头颈部血管平滑肌瘤临床特点分析

Analysis of clinical features of head and neck angioleiomyomas

摘要:

目的 分析总结头颈部血管平滑肌瘤(ALM)的临床特点，为其临床诊疗提供参考。方法 回顾性分析2009年11月—2021年7月收治的17例头颈部ALM患者的临床病历及随访资料。结果 17例头颈部ALM患者，其中男7例，女10例，平均年龄49.5岁。发病部位位于耳廓、面颊部各6例，鼻前庭1例，鼻腔2例，鼻咽部1例，左上唇侧前庭沟1例。位于鼻前庭者诉鼻塞，位于鼻咽部者诉鼻塞伴涕中带血，其余患者均表现为无痛性肿块。17例患者，6例行CT检查，其中4例行增强CT扫描后可见肿物血管丰富，呈明显强化或不均匀强化；6例行鼻内镜检查；5例行细针穿刺细胞学检查均查见血细胞。15例由HE染色确诊，2例由HE染色和免疫组化确诊。根据Morimoto分类方法，实体型8例，静脉型4例，海绵型4例，实体型-海绵型1例。所有患者均采用手术治疗，其中1例位于鼻咽部的肿物术中大量出血，约2 000 mL，予以电凝、压迫止血，其余病例术中均无明显出血。术后平均随访5年，均无复发、恶变。结论 头颈部ALM临床表现各异，组织学和免疫组化检测可明确诊断。对位于头颈部腔隙内、瘤体较大、完整切除困难且术前影像学检查提示瘤体血供丰富的病例，术前可考虑行瘤体血管栓塞以减少术中出血。手术切除预后良好。

关键词:头颈部肿瘤;血管平滑肌瘤;诊断;外科手术

Abstract:

Objective To study the clinical characteristics of angioleiomyomas (ALM) in the head and neck, and to provide reference for its clinical diagnosis and treatment.Methods The clinical records and follow-up data of 17 patients with ALM of head and neck hospitalized from Nov 2009 to July 2021 were analyzed retrospectively.Results 10 females and 7 males were included, with an average age of 49.5 years. The locations and numbers of cases of the tumor were as follows: auricle (n=6), cheek (n=6), nasal vestibule (n=1), nasal cavity (n=2), nasopharynx (n=1), vestibular groove on the left upper lip side (n=1). Fifteen patients showed painless masses, one cases with nasal vestibule AML complained of nasal obstruction and one with nasopharyngeal AML showed nasal obstruction with blood in the snot. Computed tomography (CT) was performed in 6 cases, and 4 of them showed vascularity of the mass with significant enhancement or heterogeneous enhancement in enhanced CT scan. Nasal endoscopic examination was performed in 6 cases. The results of fine needle aspiration in 5 cases were blood cells. The diagnosis was confirmed by HE staining in 15 cases, and HE staining plus immunohistochemistry in 2. According to the Morimoto’s classification, the histological subtypes were reported as solid in eight cases, venous in four, cavernous in four and solid-cavernous in one. All cases were surgically treated, and massive intraoperative bleeding (about 2 000 mL) occurred in one case of nasopharyneal mass, which was staunched by electrocoagulation and compression. Postoperative follow-up revealed neither recurrence nor malignant change.Conclusions The clinical manifestations of AML in the head and neck are different. A definite diagnosis of the disease depends upon the histological and immunohistochemical examinations. For the cases with large masses located in the space of the head and neck, difficult to be removed completely and abundant blood supply revealed in the preoperative imaging test, preoperative vascular embolization may be considered to reduce intraoperative bleeding. Surgical excision is the optimal treatment with excellent prognosis.

Key words:Head and neck neoplasms;Angioleiomyomas;Diagnosis;Surgery