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喉外骨肉瘤:一种极为罕见的肿瘤。

Extraskeletal osteosarcoma of the larynx: an extremely unusual tumour.

作者信息

Bhatt Nikita R, Kakked Gaurav A, Merchant Rajiv, Bhatt Rajiv

机构信息

Department of Surgery, SSG Hospital, Baroda, Gujarat, India.

Medical College Baroda, Baroda, Gujarat, India.

出版信息

BMJ Case Rep. 2014 Oct 16;2014:bcr2014206759. doi: 10.1136/bcr-2014-206759.

Abstract

Osteosarcoma of the larynx is probably the rarest mesenchymal tumour of the larynx, with only 16 cases reported so far. The majority of them occur in males between the sixth and eighth decades of life. Patients usually present with non-specific symptoms such as dysphonia and upper airway compromise. The most common site of distant metastasis is the lung. Clinically, the tumour follows an aggressive course and is associated with high mortality. The case we present is unusual as it occurred at a young age (38 years) as compared with the norm and it did not arise from the endolarynx, unlike many of the other cases. This was the only known case where a Pearson near-total laryngectomy was performed whereby the patient's natural speech mechanism was preserved. This surgery was possible because the contralateral half of the larynx was clearly disease free and the interarytenoid region was uninvolved. The patient underwent postoperative adjuvant external beam radiotherapy beginning 4 weeks after surgery. The patient is doing well after 15 months of follow-up and shows no signs of recurrence.

摘要

喉骨肉瘤可能是喉部最罕见的间叶组织肿瘤,迄今为止仅报道过16例。其中大多数发生于60至80岁的男性。患者通常表现为声音嘶哑和上呼吸道梗阻等非特异性症状。远处转移最常见的部位是肺。临床上,该肿瘤病程进展迅速,死亡率高。我们报道的这例病例不同寻常,因为与一般情况相比,它发生在年轻患者(38岁)身上,而且与其他许多病例不同,它并非起源于喉内。这是唯一一例已知的实施了Pearson近全喉切除术且保留了患者自然发声机制的病例。之所以能够进行这种手术,是因为对侧喉半侧无明显病变,且杓间区未受累。患者术后4周开始接受辅助性体外束放疗。经过15个月的随访,患者情况良好,未显示复发迹象。

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本文引用的文献

1
Osteosarcomas of the larynx.喉骨肉瘤
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