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表现为吞咽困难的咽旁间隙横纹肌瘤。

Rhabdomyoma of the parapharyngeal space presenting with dysphagia.

作者信息

Pichi Barbara, Manciocco Valentina, Marchesi Paolo, Pellini Raul, Ruscito Paolo, Vidiri Antonello, Covello Renato, Spriano Giusepe

机构信息

Department of Otolaryngology, Head and Neck Surgery, National Cancer Institute Regina Elena, Via E. Chianesi 53, 00144 Rome, Italy.

出版信息

Dysphagia. 2008 Jun;23(2):202-4. doi: 10.1007/s00455-007-9125-0. Epub 2007 Oct 27.

Abstract

Rhabdomyoma is an exceedingly rare soft tissue benign tumor of skeletal muscle origin classified into cardiac and extracardiac types based on location. Extracardiac rhabdomyoma is further classified into adult, genital, and fetal type depending on the degree of differentiation. Adult rhabdomyomas are rare, but morphologically characteristic, benign mesenchymal tumors with mature skeletal muscle differentiation that in 90% of cases arise in the head and neck region, mainly in the mucosa of the oropharynx, nasopharynx, and larynx, from the branchial musculature of third and fourth branchial arches. Most patients are between 40 and 70 years old, with a mean age of 60 years with a male predominance. Usually presenting symptoms include upper airway obstruction, Eustachian tube dysfunction, and mucosal or neck mass, but rarely does it arise as pure dysphagia. This article presents a case of parapharyngeal rhabdomyoma presenting with only progressive dysphagia.

摘要

横纹肌瘤是一种极为罕见的起源于骨骼肌的软组织良性肿瘤,根据位置可分为心脏型和心脏外型。心脏外横纹肌瘤根据分化程度进一步分为成人型、生殖器型和胎儿型。成人横纹肌瘤罕见,但形态特征明显,是具有成熟骨骼肌分化的良性间叶性肿瘤,90%的病例发生在头颈部区域,主要位于口咽、鼻咽和喉的黏膜,起源于第三和第四鳃弓的鳃肌组织。大多数患者年龄在40至70岁之间,平均年龄为60岁,男性居多。通常出现的症状包括上呼吸道梗阻、咽鼓管功能障碍以及黏膜或颈部肿块,但很少仅以单纯吞咽困难为表现。本文介绍了一例仅表现为进行性吞咽困难的咽旁横纹肌瘤病例。

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