Sikora Andrew G, Shnayder Yelizaveta, Yee Herman, DeLacure Mark D
Department of Otolaryngology-Head and Neck Surgery, New York University School of Medicine, New York, New York, USA.
Ann Otol Rhinol Laryngol. 2008 Mar;117(3):172-6. doi: 10.1177/000348940811700303.
Kaposi sarcoma (KS) is a vascular tumor that can affect the mucosa of the upper aerodigestive tract. Although KS is the most common malignancy in patients with acquired immunodeficiency syndrome, it is rare in immunocompetent persons. We describe an unusual presentation of KS in 2 related individuals and describe our attempts to determine whether oropharyngeal KS is associated with human herpesvirus 8 (HHV-8).
All relevant clinical and surgical information, including information on tumor histopathologic and human immunodeficiency virus (HIV) serologic tests, was abstracted from the patient charts and electronic databases. HHV-8 immunohistochemistry was performed on paraffin-fixed specimens.
Both patient 1 and patient 2 (the nephew of patient 1) were referred for workup of a tonsillar mass that was pathologically confirmed to be KS. In each case, HIV serologic testing was negative, and a screening immunologic workup, including a quantitative natural killer cell count, a B- and T-lymphocyte count, and immunoglobulin analysis, also yielded findings that were within normal limits. Immunohistochemistry performed on 1 pathological specimen showed positive staining for the presence of HHV-8, the etiologic agent of KS.
The presence of oropharyngeal KS in 2 related HIV-negative individuals supports a role for genetic factors in susceptibility to KS, a common exposure to an infectious agent such as HHV-8, or both. Whereas most KS cases in industrialized countries are associated with immunodeficiency, clinical and laboratory data do not suggest that either of the patients described in this report are immunodeficient. Their susceptibility to KS may be secondary to a subtle inherited defect in host resistance to HHV-8, or another unknown factor.
卡波西肉瘤（KS）是一种可累及上呼吸道消化道黏膜的血管性肿瘤。虽然KS是获得性免疫缺陷综合征患者中最常见的恶性肿瘤，但在免疫功能正常者中较为罕见。我们描述了2名相关个体中KS的不寻常表现，并阐述了我们为确定口咽KS是否与人类疱疹病毒8型（HHV - 8）相关所做的尝试。
从患者病历和电子数据库中提取所有相关的临床和手术信息，包括肿瘤组织病理学和人类免疫缺陷病毒（HIV）血清学检测信息。对石蜡包埋标本进行HHV - 8免疫组化检测。
患者1和患者2（患者1的侄子）因扁桃体肿物前来检查，病理证实为KS。在每例中，HIV血清学检测均为阴性，包括定量自然杀伤细胞计数、B和T淋巴细胞计数以及免疫球蛋白分析在内的筛查性免疫检查结果也均在正常范围内。对1份病理标本进行的免疫组化显示HHV - 8（KS的病原体）染色呈阳性。
2名相关的HIV阴性个体中出现口咽KS，这支持遗传因素在KS易感性中发挥作用，可能是共同暴露于如HHV - 8等感染因子，或两者兼而有之。在工业化国家，大多数KS病例与免疫缺陷有关，而本报告中描述的两名患者的临床和实验室数据均未表明他们存在免疫缺陷。他们对KS的易感性可能继发于宿主对HHV - 8抵抗力的细微遗传缺陷或另一个未知因素。
