Zhang Liyang, Liao Quan, Hu Ya, Zhao Yupei
Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, No, 1, Shuaifu Garden, Dongcheng District, 100730 Beijing, China.
World J Surg Oncol. 2014 Jul 17;12:218. doi: 10.1186/1477-7819-12-218.
Paragangliomas are neoplasms that arise from extra-adrenal chromaffin cells. Pancreatic paragangliomas are rare, and few are malignant. To the best of our knowledge, no cases of functional pancreatic paragangliomas have been reported in the literature to date. We present two cases of pancreatic paragangliomas with pathological confirmation. In the case 1, clinical testing and pathological analysis revealed functional and malignant characteristics of the tumor, which carried a poor prognosis. In case 2, functional paraganglioma was suspected. The clinical presentations and outcomes of these two patients are summarized, and the relevant literature is reviewed. Because of the small number of cases reported previously, few characteristics of these tumors are known. The best methods of predicting the malignant and functional potential of these tumors remain unknown. We propose careful preoperative treatment and close postoperative follow-up of paraganglioma patients because of the functional and malignant potential of these tumors.
副神经节瘤是起源于肾上腺外嗜铬细胞的肿瘤。胰腺副神经节瘤罕见，恶性者更少。据我们所知，迄今为止文献中尚无功能性胰腺副神经节瘤的病例报道。我们报告两例经病理证实的胰腺副神经节瘤。病例1中，临床检查和病理分析显示肿瘤具有功能性和恶性特征，预后较差。病例2怀疑为功能性副神经节瘤。总结了这两名患者的临床表现及转归，并复习相关文献。由于既往报道的病例数较少，这些肿瘤的特征鲜为人知。预测这些肿瘤恶性及功能潜能的最佳方法仍不清楚。鉴于这些肿瘤具有功能及恶性潜能，我们建议对副神经节瘤患者进行仔细的术前治疗及密切的术后随访。
