancer events within 5 years prior to baseline, might limit interpretation of skin cancer risk in this cohort. These findings support the safety profile of ixekizumab for patients requiring long-term psoriasis control.

Extra-mammary Paget's disease of the penis.

Paget's disease was first described in 1874 as an eczematoid changes of the nipple associated with underlying breast carcinoma. Extra-mammary Paget's disease (EMPD) is rare with involvement of the male genitalia described in small case series and management options varying according to location and extent. The diagnosis of EMPD requires a high index of clinical suspicion and close liaison with the multidisciplinary team, particularly histopathology. We present two cases of EMPD affecting the male external genitalia that highlight important learnings in the presentation, diagnosis, and management of EMPD.

Stage IA melanoma follow-up: Exploring the level of confidence of South Australian general practitioners in undertaking surveillance skin checks including considerations for shared care.

BACKGROUND: The literature highlights the role of Australian general practitioners (GP) in the management of skin cancers. With melanoma incidences on the rise, there have been discussions into whether lower-risk stage IA patients could safely be followed up by their GPs for annual surveillance full skin examinations (FSE). This study explores the level of confidence of South Australian (SA) GPs in undertaking FSEs including factors that could support discussions around shared care between GPs and dermatology units for lower-risk patients. METHODS: An online survey was designed and distributed to SA GPs via email, newsletters and social media between 5 December 2021 and 30 January 2022. Descriptive statistics were used to describe survey responses. Pearson's Chi-squared analysis was used to investigate associations between key variables of interest and explanatory variables. Logistic regression analysis was used to model odds ratios for associations between the dependent variable and independent variables. RESULTS: A total of 135 responses were obtained. Forty-four per cent of GPs were comfortable undertaking annual FSEs, 41% were uncomfortable and 15% were unsure. Scope of work, >20 years experience and additional training had statistically significant relationships (p < 0.05). Dermoscopy and detecting melanoma recurrences were reported to be skills with lower levels of confidence. With regards to shared care, 77% indicated that they would feel supported undertaking FSEs if rapid access referral pathways were allocated for patients who developed suspicious lesions. Preferred upskilling modalities included, face-to-face sessions in a dermatology unit (39%), dermatologist run webinars (25%) and certificate courses (20%). CONCLUSIONS: At present, there is a subset of SA GPs who are comfortable undertaking FSEs and therefore could be engaged in shared care with specialists. Further considerations have to be made in the areas of upskilling and supporting the workforce to enhance engagement in shared care.

Eruptive basal cell carcinoma and lenalidomide: rising awareness among dermatologists.

Because of its higher level of safety compared to its parent medicine, thalidomide, lenalidomide (L) is chosen for the treatment of multiple myeloma. We report a patient who, within a month of using L, developed more than 10 basal cell carcinomas.

Incipient Melanonychia: Benign Finding or Occult Malignancy? A Case Report of Subungual Melanoma.

Subungual melanomas are rare neoplasms that tend to debut as longitudinal melanonychia. They are primarily found in patients over 60 years of age and are usually diagnosed late, representing a diagnostic challenge. We present a case report of a 59-year-old female Hispanic patient who initially presented with melanonychia and was eventually diagnosed with subungual melanoma in situ. She was surgically treated and, after three months, remained healthy. Relevant risk factors, clinical and onychoscopic findings, diagnostic criteria, and treatment options are also discussed. Since many benign entities present similarly, high clinical suspicion is critical for diagnosing this entity.

Factors associated with the use of adjuvant radiation therapy in stage III melanoma.

Objective: The role of radiation therapy (RT) in melanoma has historically been limited to palliative care, with surgery as the primary treatment modality. However, adjuvant RT can be a powerful tool in certain cases and its application in melanoma has been increasingly explored in recent years. The aim of this study is to explore national patterns of care and associations surrounding the use of adjuvant RT for stage III melanoma. Methods: The National Cancer Data Base (NCDB) was used to identify patients who were diagnosed with stage III melanoma between 2004 and 2014. Exclusion criteria included those with distant metastatic disease, in-situ histology, no confirmed positive nodes, palliative intent therapy, and dosing regimens inconsistent with National Comprehensive Cancer Network (NCCN) guidelines for adjuvant RT in melanoma. Patients treated with and without adjuvant RT were compared and factors associated with use of adjuvant RT were identified using multivariable logistic regression analyses. Results: A total of 7,758 cases of stage III melanoma were analyzed, of which 11.7% received adjuvant RT. The mean age of the overall cohort was 58.5 years, and the majority of patients were male (64.7%), white (96.6%), on private insurance (51.3%), and presented to a non-high-volume facility (90.3%). Multivariable regression analyses revealed that patients who present to the hospital in 2009-2014 as compared to 2004-2008 (odds ratio [OR] 1.61, 95% confidence interval [CI] 1.36-1.92), had 4 or more positive nodes (OR 4.30, 95% CI 3.67-5.04), and had microscopic residual tumor (OR 2.11, 95% CI 1.46-3.04) were more likely to receive adjuvant RT. Factors that were negatively associated with receiving adjuvant RT included female gender (OR 0.72, 95% CI 0.61-0.85) and median income of at least $63,000 (OR 0.66, 95% CI 0.52-0.83). Conclusions: This study demonstrates the rising use of RT for stage III melanoma in recent years and identifies demographic, social, clinical, and hospital-specific factors associated with patients receiving adjuvant RT. Further investigation is needed to explore disease benefits to improve guidance on the utilization of RT in melanoma.

Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3 months. The duration of recurrence is between 3 and 24 months. The size of the tumor ranges from 7.2 to 10.5 cm. The wide local excision margins range from 2 to 4 cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.

The skin through reflectance confocal microscopy -Historical background, technical principles, and its correlation with histopathology

Abstract Since its first introduction into medical practice, reflectance confocal microscopy (RCM) has been a valuable non-invasive diagnostic tool for the assessment of benign and malignant neoplasms of the skin. It has also been used as an adjunct for diagnosing equivocal cutaneous neoplasms that lack characteristic clinical or dermoscopic features. The use of RCM has led to a decreased number of biopsies of benign lesions. Multiple published studies show a strong correlation between RCM and histopathology thereby creating a bridge between clinical aspects, dermoscopy, and histopathology. Dermatopathologists may potentially play an important role in the interpretation of confocal images, by their ability to correlate histopathologic findings. RCM has also been shown to be an important adjunct to delineating tumoral margins during surgery, as well as for monitoring the non-surgical treatment of skin cancers. Advanced technology with smaller probes, such as the VivaScope 3000, has allowed access to lesions in previously inaccessible anatomic locations. This review explains the technical principles of RCM and describes the most common RCM features of normal skin with their corresponding histological correlation.