Poster Presentation 5th International Symposium on Phaeochromocytoma and Paraganglioma 2017

A rare case of cardiac paraganglioma with reoperation and long-time follow-up (#86)

Zheng Pei Zeng 1 , Xiao Ping Xing 2 , Shi Chen 2 , Lin Lu 2 , Qi Sun 2 , Qi Miao 3
  1. Peking Union Medical College Hospital, 1, Shuaifuyuan Wangfujing, BEIJING, China
  2. Endocrinology, Peking Union Medical College Hospital, Beijing
  3. Cardiac Surgery, Peking Union Medical College Hospital, Beijing

Objective: Paragangliomas are rare neuroendocrine tumors arising from the ganglia of the sympathetic or parasympathetic nervous system. Here we report a rare case with cardiac paraganglioma successfully treated by reoperations.

Methods: A 53-year-old male with cardiac paraganglioma was admitted to PUMCH in 2017.

Results: He was admitted to the cardiovascular hospital with paroxysmal headache, palpitation, profuse sweating and hypertension in 2003, and the CT scan showed intra-atrial tumor. He had had an operation on his cardiac tumor which grow into left atrium(6*7cm) and right atrium(3*4cm) during cardiopulmonary bypass in 2003 without α-block peraperation. The pathology showed paraganglioma with immunohistochemical staining: CgA(+),Syn(+),Actin(-),Des(-),CD34(-). The blood pressure return nomal after the operation but PET/CT still showed positive mass in left atrium region. Then, He was admitted to PUMCH in December, 2003. The lab examination showed 24h urinary DA 239.68-326.76ug/24h,NE 56.67-100.56ug/24h,E 2.43-5.50ug/24h. CT scan showed a low density mass(3.4*3cm) in left atrium, near inferior vena cava with heterogeneous enhancement after intravenous contrast agent. Octreotide scan revealed positive with MIBG scan negative . From 2003 to 2015, he was followed up in PUMCH. The lab examination and CT scan of the patient were shown in Table1 and Fig1. In 2015, he had had another operation after the prepration of phenoxybenzamine for one month. The lab examination revealed 24h Urinary DA 191.87-228.38 ug/24h,NE 23.71-39.15ug/24h,E 2.76-3.26ug/24h after the operation. His blood pressure and cardiac CT scan remain normal until now.

Conclusions: As a rare neuroendocrine tumor, cardiac paraganglioma often require reoperation to cure, and it is important for a long-time follow-up.

 

Table1: The lab examination of the patient in his follow-up