关键词: catecholamine secreting tumor cerebellopontine angle extra‐adrenal paraganglioma head and neck neoplasms hypertension hypoxia‐inducible factor (HIF)‐1α

来  源:   DOI:10.1002/ccr3.8584   PDF(Pubmed)

Abstract:
Paragangliomas (PGLs) are rare neuroendocrine tumors. Sometimes, these tumors secrete excessive catecholamines, which results in the manifestations of various signs and symptoms, usually with a triad of hypertension, tachycardia, and headache. We report the case of a 42-year-old woman presenting with uncontrolled hypertension, right facial palsy, vomiting, and disturbed gait. Diagnosis for PGL was confirmed on postoperative histological examination of the excised mass and correlated with preoperative clinical and radiological findings. Tumor excision was done via a suboccipital craniotomy approach. Our case presents the typically severe features of a jugulotympanic PGL, but most importantly, it highlights the necessity of biochemical diagnosing, thorough probing of the causes of hypertension, and a multi-disciplinary approach in dealing with these tumors. Moreover, the case emphasizes necessitating the use of preoperative embolization in vascular tumors of the head and neck to avoid a hemorrhagic crisis during surgery. Unfortunately, due to a lack of adequate hospital funds, the surgeon had to proceed without preoperative embolization. Despite such a risk, the excision was a success.
摘要:
副神经节瘤(PGL)是罕见的神经内分泌肿瘤。有时候,这些肿瘤分泌过量的儿茶酚胺,导致各种体征和症状的表现,通常伴有高血压三联症,心动过速,和头痛。我们报告了一个42岁的女性,患有不受控制的高血压,右侧面神经麻痹,呕吐,和不安的步态。在切除肿块的术后组织学检查中证实了PGL的诊断,并与术前临床和放射学发现相关。通过枕下开颅手术进行肿瘤切除。我们的病例表现出典型的严重特征,但最重要的是,它强调了生化诊断的必要性,彻底调查高血压的原因,以及处理这些肿瘤的多学科方法。此外,该病例强调必须在头颈部血管肿瘤中使用术前栓塞术,以避免手术期间的出血性危机。不幸的是,由于缺乏足够的医院资金,外科医生不得不在没有术前栓塞的情况下进行手术.尽管有这样的风险,切除是成功的。
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