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一名患有严重睡眠呼吸障碍的患者的鼻咽脊索瘤:病例报告。

Nasopharyngeal chordoma in a patient with a severe form of sleep-disordered breathing: A case report.

作者信息

Radzikowska Joanna, Gronkiewicz Zuzanna, Kukwa Andrzej, Lisik Wojciech, Czarnecka Anna M, Krzeski Antoni, Kukwa Wojciech

机构信息

Department of Otorhinolaryngology, Medical University of Warsaw, Warsaw 00-739, Poland.

Department of Otolaryngology and Head and Neck Disease, University of Varmia and Mazuria School of Medicine, Olsztyn 10-082, Poland.

出版信息

Oncol Lett. 2015 Sep;10(3):1805-1809. doi: 10.3892/ol.2015.3393. Epub 2015 Jun 19.

Abstract

Nasopharyngeal chordoma is a rare type of malignant neoplasm that originates in the remnants of the notochord, a primitive tissue of embryonic origin preserved outside the axial skeleton. Approximately one-third of chordomas are located in the base of the skull, in the midline of the body. The slow growth rate of the tumor, which gradually fills the nasopharyngeal cavity, contributes to a delayed oncological diagnosis. Among its isolated and non-specific symptoms, the obstruction of the nasopharynx is dominant, thus, sleep-disordered breathing (SDB) may occur. The current study presents the case of a 32-year-old female patient who was incidentally diagnosed with a nasopharyngeal chordoma during a diagnostic examination for SDB. The diagnostic examination was performed as a part of a research program for pathologically obese patients who qualified for bariatric surgery. Following tumor resection, a significant improvement in various polysomnographic parameters occurred, including a decrease in the apnea hypopnea index from 53.5 to 6.4 and an increase in the mean saturation rate from 92.5 to 95%, confirming that an association exists between tumor obstruction of the nasopharynx and SDB. The incidental diagnosis of this rare type of neoplasm drew attention to diagnostic and therapeutic problems associated with nasopharyngeal chordomas. Furthermore, it indicated the necessity for the accurate laryngological examination of patients with SDB.

摘要

鼻咽脊索瘤是一种罕见的恶性肿瘤,起源于脊索的残余部分,脊索是一种起源于胚胎的原始组织,保留在中轴骨骼之外。大约三分之一的脊索瘤位于颅底,身体中线部位。肿瘤生长缓慢,逐渐填满鼻咽腔,这导致肿瘤诊断延迟。在其孤立且非特异性的症状中,鼻咽阻塞最为突出,因此可能会出现睡眠呼吸紊乱(SDB)。本研究报告了一例32岁女性患者的病例,该患者在因睡眠呼吸紊乱进行诊断性检查时偶然被诊断为鼻咽脊索瘤。该诊断性检查是作为一项针对符合减重手术条件的病态肥胖患者的研究项目的一部分进行的。肿瘤切除后,多项多导睡眠图参数有显著改善,包括呼吸暂停低通气指数从53.5降至6.4,平均血氧饱和度从92.5%升至95%,证实鼻咽肿瘤阻塞与睡眠呼吸紊乱之间存在关联。这种罕见肿瘤的偶然诊断引发了对鼻咽脊索瘤相关诊断和治疗问题的关注。此外,这表明对睡眠呼吸紊乱患者进行准确的喉镜检查是必要的。

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本文引用的文献

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Sleep-disordered breathing.睡眠呼吸紊乱。
Neurol Clin. 2012 Nov;30(4):1095-136. doi: 10.1016/j.ncl.2012.08.003. Epub 2012 Sep 15.
5
Extraosseous chordoma of the nasopharynx.鼻咽部骨外脊索瘤
AJNR Am J Neuroradiol. 2009 Apr;30(4):803-7. doi: 10.3174/ajnr.A1446. Epub 2009 Feb 4.
7
Chordoma: the nonsarcoma primary bone tumor.脊索瘤:非肉瘤性原发性骨肿瘤。
Oncologist. 2007 Nov;12(11):1344-50. doi: 10.1634/theoncologist.12-11-1344.

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