Abstract：
Syringocystadenocarcinoma papilliferum (SCACP) is a rare and aggressive malignant adnexal tumor originating from apocrine or pluripotent appendageal glands, often associated with a preceding syringocystadenoma papilliferum (SCAP) or nevus sebaceus (NS). This systematic review rigorously examines SCACP through an analysis of 78 cases documented between 1980 and 2024. The study aims to provide a comprehensive review of the clinical manifestations, diagnosis, treatment modalities, and outcomes associated with SCACP, while also reappraising its associations, particularly with NS. SCACP predominantly affects older adults, with an average age of 66.3 years and a slight male predominance, commonly presenting as ulcerated nodules or plaques on the scalp. This review highlights the aggressive nature of SCACP, evidenced by significant rates of metastasis and recurrence. Treatment is primarily surgical, with Mohs micrographic surgery offering potential benefits in terms of margin control and cosmetic outcomes. The association of SCACP with NS is critically evaluated, suggesting a complex etiopathogenesis and underscoring the importance of recognizing this association for timely diagnosis and management. Our review also briefly discusses potential pitfalls faced by clinicians in the diagnosis of SCACP. Our findings emphasize the need for standardized treatment protocols and further research into targeted therapies to improve patient outcomes in SCACP.
摘要：
视神经囊腺癌乳头瘤（SCACP）是一种罕见且侵袭性的恶性附件肿瘤，起源于大汗腺或多能附件腺，通常与先前的乳头瘤（SCAP）或色斑痣（NS）有关。本系统综述通过对1980年至2024年间记录的78例病例的分析，严格检查了SCACP。该研究旨在提供对临床表现的全面审查，诊断,治疗方式，以及与SCACP相关的结果，在重新评估其协会的同时，特别是NS。SCACP主要影响老年人，平均年龄为66.3岁，男性占主导地位，通常表现为头皮上的溃疡结节或斑块。这篇综述强调了SCACP的侵略性，显著的转移和复发率证明了这一点。治疗主要是手术，Mohs显微手术在切缘控制和美容效果方面提供了潜在的好处。对SCACP与NS的关联进行了严格评估，提示复杂的病因，并强调认识到这种关联对于及时诊断和治疗的重要性。我们的评论还简要讨论了临床医生在SCACP诊断中面临的潜在陷阱。我们的发现强调了标准化治疗方案和进一步研究靶向治疗以改善SCACP患者预后的必要性。
