- 作者列表："Sandhu N","Sauvageau AP","Groman A","Bogner PN
BACKGROUND:Cutaneous leiomyosarcoma is a rare dermal neoplasm usually arising from the pilar smooth muscle. It is considered a relatively indolent neoplasm, and there is debate whether designation as sarcoma is appropriate. Owing to some conflicting data in the literature, however, its behavior warrants further clarification. OBJECTIVE:To determine the clinical behavior and demographic and pathologic characteristics of cutaneous leiomyosarcoma. MATERIALS AND METHODS:The Surveillance, Epidemiology and End Results database was used to collect data on cutaneous leiomyosarcoma and 2 reference populations: cutaneous angiosarcoma (aggressive) and atypical fibroxanthoma (indolent). Demographic and oncologic characteristics were examined, and overall survivals (OS) and disease-specific survivals were compared. RESULTS:Leiomyosarcoma and atypical fibroxanthoma displayed lower stage (localized: 69.7% and 66.8% respectively), smaller size (<3 cm: 90.5% and 72%), and lower rates of disease-specific mortality (2.9% and 7.8%) compared with angiosarcoma. Patients with leiomyosarcoma had a 5-year disease-specific survival rate of 98% and OS rate of 85%. CONCLUSION:Cutaneous leiomyosarcoma shows outcomes similar to atypical fibroxanthoma. It is nearly always indolent and should be distinguished from more aggressive cutaneous and subcutaneous sarcomas. Clear communication of the biologic potential may be best achieved using alternate diagnostic terminology such as "atypical intradermal smooth-muscle neoplasm."
背景: 皮肤平滑肌肉瘤是一种罕见的皮肤肿瘤，通常起源于皮平滑肌。它被认为是一种相对惰性的肿瘤，命名为肉瘤是否合适存在争议。然而，由于文献中的一些相互矛盾的数据，其行为值得进一步澄清。 目的: 探讨皮肤平滑肌肉瘤的临床表现、人口学和病理学特征。 材料和方法: 利用监测流行病学学和最终结果数据库收集皮肤平滑肌肉瘤和 2 个参考人群的数据: 皮肤血管肉瘤 (侵袭性) 和非典型纤维黄色瘤 (惰性)。检查人口统计学和肿瘤学特征，并比较总体生存率 (OS) 和疾病特异性生存率。 结果: 平滑肌肉瘤和非典型纤维黄色瘤表现为较低的分期 (分别为 69.7% 和 66.8%)，较小的尺寸 (<3 cm: 90.5% 和 72%)，与血管肉瘤相比，疾病特异性死亡率较低 (2.9% 和 7.8%)。平滑肌肉瘤患者的 5 年疾病特异性生存率为 98%，OS率为 8 5%。 结论: 皮肤平滑肌肉瘤的结果与非典型纤维黄色瘤相似。它几乎总是惰性的，应该与更具侵袭性的皮肤和皮下肉瘤区分开来。使用替代诊断术语如 “非典型皮内平滑肌肿瘤” 可以最好地实现生物学潜能的明确交流。
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.