Altissimi Giancarlo, Ralli Massimo, Sementilli Giulio, Fiorentino Francesco, Ciofalo Andrea, Greco Antonio, de Vincentiis Marco, Corsi Alessandro, Cianfrone Giancarlo
Department of Sense Organs, Sapienza University of Rome, Rome, Italy.
Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Rome, Italy.
Case Rep Otolaryngol. 2017;2017:7186768. doi: 10.1155/2017/7186768. Epub 2017 Nov 26.
Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extracardiac sites. Even though the head and neck region is the most common area of extracardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia, and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult-type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin, and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A, and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment.
横纹肌瘤是一种罕见的具有骨骼肌分化的良性间叶组织肿瘤,可发生于心脏或心脏外部位。尽管头颈部是心脏外横纹肌瘤最常见的发生部位,但喉部很少受累。我们报告一例85岁女性患者,有10天的呼吸困难、吞咽困难和声音嘶哑病史。头颈部计算机断层扫描显示下咽-喉部有一个强化的实性肿瘤,边界清晰,导致右侧梨状窝几乎完全闭塞,喉前庭气道腔缩小。患者接受了病变的完全内镜切除;组织学检查根据肿瘤细胞对结蛋白、肌红蛋白和肌肉特异性肌动蛋白的组织学特征和免疫反应性,而对细胞角蛋白、S-100、CD68R、嗜铬粒蛋白A和突触素无反应,诊断为成人型横纹肌瘤。由于临床和影像学特征对横纹肌瘤不具有特异性,组织学检查和免疫组化分析在成人型横纹肌瘤与其他喉部肿瘤的鉴别诊断中起着核心作用。正确的诊断对于避免不恰当的治疗至关重要。