扁桃体原发性小细胞神经内分泌癌合并下咽鳞状细胞癌：一例病例报告

文献信息

中文摘要

背景： 扁桃体原发性小细胞神经内分泌癌（SCNEC）极为罕见，常被误诊为鳞状细胞癌。其与下咽鳞状细胞癌（HPSCC）共存的情况尚未见报道。合并主动脉弓动脉瘤进一步增加了治疗难度。
病例描述： 一名71岁男性因咽痛持续一个多月就诊。影像学和病理学检查证实为右侧扁桃体SCNEC（Ki-67阳性率约80%）和左侧HPSCC，同时合并主动脉弓动脉瘤和III期慢性肾脏病。经多学科会诊后，先行主动脉腔内修复术，2周后行根治性肿瘤切除术（包括扁桃体切除术、下咽肿瘤切除术及双侧颈部淋巴结清扫术）。术后病理显示手术切缘阴性，无淋巴结转移。围手术期并发症包括左上肢肌间静脉血栓形成和肺部感染，经治疗后均好转。建议行辅助放化疗，但患者及家属拒绝。术后6个月喉镜检查未见复发。
结论： 扁桃体原发性SCNEC合并HPSCC极为罕见。完整的诊断证据链（独立的影像学表现、不同的免疫表型以及排除肺部原发来源）至关重要。对于合并动脉瘤的高危患者，多学科“先处理血管问题，再行肿瘤切除”的序贯方法可降低围手术期风险。

英文摘要

BACKGROUND: Primary small cell neuroendocrine carcinoma (SCNEC) of the tonsils is extremely rare and is often misdiagnosed as squamous cell carcinoma. Cases coexisting with hypopharyngeal squamous cell carcinoma (HPSCC) have not been reported. The presence of concurrent aortic arch aneurysm further complicates treatment.
CASE DESCRIPTION: A 71-year-old male presented with pharyngeal pain lasting over one month. Imaging and pathological examinations confirmed right tonsillar SCNEC (with approximately 80% Ki-67 positivity) and left HPSCC, along with concurrent aortic arch aneurysm and stage III chronic kidney disease. Following multidisciplinary consultation, intracavitary aortic repair was performed first, followed by radical tumor resection 2 weeks later (including tonsillectomy, hypopharyngeal tumor resection, and bilateral cervical lymph node dissection). Postoperative pathology showed negative surgical margins and no lymph node metastasis. Perioperative complications included left upper limb intermuscular vein thrombosis and pulmonary infection, both of which resolved after treatment. Adjuvant radiotherapy and chemotherapy were recommended but declined by the patient and family. No recurrence was observed on laryngoscopy at 6 months postoperatively.
CONCLUSIONS: Primary SCNEC of the tonsils combined by HPSCC is extremely rare. A complete diagnostic evidence chain (independent imaging findings, distinct immunophenotypes, and exclusion of pulmonary primary origin) is essential. For high-risk patients with arterial aneurysms, a multidisciplinary "addressing vascular issues first, then performing tumor resection" sequential approach can reduce perioperative risks.