University of California San Francisco School of Medicine, San Francisco, California.
Department of Dermatology, University of California San Francisco, San Francisco, California.
J Am Acad Dermatol. 2016 Jun;74(6):1234-1240.e9. doi: 10.1016/j.jaad.2015.12.006. Epub 2016 Feb 3.
Syringomas are benign adnexal tumors with distinct histopathologic features, including the characteristic comma ("tadpole") shaped tail comprised of dilated, cystic eccrine ducts. Clinically, syringomas typically present in adolescent females predominantly in the periorbital region. They may present as solitary or multiple lesions, and more rare sites of involvement include the genitals, palms, scalp, and the chest. Over the past 50 years, there have been >800 reported cases of syringoma either alone or in conjunction with a systemic syndrome, most commonly Down syndrome. The primary aim of this systematic review is to discuss the clinical features and associations of syringomas with a focus on the patient with multiple syringomas. Its secondary aims are to explore pathophysiology with a focus on multiple syringomas and provide comprehensive data on both traditional and novel treatments. Importantly, multiple syringomas present across a broad clinical spectrum. Though noted in many textbooks to be related to tumor syndromes, the association of syringomas with inherited tumor syndromes is only rarely reported in the literature. Despite multiple reported cases of syringoma, the pathophysiology remains poorly understood and treatment continues to pose a significant challenge.
汗管瘤是一种具有独特组织病理学特征的良性附属器肿瘤，包括特征性的逗号状（“蝌蚪”状）尾巴，由扩张的囊性小汗腺导管组成。临床上，汗管瘤通常发生于青少年女性，主要见于眶周区域。它们可以表现为单发或多发皮损，更罕见的受累部位包括生殖器、手掌、头皮和胸部。在过去的 50 年中，已有超过 800 例汗管瘤的单独或合并系统性综合征的报告，最常见的是唐氏综合征。本系统评价的主要目的是讨论汗管瘤的临床特征及其与多发性汗管瘤的关系，重点关注多发性汗管瘤患者。其次要目的是探讨其病理生理学，重点关注多发性汗管瘤，并提供传统和新型治疗方法的综合数据。重要的是，多发性汗管瘤具有广泛的临床表型。尽管在许多教科书中都提到与肿瘤综合征有关，但汗管瘤与遗传性肿瘤综合征的关系在文献中很少有报道。尽管有多个汗管瘤的报告病例，但病理生理学仍知之甚少，治疗仍然是一个重大挑战。
