Brock M E, Smith R J, Parey S E, Mobley D L
Department of Otolaryngology and Communicative Sciences, Baylor College of Medicine, Houston, TX 77030.
Int J Pediatr Otorhinolaryngol. 1987 Dec;14(2-3):133-40. doi: 10.1016/0165-5876(87)90024-3.
Lymphangiomas are relatively rare congenital hamartomas of the lymphatic system usually diagnosed in infancy and early childhood. Although the precise pathogenesis has not been clearly defined, abnormal development leads to formation of fluid-filled sinuses lined by vascular endothelium. In the head and neck, lymphangioma may encroach on vital structures and infiltrate surrounding tissues making complete surgical removal difficult. A number of children (n = 137) with lymphangioma have been treated at Texas Children's Hospital from 1972-1985. In 45 (33%), the tumors were diagnosed at birth and in an additional 51 (37%), by two years of age. Commonly affected areas included the head and neck (45%), trunk (26%), and axilla (17%). Mediastinal involvement occurred in 4%. Surgical removal was the preferred treatment method and with complete removal the recurrence rate was 6%; known incomplete removal increased this rate to 35%. Although lymphangiomas are slow growing, early removal was preferable: recurrences increased as the time interval between tumor identification and surgical intervention lengthened. Size fluctuation with a history of infection, another unfavorable prognostic factor, also increased recurrence rate.
淋巴管瘤是相对罕见的先天性淋巴系统错构瘤，通常在婴儿期和儿童早期被诊断出来。虽然确切的发病机制尚未明确，但发育异常会导致形成由血管内皮细胞衬里的充满液体的窦。在头颈部，淋巴管瘤可能侵犯重要结构并浸润周围组织，使得完全手术切除困难。1972年至1985年期间，德克萨斯儿童医院治疗了许多患有淋巴管瘤的儿童（n = 137）。其中45例（33%）在出生时被诊断出患有肿瘤，另有51例（37%）在两岁前被诊断出。常见受累部位包括头颈部（45%）、躯干（26%）和腋窝（17%）。纵隔受累的发生率为4%。手术切除是首选的治疗方法，完全切除后的复发率为6%；已知切除不完全会将该比率提高到35%。虽然淋巴管瘤生长缓慢，但早期切除更为可取：随着肿瘤确诊与手术干预之间的时间间隔延长，复发率会增加。感染史导致的大小波动是另一个不良预后因素，也会增加复发率。
