- 作者列表："Reinders EFH","Klaassen KMG","Pasch MC
BACKGROUND:Glomus tumors are rare benign painful tumors, frequently found in the subungual region. Complete surgical excision is essential for relief of symptoms. The main postoperative complications are tumor recurrence and nail dystrophy. OBJECTIVE:To evaluate the long-term outcome and the impact on quality of life (QoL) of glomus tumors after a transungual approach. MATERIALS AND METHODS:A retrospective cohort study was conducted. Twenty-six patients underwent transungual excision of subungual glomus tumors. A self-administered questionnaire was sent to evaluate the postoperative outcome. Glomus tumor-related QoL was investigated using modified nail psoriasis (NPQ10) and onychomycosis questionnaires. RESULTS:A response rate of 85% was achieved. After a mean follow-up of 63 months after transungual excision of the tumor, the mean Numeric Pain Rating Score had improved from 7.9 (±SD 1.8) preoperatively, to 0.8 (±SD 1.9) (p < .000). Quality of life improved significantly: the mean NPQ10-score improved from 5.5 (±SD 3.4) to 0.64 (±SD 2.1) (p < .000). Nail-related sequelae were not reported in any of the patients. CONCLUSION:Our study showed that glomus tumors cause impairment on QoL, mostly due to severe pain. Surgical excision with the transungual approach is an effective treatment, without permanent damage to the nail unit that gives relief of pain and improves QoL.
背景: 血管球瘤是一种罕见的良性疼痛性肿瘤，好发于甲下区域。完整的手术切除对缓解症状至关重要。术后主要并发症为肿瘤复发和甲营养不良。 目的: 评价经甲途径治疗血管球瘤的远期疗效及其对患者生活质量的影响。 材料与方法: 采用回顾性队列研究。26 例患者接受甲下血管球瘤经甲切除。发送自填问卷以评价术后结果。使用改良指甲银屑病 (NPQ10) 和甲真菌病问卷调查血管球肿瘤相关QoL。 结果: 有效率为 85%。经十二指肠切除术后平均随访 63 个月，平均数字疼痛评分从术前的 7.9 (± SD 1.8) 提高到 0.8 (± SD 1.9) (p <.000)。生活质量显著改善: 平均NPQ10-score从 5.5 (± SD 3.4) 改善至 0.64 (± SD 2.1) (p <.000)。任何患者均未报告指甲相关后遗症。 结论: 我们的研究表明，血管球瘤导致QoL受损，主要是由于严重的疼痛。经甲入路手术切除是一种有效的治疗方法，对指甲单位无永久性损伤，可缓解疼痛，提高QoL。
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.