Almofada Hesham Saleh, Timms Michael Steven, Dababo M Anas
Department of Otolaryngology-Head and Neck Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Case Rep Otolaryngol. 2017;2017:4736895. doi: 10.1155/2017/4736895. Epub 2017 Aug 10.
We report an extremely rare case of ganglioneuroma involving the external auditory canal and middle ear.
Ganglioneuromas are rare benign mature tumors thought to originate from sympathetic ganglions, with the highest incidence in the retroperitoneum, adrenal medulla, and posterior mediastinum. We present a case of ganglioneuroma of the external auditory canal and middle ear. At the age of 12 months, the patient was diagnosed with neuroblastoma stage IV with metastasis to the squamous temporal bone, bone marrow, and skull base. He received a high-risk protocol regimen resulting in complete remission. The patient later presented with recurrent right ear discharge at the age of six years and was diagnosed with ganglioneuroma of external auditory canal and middle ear after appropriate investigations. We report in this article the clinical presentation, investigations, surgical intervention, and follow-up.
After the literature review and to our knowledge, this is the first reported case of its kind. Ganglioneuroma maturing from neuroblastoma is one of the theories describing pathophysiology of the disease. Ganglioneuroma should be considered in the differential diagnosis of patients presenting with recurrent ear discharge and decreased hearing in treated cases of neuroblastoma with metastases to temporal bone.
我们报告一例极其罕见的神经节神经瘤累及外耳道和中耳的病例。
神经节神经瘤是一种罕见的良性成熟肿瘤，被认为起源于交感神经节，在腹膜后、肾上腺髓质和后纵隔发病率最高。我们呈现一例外耳道和中耳神经节神经瘤病例。该患者12个月大时被诊断为IV期神经母细胞瘤，已转移至颞骨鳞状部、骨髓和颅底。他接受了高风险方案治疗并实现完全缓解。该患者6岁时出现右耳反复流脓，经适当检查后被诊断为外耳道和中耳神经节神经瘤。我们在本文中报告其临床表现、检查、手术干预及随访情况。
经文献检索并据我们所知，这是首例此类报告病例。神经节神经瘤由神经母细胞瘤成熟而来是描述该疾病病理生理学的理论之一。对于曾患转移至颞骨的神经母细胞瘤且接受过治疗，现出现反复耳流脓和听力下降的患者，鉴别诊断时应考虑神经节神经瘤。
