Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor
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...
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Frontiers Media S.A.
2019-10-01
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Series: | Frontiers in Endocrinology |
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Online Access: | https://www.frontiersin.org/article/10.3389/fendo.2019.00713/full |
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Article |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Lin Lu Qingqing Meng Qingqing Meng Xiaoping Xing Tao Yuan Huabing Zhang Naishi Li Yining Wang Yuejuan Cheng Chunmei Bai Hao Wang Xin Cheng Yu Xiao Boju Pan Yuan Li Jian Sun Zhiyong Liang Huijuan Zhu Renzhi Wang Zhaolin Lu |
spellingShingle |
Lin Lu Qingqing Meng Qingqing Meng Xiaoping Xing Tao Yuan Huabing Zhang Naishi Li Yining Wang Yuejuan Cheng Chunmei Bai Hao Wang Xin Cheng Yu Xiao Boju Pan Yuan Li Jian Sun Zhiyong Liang Huijuan Zhu Renzhi Wang Zhaolin Lu Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor Frontiers in Endocrinology Cushing syndrome ACTH syndrome ectopic cardiac neoplasms neuroendocrine tumor chemotherapy |
author_facet |
Lin Lu Qingqing Meng Qingqing Meng Xiaoping Xing Tao Yuan Huabing Zhang Naishi Li Yining Wang Yuejuan Cheng Chunmei Bai Hao Wang Xin Cheng Yu Xiao Boju Pan Yuan Li Jian Sun Zhiyong Liang Huijuan Zhu Renzhi Wang Zhaolin Lu |
author_sort |
Lin Lu |
title |
Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor |
title_short |
Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor |
title_full |
Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor |
title_fullStr |
Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor |
title_full_unstemmed |
Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor |
title_sort |
long-term follow-up of significant improvement after captem treatment for rare adrenocorticotropin-producing cardiac neuroendocrine tumor |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Endocrinology |
issn |
1664-2392 |
publishDate |
2019-10-01 |
description |
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. |
topic |
Cushing syndrome ACTH syndrome ectopic cardiac neoplasms neuroendocrine tumor chemotherapy |
url |
https://www.frontiersin.org/article/10.3389/fendo.2019.00713/full |
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doaj-b4aaff06c24e4f44961cbbe0a937215b2020-11-25T01:32:03ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922019-10-011010.3389/fendo.2019.00713468637Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine TumorLin Lu0Qingqing Meng1Qingqing Meng2Xiaoping Xing3Tao Yuan4Huabing Zhang5Naishi Li6Yining Wang7Yuejuan Cheng8Chunmei Bai9Hao Wang10Xin Cheng11Yu Xiao12Boju Pan13Yuan Li14Jian Sun15Zhiyong Liang16Huijuan Zhu17Renzhi Wang18Zhaolin Lu19Key Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaSchool of Medicine, Tsinghua University, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Radiology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Oncology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Oncology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Nuclear Medicine, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Nuclear Medicine, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaDepartment of Neurosurgery, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaKey Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, ChinaEctopic 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.https://www.frontiersin.org/article/10.3389/fendo.2019.00713/fullCushing syndromeACTH syndromeectopiccardiac neoplasmsneuroendocrine tumorchemotherapy |