Loos B M, Wieneke J A, Thompson L D
Department of Otolaryngology-Head and Neck Surgery, Georgetown University Medical Center, Washington, DC, USA.
Laryngoscope. 2001 Jul;111(7):1197-202. doi: 10.1097/00005537-200107000-00012.
Primary laryngeal angiosarcoma (LA) is rare without a reported series evaluating these tumors.
STUDY DESIGN/METHODS: Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.
Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, frequent atypical mitotic figures, and hemorrhage. All cases tested (n = 4) demonstrated immunoreactivity with antibodies to Factor VIII-RA and CD34. All patients had surgery followed by postoperative radiation (n = 3 patients). Three patients died with disease (mean, 17 mo), whereas one patient is alive with no evidence of disease at 18 years.
LA is a rare tumor, frequently associated with previous radiation, usually involving the supraglottis with characteristic histomorphologic and immunophenotypic features. LA has a poor prognosis, making appropriate separation from other conditions important.
原发性喉血管肉瘤（LA）罕见，尚无对这些肿瘤进行评估的系列报道。
研究设计/方法：从武装部队病理研究所的耳鼻咽喉科登记处回顾性检索出5例LA患者。
3例男性和2例女性，年龄29至71岁，表现为声音嘶哑（n = 4）和咯血（n = 1）。2例患者曾接受颈部放疗。肿瘤累及声门上区（n = 4），平均大小为3.1 cm。组织学上，所有肿瘤均有相互吻合的血管通道，内衬明显异型的内皮细胞并突向管腔，可见频繁的异型有丝分裂象及出血。所有检测的病例（n = 4）对VIII因子相关抗原和CD34抗体均呈免疫反应阳性。所有患者均接受了手术，术后3例患者接受了放疗。3例患者死于疾病（平均17个月），而1例患者在18年后存活且无疾病证据。
LA是一种罕见肿瘤，常与既往放疗有关，通常累及声门上区，具有特征性的组织形态学和免疫表型特征。LA预后较差，因此与其他疾病进行恰当鉴别很重要。
