Objective Woringer-Kolopp disease (WKD), also known as localized pagetoid reticulosis, is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas. Our objective was to conduct a comprehensive review that describes and evaluates patient demographics, clinical presentation, immunohistochemical findings, management, and outcomes of WKD. Methods The databases PubMed, Embase, and Cochrane Library were searched for relevant literature. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The literature search was conducted on December 7, 2020.Studies were included if they contained primary data related to WKD. Non-pertinent studies, non-English studies, non-human studies, review articles, or studies with insufficient case information were excluded. The quality of the included studies and the risk of bias were evaluated using the Grades of Recommendation, Assessment, Development, and Evaluation Group (GRADE) criteria ( 2016), Oxford Centre for Evidence-Based Medicine: Levels of Evidence (OCEBM) (March 2009), and the Methodological Index for Non-Randomized Studies (MINORS) criteria (2003).Results A total of 84 studies with 143 patients met the inclusion criteria for this study. The most common chief complaint was an asymptomatic, slow-growing, and erythematous plaque. The average time from initial skin eruption to presentation was 97.6 months.Histologically, 128 cases of WKD displayed epidermotropism (97.7%). Various therapies, including radiotherapy, surgery, and local chemotherapy, were used. In total, 92 (78.6%) cases had complete remission and 11 (9.4%) cases recurred. Conclusions WKD is a rare cutaneous T-cell lymphoma that often presents as a single plaque on the extremities with an indolent course and a favorable prognosis. However, it is often misdiagnosed, leading to delays in treatment. The diagnosis of WKD involves clinical appraisal, a biopsy of suspicious lesions, and immunohistochemistry. Monotherapy appears to be preferred compared to combination therapy for WKD with radiotherapy demonstrating among the highest cure rates and lowest recurrence. Future retrospective and prospective studies are needed to accurately determine the epidemiology, prognosis, and effective treatments for WKD. Limitations include a possibility of missed studies, heterogeneity in reporting methods, publication, and selection bias.What is already known about this topic?• Woringer-Kolopp disease (WKD) is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas.• Qualitative systematic analysis can generate new insights compared to individual findings.What does this study add?• Demonstrates the need for accurate diagnosis of Cutaneous T-Cell Lymphomas.
Objectives: Malignant proliferating trichilemmal tumors of the scalp can exhibit aggressive presentation and recurrences. Our objective was to perform an evidence-based systematic review evaluating clinical presentation, tumor characteristics, and treatment modalities used to determine which treatment strategies had the best outcomes. Methods:The databases PubMed, Embase, and Cochrane Library were searched for relevant literature by the authors. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The results were reported using the Preferred Reporting Systems for Systematic Reviews and Meta-Analysis guidelines.Results: Thirty-nine studies with a total of 65 patients were identified. The most common presentation was a history of slowgrowing, painless swollen mass on the scalp. In total, 10 patients (15.4%) presented with spread to the regional lymph nodes and 6 (9.2%) additional patients presented with metastasis to distant locations. In total, 61 patients (93.8%) underwent surgery. Various chemotherapy and radiation therapy regimens were used. Of the 45 cases with documented follow-up, 11 (24.4%) patients had one or multiple instances of local, lymph node or metastatic tumor recurrence.Conclusions: Surgery is favored, and the exact approach should be based on clinical judgment. However, Mohs micrographic surgery should strongly be considered because of its superior margin control against such an invasive tumor. Radiotherapy and chemotherapy have been used as adjuvant therapy in aggressive cases or recurrence. Patients should be followed closely and examined often to frequently assess recurrence or metastasis. Randomized controlled trials are needed to further clarify these findings.
Individual typology angle (ITA) measures constitutive pigmentation while skin phototypes (SPT) categories are based on sun reactiveness. However, the association between the two, ITA and SPT, is yet to be established. Since both systems provide six categories, recent studies have used ITA classifications as synonymous to SPT. The results of this study indicate that these cannot be utilized interchangeably. In conclusion, poor correlation between the six objective individual typology angle categories and the subjective Fitzpatrick skin phototype categories was established along with highlighting ITAs potential in photobiologic studies and objective standardization of skin type classifications.
Objectives To conduct a systematic review on the demographics, characteristics, management, treatment, complications, and outcomes of Ewing sarcomas in the craniofacial bones. Data Sources Using Cochrane Library, EmBase, and PubMed, the authors identified 71 studies to be included. Review Methods The Cochrane Library, EmBase, and PubMed databases were used to identify literature relating to Ewing sarcomas in the craniofacial bone to conduct a systematic review. Patient demographics, clinical characteristics, and treatment strategies were extracted. Results Seventy-one studies encompassing 102 patients were identified. The most common craniofacial locations involved were the frontal bone (16.7%, n = 17), nasal cavity (16.7%, n = 17), and temporal bone (14.7%, n = 15). Stratified by location, the most common presenting symptoms were frontal bone (palpable mass, n = 8, 47.1%), nasal cavities (epistaxis, n = 9, 52.9%), and temporal bones (headache, n = 5, 33.3%). The 3 most commonly used treatment strategies were a combination of surgical intervention/radiotherapy/chemotherapy (n = 43, 43%), a combination of radiotherapy/chemotherapy (n = 18, 18%), and a combination of surgical intervention/chemotherapy (n = 15, 15%). Patients who received a combination of surgical intervention/radiotherapy/chemotherapy experienced local recurrence rate of 16.6%. However, other combinations of therapies such as surgical intervention/chemotherapy and radiotherapy/chemotherapy had a lower local recurrence rate but were limited by small sample size. Most patients (79.0%) were disease free without evidence of recurrence. Conclusion Ewing Sarcoma of the craniofacial bones has a good prognosis when treated appropriately. Given that our study was limited by retrospective data, we advise clinicians to use the findings of this article with their own clinical judgment to determine which treatment strategy they should pursue.
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