头颈部血管平滑肌瘤

目的 提高对头颈部血管平滑肌瘤（angioleiomyoma，ALM）的认识。方法　收集并分析20例头颈部ALM的临床、影像、病理及术后随访资料。结果　女性多见，发病年龄平均52.5岁。无痛性肿块13例、鼻塞或（和）反复鼻出血4例、体检意外发现病变3例。B超示呈瘤体内有较丰富散在点状或条状血流信号；MRI示T1WI呈低-等信号，T2WI呈内部为非均质高信号、边缘为环形低信号，增强后明显强化。HE染色示血管壁平滑肌呈束状排列，血管壁增厚，管腔多呈裂隙状。免疫酶标检测示calponin、desmin和SMA阳性表达；部分病例孕激素、雌激素受体阳性表达。所有病例行肿块切除后无复发、恶变及转移。结论　头颈部ALM行B超和MRI检查有助于诊断，组织学检查和免疫酶标检测可明确诊断，性激素可呈阳性表达，手术治疗预后良好。

Angioleiomyoma in the head and neck region

A BSTR ACT] OB J ECTI V E To i mprove the recognization of clinical, imaging and pathological characteristics of angioleiomyoma (ALM) in the head and neck region. METHODS We retrospectively reviewed the data of 20 patients with ALM in the head and neck region between 2000 and 2012. RESULTS Seven male and 13 female patients were included in this study. The average age was 52.5 (from 28 to 74 years). The symptom in most cases (n=14) was the painless mass, 4 tumors originating in the nasal cavity presented with nasal obstruction or (and) epistaxis, and the other 3 cases were accidentally found by physical examination. The results of B-ultrasonography in 10 ALM cases of subcutaneous or deep space were homogeneously hypoechoic echo texture, straight and linear vessels in the tumor with convergence to one point with a circumscribed margin. MRI in 5 cases demonstrated typically a well-defined mass, which showed hypointensity or isointensity to muscle on T1WI, and heterogeneous hyperintensity on T2WI. All lesions showed obviously delayed enhancement on contrast MRI. HE stain showed that the tumors were formed by bundles of spindle-shape smooth muscle cells circumscribing numerous slit-like blood vessels in most cases. Immunoperoxidase staining revealed that the tumor cells were strongly positive for calponin, desmin and smooth muscle actin (SMA) in the cytoplasm of the smooth muscle cells. The positive expression of progestogen receptor and estrogen receptor was seen in 7 cases and 4 cases respectively among 10 cases. All patients underwent surgery, and recovered well postoperatively without recurrence or malignancy. CONCLUSION The clinical manifestations of ALM are nonspecific. ALM has distinctive imaging features in B-ultrasonic and MRI examination. Histological examination and immunoperoxidase staining can make a definite diagnosis of the disease. Progestogen receptor and estrogen receptor can be expressed in ALM. The postoperative prognosis is good.