- 作者列表："Oudit D","Pham H","Grecu T","Hodgson C","Grant ME","Rashed AA","Allan D","Green AC
BACKGROUND:Giant basal cell carcinoma (GBCC) is a rare subgroup of basal cell carcinomas with a diameter of >5 cm. Current evidence about determining factors is conflicting, suggesting patient neglect, on the one hand, and biologically aggressive behaviour, on the other, with outcomes varying from clearance to death. We aimed to clarify the natural history of GBCC and its response to treatment. METHODS:We extracted information from clinical records of all patients with GBCC treated from 1998 to 2017 in a tertiary oncology hospital in northwest England. Associations between patient and tumour characteristics were investigated, and modes of treatment and outcomes were assessed. RESULTS:In the 20-year study period, 43 patients (median age 76 years; 23 (53%) female), 3 of whom had Gorlin syndrome, were treated for GBCCs. Median diameter was 6.3 cm, and median time to presentation was 5 years. Seven (16%) GBCCs arose from recurrent BCC, while the majority (84%) presented de novo. The size of GBCC was significantly correlated with delay in presentation (p = 0.03) but not with age or sex. Of 41 patients receiving definitive treatment, 19 GBCCs were treated by excision with ≤1 cm margin and none recurred during follow-up, compared with 10 recurrences of 23 treated with photodynamic therapy (PDT), and 1 of 7 recurred after radiotherapy. Two of 43 patients with GBCC (<5%) presented with extensive local invasion, one of whom also had distant metastases, and both died of the disease. CONCLUSION:The majority of GBCCs are not clinically aggressive and respond to conservative surgical treatment with a low risk of recurrence.
背景: 巨大基底细胞癌 (GBCC) 是一种罕见的基底细胞癌亚群，直径> 5 cm。目前关于决定因素的证据相互矛盾，一方面提示患者忽视，另一方面提示生物学攻击行为，结果从清除到死亡不等。我们旨在阐明GBCC的自然史及其对治疗的反应。 方法: 我们从英格兰西北部一家三级肿瘤医院 1998 年至 2017 年治疗的所有GBCC患者的临床记录中提取信息。研究了患者和肿瘤特征之间的关联，并评估了治疗模式和结果。 结果: 在 20 年的研究期间，4 3 例患者 (中位年龄 76 岁; 2 3 例 (5 3%) 女性)，其中 3 例发生Gorlin综合征，接受过GBCCs治疗。中位直径为 6.3 cm，中位就诊时间为 5 年。7 例 (16%) GBCCs来自复发性BCC，而大多数 (84%) 呈现从头开始。GBCC的大小与就诊延迟显著相关 (p = 0.03)，而与年龄或性别无关。在 4 1 例接受确定性治疗的患者中，1 9 例GBCCs采用切缘 ≤ 1 cm的切除治疗，随访期间无复发。比较 23 例光动力疗法 (PDT) 治疗后 1 0 例复发，7 例放疗后 1 例复发。43 例GBCC患者中有 2 例 (<5%) 表现为广泛的局部浸润，其中 1 例也有远处转移，均死于该病。 结论: 大多数GBCCs在临床上没有侵袭性，对保守手术治疗有反应，复发风险低。
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.