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Front. Endocrinol. | doi: 10.3389/fendo.2019.00713

Long-term follow-up of significant improvement after CAPTEM treatment for rare adrenocorticotropin-producing cardiac neuroendocrine tumor

 Lin Lu1, Qingqing Meng1, 2,  Xiaoping Xing1*, Tao Yuan1, Huabing Zhang1, Naishi Li1, Yining Wang3,  Yuejuan Cheng3, Chunmei Bai3*,  Hao Wang3, Xin Cheng3,  Yu Xiao3, Boju Pan3, Yuan Li3, Jian Sun3, Zhiyong Liang3, Huijuan Zhu1,  Renzhi Wang3 and Zhaolin Lu1
  • 1Chinese Academy of Medical Sciences and Peking Union Medical College, China
  • 2Tsinghua University, China
  • 3Peking Union Medical College Hospital (CAMS), China

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a rare cause of Cushing syndrome. If routine imaging examinations cannot identify the source of ACTH production, long-term follow-up observation is necessary to determine the etiology. We present the case of a middle-aged male with gradual weight gain and a Cushingoid appearance over 4 years; he provided written informed consent. Laboratory and endocrine tests strongly suggested EAS, although the origin was not detected by multiple imaging methods. Bilateral adrenalectomy was performed to prevent severe complications in the patient. Two and a half years later, a cardiac mass 18×23×27 mm in size at the junction between the anterior wall of the left ventricle and the middle septum was found together with multiple bone metastases by 18F-FDG PET/CT, while the 68Ga-DOTATE PET/CT findings were negative. Biopsy of the lumbar vertebrae revealed a neuroendocrine tumor (NET) with positive ACTH staining. The patient underwent chemotherapy by CAPTEM, resulting in shrinkage of the cardiac mass and a significant decrease in the ACTH level. In the case of EAS with an unusual cause, long-term follow-up observation is necessary to determine the source of ACTH production. Cardiac NETs are quite rare in EAS, so treatment selection was also challenging. CAPTEM chemotherapy proved effective in controlling the progression of tumor growth and decreasing the ACTH level in this patient.

Keywords: Cushing Syndrome, ACTH Syndrome, Ectopic, Heart Neoplasms, chemotherapy, Neuroendochrine tumor

Received: 29 Apr 2019; Accepted: 02 Oct 2019.

Copyright: © 2019 Lu, Meng, Xing, Yuan, Zhang, Li, Wang, Cheng, Bai, Wang, Cheng, Xiao, Pan, Li, Sun, Liang, Zhu, Wang and Lu. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence:
Mx. Xiaoping Xing, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100006, Beijing Municipality, China,
Mx. Chunmei Bai, Peking Union Medical College Hospital (CAMS), Beijing, China,