Song Mingzhi, Sun Kebin, Xia Tian, Zhou Lei, Li Yangyang, Sun Zhe, Zhang Yuchi, Zhang Xianbin, Sun Ran, Chen Bo, Tan Qingwei
Department of Orthopaedics, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, China.
Department of Orthopaedics, The Third Affiliated Hospital of Dalian Medical University, Dalian 116200, China.
J Thorac Dis. 2017 Mar;9(3):E202-E209. doi: 10.21037/jtd.2017.02.71.
Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. We report a rare case of pheochromocytoma of the anterior mediastinum in a 51-year-old female. A painful hard mass on the sternum was the only clinical manifestation. Imageological examination indicated that there might be a malignant mass on the anterior mediastinum and thoracic wall. The patient accepted surgical curettage and thoracic wall reconstruction. Based on pathological results and WHO definition, the final diagnosis was malignant pheochromocytoma. After six months follow-up, the patient had no recurrence or any symptom. Malignant pheochromocytoma in the anterior mediastinum invading the sternum is rare. A local painful mass may be the only clinical manifestation without special laboratory results. Surgery remains as the first choice for these patients. For this rare case, 3D reconstruction by special software may be a good method to realize individualized treatment. The final decision of the diagnosis should be based on pathological results, past medical history and WHO definition. Long-term follow-up is necessary, while other suspicious lesions should also be given sufficient attention.
嗜铬细胞瘤是一种分泌儿茶酚胺的神经内分泌肿瘤，通常发生于肾上腺髓质或交感神经节旁体。嗜铬细胞瘤累及前纵隔较为罕见，且可能不表现出典型症状。其临床表现可能不明确，准确诊断需要高度的怀疑指数。我们报告一例51岁女性前纵隔嗜铬细胞瘤的罕见病例。胸骨上的疼痛性硬块是唯一的临床表现。影像学检查表明前纵隔和胸壁可能存在恶性肿块。患者接受了手术刮除及胸壁重建。根据病理结果和世界卫生组织的定义，最终诊断为恶性嗜铬细胞瘤。随访6个月后，患者无复发及任何症状。前纵隔恶性嗜铬细胞瘤侵犯胸骨较为罕见。局部疼痛性肿块可能是唯一的临床表现，且无特殊实验室检查结果。手术仍然是这些患者的首选治疗方法。对于这一罕见病例，使用特殊软件进行三维重建可能是实现个体化治疗的良好方法。诊断的最终决定应基于病理结果、既往病史和世界卫生组织的定义。长期随访是必要的，同时对其他可疑病变也应给予充分关注。
