Oral and Maxillofacial Surgeon. University Hospital Infanta Leonor, Madrid, Spain.
Head of the Craniofacial and Maxillofacial Unit, Ruber International Hospital, Madrid, Spain.
J Med Case Rep. 2022 Feb 25;16(1):92. doi: 10.1186/s13256-021-03237-y.
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.
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.
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.
副神经节瘤是一种罕见的血管神经内分泌肿瘤，发生于肾上腺外副神经节组织。它们最常发生在颈动脉分叉处，在那里被称为颈动脉体瘤。大多数副神经节瘤是良性的、局部侵袭性的浸润性肿瘤。约 10%的副神经节瘤患者发生远处转移，10%的患者表现为多发或双侧肿瘤（多为颈动脉体瘤），10%的患者有副神经节瘤家族史。颈动脉体瘤的恶性转化已在 6%的病例中报告。
我们报告了一例 64 岁白人女性，其颈部有巨大的嗜铬细胞瘤肿块。在就诊前 20 年，患者曾尝试切除该肿块，但未成功。在过去 3 年中，患者感觉肿块增大速度加快，几乎是之前大小的两倍。血管磁共振成像显示左侧颈侧有一个 9 厘米的副神经节瘤，向外侧推移胸锁乳突肌，气管被推移 2 厘米。由于肿瘤行为发生变化，Ruber 国际医院的颌面外科团队决定在栓塞后进行手术切除。在手术过程中，肿瘤被从颈动脉分叉处轻轻分离并从颈动脉分叉处顺利取出。两个紧贴颈动脉内膜和后撕裂孔的小淋巴结被留在原处，以避免任何潜在的危及生命的并发症。最终的活检证实了最初的诊断为颈动脉体副神经节瘤，Ki-67 表达为 19%。由于肿瘤的侵袭性生长行为和 Ki-67 表达较高，患者被转介到 Ruber 国际医院的 CyberKnife 单位接受剩余淋巴结的治疗。
颈副神经节瘤的治疗较为困难，仍然是一个挑战。尽管手术或放射治疗的肿瘤控制率较高，但我们目前缺乏最佳治疗方案的共识。然而，在选择复杂的病例中，如我们报告的病例，手术和放射外科的联合应用可能允许通过最小的发病率实现肿瘤的完全局部控制。
