CT and MR imaging findings of solitary nevus lipomatosus cutaneous superficialis: radiological-pathological correlation.
- 作者列表："Kawaguchi M","Kato H","Tomita H","Hara A","Matsuo M
OBJECTIVE:This study assessed the CT and MRI findings of solitary nevus lipomatosus cutaneous superficialis (NLCS). MATERIALS AND METHODS:Eleven patients with histopathologically and clinically confirmed solitary NLCS who underwent CT and/or MRI were enrolled. Radiological and histopathological findings of elevated lesions located above the level of the surrounding normal skin surface and coexisting subcutaneous lipoma-like lesions were assessed retrospectively. RESULTS:Elevated skin lesions were observed in all 11 patients; these lesions were pedunculated in 4 patients (36%) and broad-based in 7 (64%). The CT attenuation of elevated lesions was fat attenuation in 2 out of 7 patients (29%), slightly increased fat attenuation in 4 out of 7 (57%), and combined fat and soft-tissue attenuation in 1 out of 7 (14%). The MR signal intensity of elevated lesions on T1-weighted images was fat signal intensity in 2 out of 6 patients (33%), slightly decreased fat signal intensity in 3 out of 6 (50%), and combined fat signal intensity and hypointensity in 1 out of 6 (17%). Subcutaneous lipoma-like lesions with fat attenuation and/or fat signal intensity were observed in 6 out of 11 patients (55%). Histopathologically, various amounts of fatty tissue and collagenous fiber were observed within the elevated lesions in all 11 patients. CONCLUSION:The CT and MRI features of solitary NLCS were the broad-based or pedunculated elevated lesions, including fatty components. Additionally, subcutaneous lipoma-like lesions were frequently observed.
目的: 探讨单发性皮肤浅表脂肪瘤痣 (NLCS) 的CT和MRI表现。 材料和方法: 11 例经病理和临床证实的孤立性NLCS患者接受了CT和/或MRI检查。回顾性评估位于周围正常皮肤表面水平以上的隆起病变和并存的皮下脂肪瘤样病变的放射学和组织病理学表现。 结果: 11 例患者均出现皮损隆起，4 例 (36%) 有蒂病变，7 例 (6 4%) 有蒂病变。7 例患者中 2 例升高病灶的CT衰减为脂肪衰减 (2 9%)，7 例患者中 4 例 (57%) 脂肪衰减轻度增加，7 人中有 1 人 (4 4%) 合并脂肪和软组织衰减。MR信号强度的隆起性病变组T 1 加权像胖旌旗灯号强度 2 的 6 例患者 (3 3 %), 略有下降脂肪旌旗灯号强度 3 6 (50%),并结合脂肪信号强度和低信号在 1 出 6 (1 7%)。11 例患者中 6 例 (55%) 观察到脂肪衰减和/或脂肪信号强度的皮下脂肪瘤样病变。组织病理学观察，11 例患者的隆起病灶内均可见大量脂肪组织和胶原纤维。 结论: 单发NLCS的CT和MRI表现为广泛的或带蒂的隆起性病变，包括脂肪成分。此外，经常观察到皮下脂肪瘤样病变。
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.