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喉腺性错构瘤:病例报告

Glandular hamartoma of the larynx: report of a case.

作者信息

Leoncini Giuseppe, Maio Vincenza, Mirabile Lorenzo, Baggi Roberto, Franchi Alessandro

机构信息

Department of Human Pathology and Oncology, University of Florence, Viale G.B., Morgagni 85, 50134, Florence, Italy.

出版信息

Auris Nasus Larynx. 2008 Mar;35(1):149-51. doi: 10.1016/j.anl.2007.03.015. Epub 2007 Sep 11.

Abstract

Glandular hamartoma is an extremely rare congenital malformation of the larynx. Presenting symptoms result from airways obstruction and may include slowly rising respiratory distress, stridor, changes in voice, eating and activity levels. Management consists in local mass excision with a good functional result and prognosis. Recurrences are usually associated with incomplete removal. We present a 3-month-old infant with a history of stridor and respiratory distress caused by a firm 0.4cm wide and 1.4cm long mass arising in the supraglottic region and detected with endoscopic approach. The lesion was excised endoscopically with an uneventful postoperative course and an excellent long-term prognosis. Histopathologically the lesion consisted of mature tissues with abnormal growth and disorganized architecture, chiefly composed of mature glandular structures, smooth muscular fibers, mature fat, surrounded by fibrous stroma and covered by typical squamous epithelium. The aim of our report is to underline how this condition must be considered by physicians, paediatricians and anaesthetists as an important cause of airway obstruction.

摘要

腺性错构瘤是一种极其罕见的喉部先天性畸形。其出现的症状是由气道阻塞引起的,可能包括逐渐加重的呼吸窘迫、喘鸣、声音改变、饮食和活动水平变化。治疗方法是局部肿块切除,功能恢复良好,预后较好。复发通常与切除不完全有关。我们报告一例3个月大的婴儿,有喘鸣和呼吸窘迫史,由内镜检查发现声门上区有一个质地坚硬、宽0.4厘米、长1.4厘米的肿块。该病变通过内镜切除,术后过程顺利,长期预后良好。组织病理学检查显示,病变由生长异常和结构紊乱的成熟组织组成,主要由成熟的腺结构、平滑肌纤维、成熟脂肪组成,周围有纤维基质,表面覆盖典型的鳞状上皮。我们报告的目的是强调医生、儿科医生和麻醉师必须将这种情况视为气道阻塞的一个重要原因。

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