Abstract：
BACKGROUND: Paragangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases.
METHODS: We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes.
CONCLUSIONS: The management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.
METHODS: We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes.
CONCLUSIONS: The management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.
摘要：
背景：副神经节瘤是一种罕见的血管神经内分泌肿瘤，发生在肾上腺神经节外组织。它们最常见于颈动脉分叉处，它们被称为颈动脉体瘤。大多数副神经节瘤是良性的，当地的侵略性，浸润性肿瘤.约10%的副神经节瘤患者发生远处转移，10％存在多发或双侧肿瘤（主要是颈动脉体瘤），10%有副神经节瘤家族史。6％的病例报道了颈动脉体瘤的恶性转化。
方法：我们介绍了一名64岁的高加索女性，颈部巨大的胶体瘤块。咨询前20年，患者尝试切除肿块失败。在过去的3年里,患者感觉到肿块以增加的速度增大，几乎是以前的两倍。血管造影磁共振成像显示，左子宫颈有9厘米的气管旁肿块，横向移位了胸锁乳突肌和2cm的气管。由于肿瘤行为的改变,Ruber国际医院的颌面团队决定在栓塞后手术切除肿瘤。在手术过程中，将肿瘤从颈动脉轻轻解剖，并从颈动脉分叉处顺利取出。将两个紧密粘附于颈内动脉外膜的小结节和后部撕裂孔留在原处，以避免任何可能危及生命的并发症。最终活检证实了颈动脉体副神经节瘤的初步诊断，并显示Ki-67表达为19％。由于肿瘤的侵袭性生长行为和高Ki-67表达，患者被转诊至Ruber国际医院射波刀科治疗其余淋巴结.
结论：宫颈副神经节瘤的治疗是困难的，仍然是一个挑战。尽管手术或放射治疗控制肿瘤的可能性很高，我们目前对最佳治疗方案缺乏共识.然而，在选定的复杂案例中，比如我们提出的案例，手术和放射外科的结合可以使局部肿瘤完全控制，发病率最低。
方法：我们介绍了一名64岁的高加索女性，颈部巨大的胶体瘤块。咨询前20年，患者尝试切除肿块失败。在过去的3年里,患者感觉到肿块以增加的速度增大，几乎是以前的两倍。血管造影磁共振成像显示，左子宫颈有9厘米的气管旁肿块，横向移位了胸锁乳突肌和2cm的气管。由于肿瘤行为的改变,Ruber国际医院的颌面团队决定在栓塞后手术切除肿瘤。在手术过程中，将肿瘤从颈动脉轻轻解剖，并从颈动脉分叉处顺利取出。将两个紧密粘附于颈内动脉外膜的小结节和后部撕裂孔留在原处，以避免任何可能危及生命的并发症。最终活检证实了颈动脉体副神经节瘤的初步诊断，并显示Ki-67表达为19％。由于肿瘤的侵袭性生长行为和高Ki-67表达，患者被转诊至Ruber国际医院射波刀科治疗其余淋巴结.
结论：宫颈副神经节瘤的治疗是困难的，仍然是一个挑战。尽管手术或放射治疗控制肿瘤的可能性很高，我们目前对最佳治疗方案缺乏共识.然而，在选定的复杂案例中，比如我们提出的案例，手术和放射外科的结合可以使局部肿瘤完全控制，发病率最低。
