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Favorable outcome in advanced pheochromocytoma and paraganglioma after hypofractionated intensity modulated radiotherapy

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Abstract

Purpose

The purpose of this study was to review outcomes of patients with advanced/metastatic pheochromocytoma/paraganglioma (PPGL) treated at our institution with Intensity-modulated radiotherapy (IMRT), describe the treatment outcomes, and determine predictors.

Methods

A retrospective study on patients with advanced/metastatic PPGL who received IMRT at Peking Union Medical College Hospital between 2014 and 2019. A total of 14 patients with 17 lesions were included in this study. Ultra-hypofractionated radiation therapy was used for 7 lesions in 5 patients, while hypofractionated radiation therapy was used for 8 lesions in 7 patients. 2 patients got conventional fractionated radiotherapy. Patients who received external beam radiation therapy were given a median total radiation dose of 74.4/130 Gy (BED10/3) in a median of 13 fractions.

Results

OS at 2 years was 78% for all patients. For lesions evaluated by RECIST response, at least stable disease of the target lesion was achieved in 94% and distant progression in 28.5%, with an average time to progression of 5.2 months. Patients with locally advanced primary tumors or recurred in situ (n = 8) achieved 100% local control, and none of them got recurrence or distant metastasis after radiotherapy at last follow-up (median 29 months). Of patients with catecholamine-related syndromes (n = 12), 91% of symptomatic lesions improved following radiation therapy and a more than 50% decline in catecholamines.

Conclusions

We have found hypofractionated IMRT effective as an additional therapy for patients with advanced primary tumors or recurrence in situ and not amenable to complete surgical resection.

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Funding

This research was funded by the grant from the Ministry of Science and Technology of China, grant number 2016YFC0105207, and CAMS Innovation Fund for Medical Sciences (CIFMS) 2021-I2M-C&T-B-002, 2017-I2M-1–001.

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Authors and Affiliations

  1. Department of Radiation Oncology, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, Dongdansantiao No. 5, Dongcheng District, Beijing, China

    Z. Gu, K. Hu, Y. Liang, F. Zhang & X. Hou

  2. Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People’s Republic of China, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China

    A. Tong

Authors
  1. Z. Gu
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  2. K. Hu
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  3. Y. Liang
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  4. F. Zhang
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  5. A. Tong
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  6. X. Hou
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Corresponding author

Correspondence to X. Hou.

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Conflict of interest

The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of this review.

Compliance with ethics requirements

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Statement of human and animal rights

All procedures were approved by Peking Union Medical College Hospital Ethics Committee (Approval No. S-K 1886), and the document (scanned version in Chinese) is uploaded to the attachment.

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Informed consent was obtained from all individual participants included in the study.

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Gu, Z., Hu, K., Liang, Y. et al. Favorable outcome in advanced pheochromocytoma and paraganglioma after hypofractionated intensity modulated radiotherapy. J Endocrinol Invest 46, 477–485 (2023). https://doi.org/10.1007/s40618-022-01908-1

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  • DOI: https://doi.org/10.1007/s40618-022-01908-1

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