Cutaneous spindle cell adenolipoma with eccrine and apocrine differentiation.
- 作者列表："Farah M","Chung HJ
:Cutaneous spindle cell adenolipoma (SCAL) is a recently described rare variant of lipoma with 11 cases reported to date. Here we report a consultation case of a 77-year-old male who presented with a nodule on the right nasolabial fold, diagnosed as apocrine fibroadenoma or sebaceous hyperplasia by an outside pathologist. The specimen revealed an ill-defined dermal tumor composed of mature adipocytes, bland spindle cells, ropey collagen, and dilated eccrine and apocrine glands and ducts in a fibromyxoid stroma. The spindle cells were positive for CD34 and negative for S100 protein and SOX10. These findings are consistent with those of cutaneous SCAL. The pathogenesis of this entity is controversial and includes a hamartomatous process, derivation from adipose tissue surrounding eccrine glands, or preexisting glands entrapment within a growing lipoma. In the present case, the glandular component is extensive and includes both eccrine and apocrine differentiation, which has not been previously described and further supports the hamartomatous nature. Awareness of this rare entity is helpful to prevent confusion with other look-alike primary and metastatic cutaneous lesions.
: 皮肤梭形细胞腺脂肪瘤 (SCAL) 是最近描述的一种罕见的脂肪瘤变异型，迄今报告 11 例。在此我们报告一例 77 岁男性会诊病例，表现为右侧鼻唇沟结节，经外病理学家诊断为顶泌汗腺纤维腺瘤或皮脂腺增生。标本显示一个不明确的真皮肿瘤，由成熟的脂肪细胞、淡而无味的梭形细胞、粗大的胶原和扩张的小汗腺和大汗腺以及纤维黏液样间质中的导管组成。梭形细胞CD34 阳性，S100 蛋白和sox10 阴性。这些发现与皮肤SCAL的发现一致。该实体的发病机制存在争议，包括错构瘤过程，源自小汗腺周围的脂肪组织，或生长中的脂肪瘤内预先存在的腺体卡压。在本例中，腺体成分广泛，同时包括小汗腺和大汗腺分化，这一点以前没有描述过，进一步支持错构瘤性。对这种罕见实体的认识有助于防止与其他外观相似的原发性和转移性皮肤病变混淆。
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.