Department of Neurosurgery, Universidade de Caxias do Sul, Caxias do Sul, Brazil.
Acta Neurochir (Wien). 2010 Mar;152(3):475-80. doi: 10.1007/s00701-009-0362-x.
Paragangliomas are tumors that arise from the paraganglion system, which is a component of the neuroendocrine system. Approximately 10% are located in the extra-adrenal paraganglion system. Paragangliomas of the spine, however, are rare. They usually present as an intradural tumor in the cauda equina. There are only three reports of primary intraosseous paragangliomas of the sacrum.
A 69-year-old man presented with low back pain and urinary incontinence. Imaging revealed a large intraosseous mass at S2, S3 and S4. Surgical resection was accomplished through a posterior midline incision exposing the spine from L5 to the coccyx. The tumor was located in the extradural space. It was friable, grayish and bleeding. Total tumor removal was performed, with normal bone margins. Follow-up at 2 years showed complete resolution of the preoperative symptoms and no evidence of local recurrence.
Although rare, the possibility of paraganglioma should be included in the differential diagnosis of sacral tumors. The majority of the spinal paragangliomas are benign, slowly growing tumors with low proliferative activity. Despite these characteristics, local recurrence has been reported in cases of both macroscopically total and subtotal resection. Postoperative radiation therapy for patients with incomplete excision may not prevent recurrence, so gross tumor removal should be the goal of surgery.
副神经节瘤起源于副节神经系统，这是神经内分泌系统的一个组成部分。大约 10%的副神经节瘤位于肾上腺外的副节神经系统。然而，脊柱副神经节瘤很少见。它们通常表现为马尾神经根的硬膜内肿瘤。仅有三例报告发生于骶骨的原发性骨内副节瘤。
一名 69 岁男性因腰痛和尿失禁就诊。影像学检查显示 S2、S3 和 S4 存在大的骨内肿块。通过后路正中切口完成了手术切除，显露了从 L5 到尾骨的脊柱。肿瘤位于硬膜外腔。它易碎、灰白色且有出血。进行了全肿瘤切除，骨缘正常。2 年随访显示术前症状完全缓解，无局部复发迹象。
尽管罕见，但在骶骨肿瘤的鉴别诊断中应考虑副神经节瘤的可能性。大多数脊柱副神经节瘤是良性、生长缓慢、增殖活性低的肿瘤。尽管具有这些特征，但在肉眼全切除和次全切除的病例中均有局部复发的报道。对于不完全切除的患者，术后放疗可能无法预防复发，因此大体肿瘤切除应是手术的目标。
