Periocular intraepithelial sebaceous neoplasia: critical appraisal of nomenclature and prognostic importance.
- 作者列表："Margo CE
:Intraepithelial sebaceous neoplasia in the forms (or subclassification) of pagetoid spread and carcinoma in situ is a common feature of periocular sebaceous carcinoma and is associated with less favourable outcomes. Seminal studies of periocular sebaceous carcinoma in the 1980s indicated that the two patterns of intraepithelial spread had differing influences on prognosis with pagetoid invasion being worse. Later studies reported conflicting results, but careful inspection of those studies revealed considerable variation in what was meant by pagetoid invasion. Different interpretations of pagetoid spread continue, leading to ambiguous results in clinical studies and miscommunication with potential unintended decisions affecting clinical management. This paper reviews the background leading to the frequent interchangeable use of pagetoid spread with in situ sebaceous carcinoma and how this problem confounds interpretation of clinical studies. The author recommends that for effective communication, all morphological patterns of in situ spread of sebaceous carcinoma fall under the term intraepithelial sebaceous neoplasia, which can be accompanied by subclassification whenever desired.
: Pagetoid扩散和原位癌形式 (或亚分类) 的上皮内皮脂腺瘤是眼周皮脂腺癌的常见特征，与较差的预后相关。20 世纪 80 年代对眼周皮脂腺癌的精液研究表明，两种上皮内扩散模式对预后的影响不同，pagetoid浸润更差。后来的研究报告了相互矛盾的结果，但仔细检查这些研究发现，pagetoid入侵的含义有相当大的差异。Pagetoid传播的不同解释仍在继续，导致临床研究的结果模棱两可，与影响临床管理的潜在意外决策沟通不畅。本文综述了导致pagetoid扩散与原位皮脂腺癌频繁互换使用的背景，以及这个问题如何混淆临床研究的解释。作者建议，为了有效沟通，皮脂腺癌原位扩散的所有形态模式都落在 “上皮内皮脂腺瘤” 一词之下，只要需要，可以伴有亚分类。
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.