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一名儿科女性患者出现伴有多灶性骨受累的咽旁巨大神经节细胞瘤。

Parapharyngeal giant ganglioneuroma with multifocal bone involvement in a pediatric female patient.

作者信息

Salimov Asif, Süslü Nilda, Kuşçu Oğuz, Süslü Ahmet Emre, Akyol Mehmet Umut, Gököz Özay, Özoğul Ece

机构信息

Department of Otolaryngology Head and Neck Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

出版信息

Turk J Pediatr. 2016;58(6):669-674. doi: 10.24953/turkjped.2016.06.016.

Abstract

Ganglioneuroma is a rare benign tumor that originates from neural crest. Tumor tends to be slow growing, asymptomatic but can cause symptoms because of pressure to neighboring structures. In the head and neck region they are relatively rarely seen. We hereby present a rare case of multiple ganglioneuromas that were located in parapharyngeal space, iliac bone and other bones in a 13-year-old girl. Patient underwent surgery for the excision of a large mass, extending from parapharyngeal space to neck, with transparotid and transcervical combined approach. After operation, MIBG (iodine-123-meta-iodobenzylguanidine) scintigraphy was performed and involvement of parietooccipital bone, lumbal vertebra, right iliac wing medial cortex and left humerus were detected. No adjuvant therapy was given to the patient. There is no evidence of recurrence in the head and neck region in the following 12 months. In conclusion, complete surgical excision of the tumor, if possible, is the treatment of choice with high success rate. Close clinical and radiological follow-up for these tumors after surgery should be made.

摘要

神经节瘤是一种起源于神经嵴的罕见良性肿瘤。肿瘤往往生长缓慢,无症状,但由于对邻近结构的压迫可导致症状。在头颈部区域相对少见。我们在此报告一例罕见的多发神经节瘤病例,发生于一名13岁女孩的咽旁间隙、髂骨及其他骨骼。患者接受了手术,采用经腮腺和经颈联合入路切除了一个从咽旁间隙延伸至颈部的巨大肿块。术后进行了MIBG(碘-123-间碘苄胍)闪烁扫描,检测到顶枕骨、腰椎、右髂骨翼内侧皮质和左肱骨受累。未对该患者进行辅助治疗。在随后的12个月里,头颈部区域无复发迹象。总之,若可能,完整手术切除肿瘤是首选治疗方法,成功率高。术后应对这些肿瘤进行密切的临床和影像学随访。

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