- 作者列表："Giavina-Bianchi MH","Festa-Neto C","Sanches JA","Teixeira MP","Waldvogel BC
BACKGROUND:The incidence and mortality of melanoma is increasing in many countries, including Brazil. Survival studies are still scarce in our country, but much needed to know and address this problem better. OBJECTIVE:To analyze the disease-specific survival of patients with invasive melanoma and to correlate it with clinical and histopathological variables. METHODS:Retrospective cohort analysis of 565 cases of invasive melanoma in a tertiary hospital with the objective of testing variables that could be associated with a worse prognosis, such as gender, phototype, thickness, histological type and presence of pre-existing clinical lesion at the site of the tumor. RESULTS:The worst survival rates were significantly associated with thicker tumors (p<0.001), male sex (p=0.014), high phototype (p=0.047), nodular melanoma (p=0.024) and "de novo" lesions (p=0.005). When all variables were adjusted for melanoma thickness, male patients (p=0.011) and "de novo" melanomas (p=0.025) remained associated with worse survival. STUDY LIMITATIONS:Retrospective study of a single tertiary hospital. CONCLUSIONS:Although the causes are still unknown, melanoma-specific survival was statistically worse for males and for "de novo" melanomas even after adjustment of tumor thickness.
背景: 在包括巴西在内的许多国家，黑色素瘤的发病率和死亡率都在增加。在我国，生存研究仍然很少，但是需要更好地了解和解决这个问题。 目的: 分析侵袭性黑色素瘤患者的疾病特异性生存，并将其与临床和组织病理学变量进行相关性分析。 方法: 回顾性队列分析 565 例侵袭性黑色素瘤在三级医院，目的测试变量可能与预后较差，如性别，光型，厚度，组织学类型和肿瘤部位存在预先存在的临床病变。 结果: 最差生存率与肿瘤较厚 (p<0.001) 、男性 (p = 0.014) 、高光型 (p = 0.047) 、结节性黑色素瘤 (p = 0.024) 和 “新生” 病变 (p = 0.005)。当调整所有变量的黑色素瘤厚度时，男性患者 (p = 0.011) 和 “新生” 黑色素瘤 (p = 0.025) 仍然与较差的生存率相关。 研究局限性: 单个三级医院的回顾性研究。 结论: 尽管病因仍然未知，但即使在调整肿瘤厚度后，男性和 “新生” 黑色素瘤的黑色素瘤特异性生存率在统计学上更差。
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.