原发性喉副神经节瘤伴多系统转移：一例病例报告

文献信息

中文摘要

背景： 喉副神经节瘤（LP）是一种罕见的神经内分泌肿瘤，其转移行为的证据有限。本病例的独特之处在于描述了LP伴多系统转移的诊断过程、治疗决策和临床结局，有助于更好地理解其侵袭性病程和管理挑战。
患者关切： 患者表现为进行性吞咽困难，后来伴有声音嘶哑和呼吸困难。
诊断： 临床检查和影像学显示喉部肿块。组织病理学和免疫组化分析证实为LP。
干预： 患者在拒绝部分喉切除术后接受了气管造口术。由于术后5个月肿瘤进展和吞咽困难，进行了部分喉切除术并广泛切除肿瘤。
结果： 术后四个月，恢复顺利，无局部复发，并成功拔除气管造口管。然而，在初次诊断后13个月（2020年11月22日），在肺、胰腺和皮下组织中发现多系统转移。患者于2021年2月9日因全身衰竭死亡。
结论： LP的诊断依赖于组织病理学和免疫组化的联合确认。完整手术切除对于预防复发至关重要。一旦发生转移，预后仍然很差，强调了早期诊断和多学科治疗的重要性。

英文摘要

RATIONALE: Laryngeal paraganglioma (LP) is a rare neuroendocrine tumor with limited evidence on its metastatic behavior. This case is unique because it describes the diagnostic process, therapeutic decisions, and clinical outcome of LP with multisystem metastases, contributing to a better understanding of its aggressive course and management challenges.
PATIENT CONCERNS: The patient presented with progressive dysphagia, which was later accompanied by hoarseness and dyspnea.
DIAGNOSES: Clinical examination and imaging revealed a mass involving the larynx. Histopathological and immunohistochemical analyses confirmed LP.
INTERVENTIONS: The patient underwent tracheostomy after declining partial laryngectomy. Due to tumor progression 5 months post-surgery and dysphagia, partial laryngectomy with extensive tumor resection was performed.
OUTCOMES: Four months postoperatively, recovery was uneventful, with no local recurrence and successful tracheostomy tube removal. However, at 13 months after initial diagnosis (November 22, 2020), multiple systemic metastases were detected in the lungs, pancreas, and subcutaneous tissues. The patient succumbed to systemic failure on February 9, 2021.
LESSONS: LP diagnosis relies on combined histopathological and immunohistochemical confirmation. Complete surgical resection is critical for preventing recurrence. Once metastasis develops, the prognosis remains poor, underscoring the importance of early diagnosis and multidisciplinary treatment.