Oral and salivary gland angiosarcoma: A clinicopathologic study of 29 cases
Oral and salivary gland angiosarcoma: A clinicopathologic study of 29 cases
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DOI：
10.1097/01.mp.0000056986.08999.fd
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发表时间：
2003-03-01
期刊：
影响因子：
7.5
通讯作者：
Childers, ELB
中科院分区：
文献类型：
--
作者：
Fanburg-Smith, JC;Furlong, MA;Childers, ELB
摘要口腔及唾液腺之血管瘤极为罕见，多为病例报告。我们想研究一系列口腔和唾液腺血管瘤的临床病理特征。编码为“血管肉瘤”的病例来自武装部队病理研究所口腔颌面部病理科。审查并记录患者文件夹和病理学;获得免疫组织化学和随访。入选需要口腔或唾液腺位置、血管形成生长、细胞学检查、有丝分裂和血管标记物。排除皮肤、骨和皮下血管瘤。包括原发性和继发性（转移性）口腔血管瘤。22例原发性血管瘤分别位于舌9例、腮腺4例、上颌窦4例、颌下腺3例、软腭和硬腭各1例。7例继发性血管瘤累及牙龈4例，腮腺3例。总体而言，患者年龄范围为6-90岁（平均55岁）。男15例，女14例。症状包括一个肿块，最近扩大和出血。肿瘤大小范围为0.8-7.0 cm（平均2.6 cm）。组织学上，所有肿瘤均为血管形成; 86%为实性肿瘤，17%有明显的乳头状区域。8例（28%）被归类为上皮样亚型。免疫组化染色显示19/21例肿瘤细胞表达因子VII γ g，16/19例表达CD 31，7/12例表达CD 34，1/1例表达Ulex。原发性肿瘤分为低级别（n = 7，除唾液腺外的所有位置）、中等级别（n = 7）和高级别（n = 8）;所有继发性肿瘤均为高级别。对14/22例原发性和7/7例继发性血管瘤进行了随访。在原发性肿瘤中，2例舌血管肉瘤患者在1年和9年时死亡，但4例患者平均存活7.3年（范围，1-13年）。4例原发性涎腺血管肉瘤患者平均存活5.8年（范围1-14年），1例仅晚期（15年）转移和死亡（20年）。三名原发性唇血管肉瘤患者在平均14.3年（范围，13-16年）内无疾病。在继发性肿瘤中，3例涎腺血管肉瘤患者在1年内死亡，4例继发性牙龈血管肉瘤患者均在3年内死于疾病。通过对原发性口腔和唾液腺血管肉瘤患者的分级随访评估，5例高分级肿瘤患者在平均7.6年内没有疾病证据（范围，1-16年），3例中度肿瘤患者在平均12.7年内没有疾病证据（范围，11-14年），2例中度肿瘤患者在9年和20年死于疾病，3例低度肿瘤患者在平均6.3年内没有疾病证据（范围，1-14年），1例低级别肿瘤患者在1年时死于疾病。原发性口腔和唾液腺血管瘤虽然罕见，但大多数累及成人的舌、腮腺和唇，通常预后相对较好。虽然该区域最常见的血管肉瘤形态是梭形血管形成和实性，但几乎三分之一的口腔和唾液腺血管肉瘤是罕见的上皮样血管肉瘤变体。大多数牙龈和少数腮腺血管瘤似乎是其他部位的转移，许多患者在3年内死亡。尽管主要是高或中等级别的形态，但原发于舌、唾液腺和唇的血管肉瘤患者的预后好于原发于皮肤或深部软组织血管肉瘤的患者，包括那些原发于舌、唾液腺和唇的血管肉瘤患者。
Angiosarcomas of the oral and salivary gland area are extremely rare, mostly presented as case reports. We wanted to study the clinicopathologic features of a series of oral and salivary gland angiosarcomas. Cases coded as "angiosarcoma" were retrieved from the Oral and Maxillofacial Pathology Department of the Armed Forces Institute of Pathology. Patient folders and pathology were reviewed and recorded; immunohistochemistry and follow-up were obtained. Inclusion required oral or salivary gland location, vasoformative growth, cytologic atypia, mitoses, and vascular markers. Skin, bone, and subcutaneous angiosarcomas were excluded. Primary and secondary (metastatic) oral angiosarcomas were included. The 22 primary angiosarcomas involved tongue (n = 9), parotid (n = 4), Up (n = 4), submandibular gland (n = 3), and 1 each of soft and hard palate. The 7 secondary angiosarcomas involved the gingiva (n = 4) and parotid gland (n = 3). Overall, patient ages ranged from 6-90 years (mean, 55 years). There were 15 males and 14 females. Symptoms included a mass with recent enlargement and bleeding. Tumor sizes ranged from 0.8-7.0 cm (mean, 2.6 cm). Histologically, all tumors were vasoformative; 86% had solid and 17% had distinctive papillary areas. Eight (28%) were classified as the epitheliold subtype. Immunohistochemical stains showed that the tumor cells were positive for Factor VIIIrag in 19/21, CD31 in 16/19, CD34 in 7/12, and Ulex in 1/1. Primary tumors were classified as low grade (n = 7, in all locations except salivary gland), intermediate (n 7), and high grade (n = 8); all secondary tumors were high grade. Follow-up was available on 14/22 primary and 7/7 secondary angiosarcomas. Of primary tumors, two tongue angiosarcoma patients died at 1 and 9 years, but 4 were alive without disease over a mean of 7.3 years (range, 1-13 years). Four primary salivary gland angiosarcoma patients were alive without disease over a mean of 5.8 years (range, 1-14 years), and 1 had only a late (15 years) metastasis and death (at 20 years). Three primary lip angiosarcoma patients were without disease over a mean of 14.3 years (range, 13-16 years). Of secondary tumors, three salivary gland angiosarcoma patients died within 1 year, and all four secondary gingival angiosarcoma patients died of disease within 3 years. Assessing follow-up of primary oral and salivary gland angiosarcoma patients by grade, 5 patients with high-grade tumors had no evidence of disease over a mean of 7.6 years (range, 1-16 years), 3 patients with intermediate-grade tumors had no evidence of disease over a mean of 12.7 years (range, 11-14 years), 2 patients with intermediate-grade tumors died of disease at 9 and 20 years, 3 patients with low-grade tumors had no evidence of disease over a mean of 6.3 years (range, 1-14 years), and 1 patient with low-grade tumor died of disease at 1 year. Primary oral and salivary gland angiosarcomas, albeit rare, mostly involve the tongue, parotid gland, and lip of adults, often with relatively good outcome. Although the most common angiosarcoma morphology in this area is spindled vasoformative and solid, almost one third of oral and salivary gland angiosarcomas are the rare epithelioid angiosarcoma variant. Most gingival and few parotid angiosarcomas appear to be metastases from other locations, with many patients succumbing to death within 3 years. Despite predominantly high- or intermediate-grade morphology, patients with primary angiosarcoma of the tongue, salivary gland, and lip have a better prognosis than do patients with primary cutaneous or deep soft tissue angiosarcoma, including those patient