Marini Katerina, Florou Vasiliki, Skliris James Philip, Marangos Nikolaos, Kamargiannis Nikolaos
Department of Otorhinolaryngology-Head and Neck Surgery, 'G. Gennimatas' General Hospital, Thessaloniki, GRC.
Department of Pathology, 'G. Papanikolaou' General Hospital, Thessaloniki, GRC.
Cureus. 2023 Jul 17;15(7):e41997. doi: 10.7759/cureus.41997. eCollection 2023 Jul.
Paragangliomas are mostly benign, slow-growing, hypervascular tumors originating from neural crest derivatives. Head and neck (H&N) paragangliomas represent <1% of all H&N tumors and <5% are malignant. They are mostly non-secreting tumors that originate from autonomous parasympathetic paraganglia. We present a case of right middle ear jugulotympanic paraganglioma, a subtype of H&N paragangliomas, which had been misdiagnosed as otosclerosis for about 10 years. The patient was suffering from worsening tinnitus along with hearing impairment. High clinical suspicion of jugular paraganglioma prevented us from taking a biopsy. Complete surgical excision after preoperative embolization was decided. Embolization resulted in facial nerve paralysis, however, facial nerve rerouting was performed during the complete surgical excision of the tumor. The patient remains disease-free three years postoperatively, with House-Brackmann III facial nerve paralysis.
副神经节瘤大多为良性、生长缓慢的富血管肿瘤，起源于神经嵴衍生物。头颈部（H&N）副神经节瘤占所有头颈部肿瘤的比例不到1%，其中恶性的占比不到5%。它们大多是起源于自主副交感神经节的无分泌功能肿瘤。我们报告一例右中耳颈静脉鼓室副神经节瘤，这是头颈部副神经节瘤的一种亚型，曾被误诊为耳硬化症约10年。患者伴有耳鸣加重及听力障碍。对颈静脉副神经节瘤的高度临床怀疑使我们未进行活检。决定在术前栓塞后进行完整手术切除。栓塞导致面神经麻痹，然而，在肿瘤完整手术切除过程中进行了面神经改道。患者术后三年无疾病复发，但存在House-Brackmann III级面神经麻痹。
