进展缓慢的局部肿瘤如咽部肿块:你的诊断是什么?

S. Touihmi
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摘要

简介:淀粉样变性是一种特发性疾病,其特征是正常可溶性蛋白以异常纤维状形式在细胞外沉积。咽部受累很少见。病例总结:24岁男性,因慢性双侧鼻塞就诊,伴有上肢固体吞咽困难。鼻内窥镜检查发现多发性肿块累及咽部。颈部计算机断层扫描(CT)显示组织异质过程浸润鼻咽部、口咽部、软腭和下咽前壁。实现了全麻下直接喉镜检查。下咽活检标本解剖病理检查发现无定形脱细胞间质沉积,刚果红标记,偏振光下呈黄绿色苹果双折射。免疫组化诊断为AL型淀粉样变。排除全身累及的淀粉样变评估未见异常。在接下来的几个月里,患者的情况很好,但在7个月的随访中,咽淀粉样蛋白肿瘤复发,需要进行额外的保守内镜切除。讨论:头颈部淀粉样变是一种罕见的良性疾病,通常以局部淀粉样变的形式出现,很少累及咽部。临床症状和影像学对咽部受累特异性差,宏观上,诊断是解剖病理学。鉴于此病罕见,必须排除全身性累及。咽淀粉样蛋白肿瘤很难治疗,尽管手术切除,但通常会复发。
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Slow Progressing Localized Tumor Like Pharyngeal Mass: What Is Your Diagnosis?
Introduction: Amyloidosis is an idiopathic disorder characterized by the extracellular deposition of normally soluble proteins in an abnormal fibrillar form. Pharyngeal involvement is rare. Case summary: 24 years old man consulted for chronic bilateral nasal obstruction, associated with upper dysphagia to solids. Nasal endoscopy found multiples masses involving the the pharynx. A cervical computed tomography (CT) scan of the neck had shown tissular heterogeneous process infiltrating the walls of the nasopharynx, oropharynx: soft palate, and the anterior wall of the hypopharynx. A direct laryngoscopy under general anesthesia was realized. The anatomopathological examination of the hypopharyngeal biopsy sample found amorphous acellular interstitial deposits, marked by Congo red, showing yellow-green apple birefringence under polarized light. Immunohistochemistry diagnosed type AL amyloidosis. The amyloidosis assessment to rule out systemic involvement showed no abnormalities. The patient did well over the next several months, but recurrence of the pharyngeal amyloid tumor necessitated additional conservative endoscopic excisions in the 7 months follow up. Discussion: Amyloidosis in the head and neck is a rare and benign condition that usually takes the form of localized amyloidosis, Pharyngeal involvement is rare. Clinical symptomatology and imaging are poorly specific for pharyngeal involvement, macroscopically, the diagnosis is anatomopathological. Given the rarity of this disease, it is essential to rule out systemic involvement. Amyloid tumors in the pharynx are difficult to treat and commonly recur despite surgical excision.
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