- 作者列表："Chang C","Wu SP","Hu K","Li Z","Schreiber D","Oliver J","Givi B
OBJECTIVE:To analyze the patterns of care and survival of cutaneous angiosarcomas of the head and neck. STUDY DESIGN:Retrospective cohort study. SETTING:National Cancer Database. METHODS:The National Cancer Database was queried to select patients with cutaneous angiosarcoma of the head and neck between 2004 and 2015. For survival analysis, patients were included only if they received definitive treatment and complete data. Prognostic factors were analyzed by univariate and multivariable Cox regression. RESULTS:We identified 693 patients diagnosed with head and neck angiosarcomas during the study period. The majority were male (n = 489, 70.6%) and elderly (median, 77 years). A total of 421 patients (60.8%) met the criteria for survival analyses. These patients were treated with surgery and radiation (n = 178, 42.3%), surgery alone (n = 138, 32.8%), triple-modality therapy (n = 48, 11.4%), surgery and chemotherapy (n = 29, 6.9%), and chemoradiation (n = 28, 6.7%). With a median follow-up of 29 months, the 3-year survival was 50.1%. Patients undergoing surgery had better median survival than those who did not (38.1 vs 21.0 months, P = .04). Age, comorbidity, tumor size, and surgical margins were significant factors in univariate analyses. On multivariable analysis, age ≥75 years (hazard ratio, 2.65; 95% CI, 1.80-3.88; P < .001) and positive margins (hazard ratio, 1.91; 95% CI, 1.44-2.51; P < .001) predicted worse overall survival. CONCLUSION:Angiosarcoma of head and neck is a rare malignancy that affects the elderly. Surgical treatment with negative margins is associated with improved survival. Even with curative-intent multimodality treatment, the survival of patients aged ≥75 years is limited.
目的: 分析头颈部皮肤血管肉瘤的护理和生存模式。 研究设计: 回顾性队列研究。 单位: 国家癌症数据库。 方法: 查询国家癌症数据库，选择 2004 年至 2015 年间头颈部皮肤血管肉瘤患者。对于生存分析，仅在患者接受明确治疗和完整数据的情况下才纳入患者。通过单变量和多变量Cox回归分析预后因素。 结果: 我们在研究期间确定了 693 例诊断为头颈部血管肉瘤的患者。大多数是男性 (n = 489，70.6%) 和老年人 (中位数，77 岁)。共有 421 例患者 (60.8%) 符合生存分析标准。这些患者分别接受手术和放疗 (n = 178，42.3%) 、单纯手术 (n = 138，32.8%) 、三联疗法 (n = 48，11.4%) 、手术和化疗 (n = 29，6.9%) 和放化疗 (n = 28，6.7%)。中位随访 29 个月，3 年生存率为 50.1%。接受手术的患者中位生存期优于未接受手术的患者 (38.1 个月vs 21.0 个月，P = .04)。在单因素分析中，年龄、共病、肿瘤大小和手术切缘是重要因素。在多变量分析中，年龄 ≥ 75 岁 (风险比，2.65; 95% CI，1.80-3.88; P < .001) 和阳性边缘 (风险比，1.91; 95% CI，1.44-2.51; P < .001) 预示总生存期更差。 结论: 头颈部血管肉瘤是一种罕见的恶性肿瘤，累及老年人。切缘阴性的手术治疗与生存率改善相关。即使采用治愈性意向综合治疗，年龄 ≥ 75 岁的患者的生存期也有限。
METHODS::Blue rubber bleb naevus syndrome (BRBNS) is an extremely rare venous malformation that often manifests as multiple haemangioma-like lesions in the skin and gastrointestinal tract. The drug sirolimus plays a key role in the signalling pathway of angiogenesis and subsequent development of BRBNS and its use has been described in several case reports. We present a case series of four patients with BRBNS who exhibited good treatment response to sirolimus. All four patients were administered oral sirolimus at doses of 1.0-1.5 mg/m2 /day with a target drug level of 5-10 ng/mL and median treatment duration of 20 months. All patients had a reduction in the size of the lesions and a normalization of coagulopathy with tolerable drug adverse reactions at follow-up. Sirolimus may be effective and safe in paediatric patients with BRBNS. Further prospective studies are suggested to evaluate the long-term effectiveness of this drug.
METHODS:BACKGROUND:Human papillomavirus (HPV) infections are associated with common dermatologic and nondermatologic diseases. Although HPV vaccines are well established as preventive measures for genital warts and cervical neoplasia, their use as therapeutic agents deserves greater attention. OBJECTIVE:To evaluate the use of HPV vaccine(s) as a treatment modality for cutaneous and/or mucosal disease. METHODS:A primary literature search using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted in January 2019 by using the PubMed and Cochrane databases. RESULTS:A total of 63 articles with 4439 patients were included. The majority of patients with cutaneous warts, recurrent respiratory papillomatosis, and squamous and basal cell carcinomas were successfully treated with HPV vaccination. Preliminary data on patients with pre-existing anogenital warts, cervical intraepithelial neoplasia, anal intraepithelial neoplasia, and vulvar intraepithelial neoplasia is promising. LIMITATIONS:This review was limited by the lack of controls, patients' previous HPV vaccination status, and publication bias. CONCLUSION:The commercially available three-dose, quadrivalent HPV vaccine is a potential therapeutic option for the treatment of cutaneous warts, recurrent respiratory papillomatosis, and squamous and basal cell carcinomas. Noncommercially available HPV vaccines demonstrate therapeutic response for treating anogenital warts, cervical intraepithelial neoplasia, anal intraepithelial neoplasia, and vulvar intraepithelial neoplasia. The vaccine's efficacy as an adjunct therapy for HPV-associated cutaneous and/or mucosal disease warrants further exploration.
METHODS::Our understanding of melanoma precursors and progression to melanoma has developed as a result of advances in the field of molecular diagnostics. We now better understand the potential for genetic heterogeneity within a single lesion. Combined tumors can pose a diagnostic challenge when deciding the line between benign and malignant, which in turn has direct implications for patient management. Primary cilia (PC) are ubiquitous sensory organelles that have essential functions in cellular proliferation, differentiation, and development. The ciliation index (percentage of ciliated melanocytes) has been shown to reliably differentiate melanoma, which fail to ciliate, from melanocytic nevi, which retain PC. We therefore analyzed the potential for using the ciliation index to differentiate benign and malignant components in combined melanocytic lesions. We collected patient samples (n = 10) of unequivocal combined lesions with both melanoma and associated nevus components. Melanocytes were highlighted with SOX10 and costained with gamma-Tubulin and acetylated alpha-Tubulin to highlight the basal body and cilium, respectively. The number of melanocytes retaining cilia under high-power microscopy was examined. The melanoma component had average of 4% ciliation (SD: 7%), whereas the associated nevus component was significantly higher with 59% ciliation (SD: 17%). These data show that PC may be a reliable means of distinguishing benign from malignant components within a single tumor. The ciliation index may be a helpful tool in distinguishing challenging cases of combined lesions of melanoma in situ with a dermal nevus component from invasive melanoma, thus promoting improved staging and clinical management.