Liu Shuzhong, Zhou Xi, Song An, Huo Zhen, Li William A, Rastogi Radhika, Wang Yipeng, Liu Yong
Department of Orthopaedic Surgery.
Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission.
Medicine (Baltimore). 2019 Sep;98(39):e17145. doi: 10.1097/MD.0000000000017145.
Paragangliomas are rare neuroendocrine tumors that originate in specialized cells derived from the neural crest with metastasis to the thoracic spine being among the rarest forms. Here, we are presenting a detailed analysis of a case of malignant paraganglioma in the thoracic spinal region in a 14-year-old boy. Our focus is to emphasize the importance of considering malignant paraganglioma as a diagnosis and guiding the perioperative management upon surgical treatment. The management of these unique cases has yet to be well-documented.
A 14-year-old boy presented with a 5-month history of continuous and progressive elevated blood pressure and back pain. The patient, who had been diagnosed of malignant paraganglioma in the left posterior mediastinum for 3 months, received surgical resection of paraganglioma in the left posterior mediastinum, which had involved the left intervertebral foramen of T4. However, the tumor was not completely resected during the first operation.
Magnetic resonance imaging of spine and positron emission tomography-computed tomography showed spinal cord compression secondary to the epidural component of the T4 mass, with increased marrow infiltration of the left T4 intervertebral foramen, which was difficult to be removed. Postoperative pathology confirmed the diagnosis of spinal involvement of malignant paraganglioma.
The patient underwent biopsy and percutaneous vertebroplasty of T4 and paravertebral lesions, and needle-track cement augmentation via a posterior approach.
The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 10-month follow-up visit. There were no other complications associated with the operation during the follow-up period.
Combined efforts of specialists from orthopedics, neurosurgery, thoracic surgery, and medical oncology led to the successful diagnosis and management of this patient. Malignant paraganglioma of thoracic spine, although rare, should be part of the differential diagnosis when the patient has a history of paraganglioma and presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the malignant paraganglioma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation is also a good choice for surgical treatment. However, we need to take the potential risk of complications in bone cement applications into full consideration.
副神经节瘤是一种罕见的神经内分泌肿瘤,起源于神经嵴衍生的特殊细胞,转移至胸椎是最罕见的形式之一。在此,我们对一名14岁男孩胸椎区域恶性副神经节瘤病例进行详细分析。我们的重点是强调将恶性副神经节瘤作为诊断考虑的重要性,并指导手术治疗时的围手术期管理。这些特殊病例的管理尚未有充分的文献记载。
一名14岁男孩有持续且进行性血压升高和背痛5个月的病史。该患者3个月前被诊断为左后纵隔恶性副神经节瘤,接受了左后纵隔副神经节瘤切除术,肿瘤累及T4左侧椎间孔。然而,首次手术时肿瘤未完全切除。
脊柱磁共振成像和正电子发射断层扫描 - 计算机断层扫描显示T4肿块硬膜外成分导致脊髓受压,左侧T4椎间孔骨髓浸润增加,难以切除。术后病理证实为恶性副神经节瘤脊髓受累。
患者接受了T4及椎旁病变的活检和经皮椎体成形术,并通过后路进行针道骨水泥强化。
手术后患者神经功能缺损明显改善,10个月随访时术后恢复顺利。随访期间无其他与手术相关的并发症。
骨科、神经外科、胸外科和医学肿瘤学专家的共同努力成功诊断并治疗了该患者。胸椎恶性副神经节瘤虽然罕见,但当患者有副神经节瘤病史并出现背痛和神经根病时,应列入鉴别诊断。当肿瘤导致神经功能缺损时,我们推荐采用后路进行恶性副神经节瘤的脊髓减压。骨水泥强化成形术也是手术治疗的一个好选择。然而,我们需要充分考虑骨水泥应用中潜在的并发症风险。