Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Oper Neurosurg (Hagerstown). 2021 Oct 13;21(5):E427-E428. doi: 10.1093/ons/opab268.
Paragangliomas (PGLs) are benign hypervascular tumors that can develop in head and neck at different locations, primarily in the carotid bifurcation, jugular bulb, tympanic plexus, and vagal ganglia.1 Different gene mutations have been linked to the familial inherited forms, which can represent approximately 30% of all PGLs.1,2 These are classified into 5 different clinical syndromes: PGL 1 to 5.1 These patients have increased risk for synchronous and metachronous lesions requiring an extensive work-up for hormone secretion and other associated neoplasms, as well as attentive follow-up for lifelong management.1,3 Surgical resection is the best treatment option as it can be curative when the resection is total.2-4 Preservation of the lower cranial nerve function is central to the management of head and neck PGLs, given the gravity of bilateral injuries.3 Irradiation therapy should be considered if the risk for bilateral lower cranial nerve injuries is high.5 Surgically, intrabulbar resection with preservation of the medial wall of the jugular bulb protects the lower cranial nerve function.3 Other technical finesses, including maintaining the facial nerve in its bony fallopian canal (facial bridge), avoiding carotid artery sacrifice, preservation of the ear canal, and preoperative embolization, contributed markedly to outcome improvement.2,3 We report a case of a 34-yr-old male with PGL 3 with a left glomus jugulare tumor that recurred and a right carotid body tumor. Patient consented to surgery and photography. Image at 3:44 republished from Al-Mefty and Teixeira,3 with permission from JSNPG.
副神经节瘤(PGL)是良性的富血管肿瘤,可以在头颈部的不同位置发展,主要在颈动脉分叉处、颈静脉球、鼓膜丛和迷走神经节。1 不同的基因突变与家族遗传性形式有关,这些形式约占所有 PGL 的 30%。1,2 这些分为 5 种不同的临床综合征:PGL 1 到 5。1 这些患者有同步和异时性病变的风险增加,需要广泛的激素分泌和其他相关肿瘤的检查,以及终身管理的密切随访。1,3 手术切除是最佳治疗选择,因为当完全切除时可以治愈。2-4 保护颅神经功能是管理头颈部 PGL 的核心,因为双侧损伤的严重性。3 如果双侧颅神经损伤的风险高,应考虑放射治疗。5 手术时,保留颈静脉球内侧壁的球内切除术可保护颅神经功能。3 其他技术技巧,包括面神经保持在其骨管(面神经桥)内,避免颈总动脉牺牲,保护耳道,以及术前栓塞,显著有助于改善结果。2,3 我们报告了一例 34 岁男性的 PGL 3,左侧颈静脉球瘤复发,右侧颈动脉体瘤。患者同意手术和摄影。3 图像在 3:44 处转载自 Al-Mefty 和 Teixeira,3 经 JSNPG 许可。