背景:虽然汗管瘤是一种常见的汗腺良性肿瘤,还有一种极为罕见的恶性形式,称为丁香样小汗腺癌(SEC)。SEC通常表现出缓慢的生长和深度侵袭以及频繁的复发倾向。选择的治疗方法是根治性广泛切除术,这提出了一个困难的重建问题,尤其是当肿瘤位于面部中心时。
方法:在这种情况下,一名70岁的男性在7年前接受过原发性切除术的上唇和鼻底良性汗管瘤的同一位置被诊断为SEC.进行原发性根治性切除术,并立即进行Abbé皮瓣重建。然而,组织学显示切缘阳性,和3个额外的切除需要实现明确的边缘。初次切除后四个月,患者接受了创新的重建技术,不仅包括Abbé皮瓣,还包括功能性鼻内手术收获的鼻甲皮瓣和三级前额皮瓣.
结论:据我们所知,这是7年后首例怀疑良性汗管瘤恶性转化的病例报告。此外,从致癌和重建的角度来看,双侧使用鼻甲皮瓣重建鼻翼基底的鼻内衬里是不寻常的,在鼻腔重建中使用功能性鼻内手术来降低损伤皮瓣血管供应的风险是创新的。
BACKGROUND: Although syringoma is a common benign tumour of the sudoriferous gland, there is also an extremely rare malignant form known as syringoid eccrine carcinoma (SEC). SEC usually exhibits slow growth with deep invasion and a frequent tendency to relapse. The treatment of choice is radical wide resection, which poses a difficult reconstructive problem, especially when the tumour is located in the centre of the face.
METHODS: In this
case, a 70-year-old man was diagnosed with an SEC at the same location as a benign syringoma of the upper lip and nasal base that had undergone primary excision 7 years prior. Primary radical resection was performed with immediate Abbé flap reconstruction. Nevertheless, histology revealed positive margins, and 3 additional re-excisions were needed to achieve clear margins. Four months after the initial resection, the patient had undergone an innovative reconstruction technique including not only the Abbé flap but also a turbinate flap harvested with functional endonasal surgery and a three-stage forehead flap.
CONCLUSIONS: To the best of our knowledge, this is the first
case report of a suspect malignant transformation of a benign syringoma after 7 years. In addition, from oncoplastic and reconstructive points of view, the bilateral use of the turbinate flap for reconstructing the intranasal lining of the alar base is unusual, and the use of functional endonasal surgery in nasal reconstruction for reducing the risk of damaging the vascular supply of the flap is innovative.