病例报告：隐匿的静脉——当看似良性的喉部病变掩盖原发性喉部经典型卡波西肉瘤

文献信息

中文摘要

引言： 原发性喉部卡波西肉瘤（KS）在HIV阴性人群中极为罕见，仅有20例报道。其诊断具有挑战性，因为在早期KS中，它可能在组织学上模仿良性血管病变，从而导致诊断延迟。我们报告一例HIV阴性男性中累及喉部的原发性经典型KS，强调诊断陷阱以及HHV-8免疫组化的关键作用。
病例介绍： 一名六十多岁的男性患者，有40包年吸烟史，表现为进行性声音嘶哑和鼻塞。六年前，他曾被诊断为喉部淋巴管瘤，六年后的随后一个声门上病变被诊断为血管瘤，两者均行切除活检。数月后，他出现吞咽困难和弥漫性颈部、腋窝及纵隔淋巴结肿大。MRI显示一个5.7 cm边界不清的左声门上肿块，伴不均匀强化和坏死。喉部病变和颈部淋巴结活检显示梭形细胞增生，CD31、CD34、D2-40和HHV-8染色阳性，确诊为卡波西肉瘤。HIV检测为阴性。随后的检查发现双下肢出现新的紫罗兰色结节性病变。他接受了局部放疗和全身脂质体多柔比星治疗。治疗六个月后，他因放射性纤维化而非复发出现进行性吞咽困难和声音嘶哑。其病程因反复吸入性肺炎而复杂化，最终导致感染性休克和死亡。
结论： 原发性喉部卡波西肉瘤可发生于HIV阴性患者，并可能被误诊为淋巴管瘤或血管瘤等良性血管病变。对于喉部任何血管性梭形细胞病变，无论HIV状态如何，都应高度怀疑。HHV-8免疫组化是一项必要的确诊工具，即使初始活检看似良性，也应尽早进行。

英文摘要

INTRODUCTION: Primary laryngeal Kaposi sarcoma (KS) is exceptionally rare in HIV-negative, with only 20 reported cases. The diagnosis is challenging as it may histologically mimic benign vascular lesions in early-stage KS, which leads to delayed diagnosis. We present a case of a classic KS with primary laryngeal involvement in an HIV-negative male. highlighting the diagnostic pitfalls and the critical role of HHV-8 immunohistochemistry.
CASE PRESENTATION: A male patient in his sixties with a 40-pack-year smoking history presented with progressive hoarseness and nasal obstruction. Six years earlier, he had been diagnosed with a laryngeal lymphangioma, and a subsequent supraglottic lesion six years later was diagnosed as hemangioma, both with excisional biopsy. Several months later he developed dysphagia and diffuse cervical, axillary, and mediastinal lymphadenopathy. MRI revealed a 5.7 cm ill-defined left supraglottic mass with heterogeneous enhancement and necrosis. Biopsy of the laryngeal lesion and a cervical lymph node demonstrated spindle cell proliferation with positive staining for CD31, CD34, D2-40, and HHV-8, confirming the diagnosis of Kaposi sarcoma. HIV testing was negative. Subsequent examination revealed new violaceous nodular lesions on both lower extremities. He received local radiotherapy and systemic liposomal doxorubicin. Six months after treatment, he developed progressive dysphagia and hoarseness due to radiation-induced fibrosis, not recurrence. His course was complicated by recurrent aspiration pneumonia, leading to septic shock and death.
CONCLUSION: Primary laryngeal Kaposi sarcoma can occur in HIV-negative patients and may be misdiagnosed as benign vascular lesions such as lymphangioma or hemangioma. A high index of suspicion is warranted for any vascular spindle cell lesion of the larynx, regardless of HIV status. HHV-8 immunohistochemistry is an essential confirmatory tool that should be performed early, even when initial biopsies appear benign.