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Eruption of Metastatic Paraganglioma After Successful Therapy with 177Lu/90Y-DOTATOC and 177Lu-DOTATATE

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Abstract

Metastatic paraganglioma treatment options are limited. Peptide receptor radionuclide therapy (PRRT) has been introduced as a novel management option for metastatic neuroendocrine tumors demonstrating safety, efficacy, and increased quality of life. We present two cases of marked progression of metastatic paraganglioma following initial partial response to PRRT. Given their positivity on 68Ga-DOTATATE PET/CT and 111In-octreotide SPECT, they underwent PRRT. Imaging following treatment revealed significant improvement in size and intensity, with some foci nearly completely resolved in one patient, and disease regression with a decrease in the number and size of bone and liver lesions in the second patient. Within months, repeat imaging in both patients revealed extensive metastatic disease with new lesions, which eventually lead to their deaths. The mechanism for rapid disease progression after partial response is not well understood, although it could be related to initially high Ki-67 levels or 18F-FDG PET/CT SUVmax values. However, naturally rapid disease progression despite PRRT response cannot be excluded. This finding warrants the importance of proper patient counseling along with early and accurate pre-PRRT assessment, taking into consideration the above potential risk factors for therapy response in order to personalize treatment regimens and achieve maximum patient benefit.

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NCT00004847

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Acknowledgements

We express our sincere gratitude to the patients and their family members for their participation in this study.

Funding

This study was funded by the National Institutes of Health (grant number Z1AHD008735) awarded to Karel Pacak. This work was supported, in part, by the Intramural Research Program of the National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development and was supported, in part, by the Intramural Research Program of the Center for Cancer Research, National Cancer Institute.

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Author notes

  1. Katherine I. Wolf and Abhishek Jha contributed equally to this work.

Authors and Affiliations

  1. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, 20892, USA

    Katherine I. Wolf, Abhishek Jha, Ingo Janssen, Melissa K. Gonzales & Karel Pacak

  2. Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Centre, Nijmegen, The Netherlands

    Anouk van Berkel & Henri J. K. M. Timmers

  3. Clinic of Radiology and Nuclear Medicine, Division of Nuclear Medicine, University Basel Hospital, Basel, Switzerland

    Damian Wild

  4. Positron Emission Tomography Department, National Institutes of Health Clinical Center, National Institutes of Health, 10 Center Dr., Bldg. 10, Rooms 1C-401 and 490, Bethesda, MD, 20892, USA

    Corina M. Millo

  5. Department of Radiology and Nuclear Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

    M. J. R. Janssen

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  1. Katherine I. Wolf
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Correspondence to Karel Pacak.

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Conflict of Interest

Katherine I. Wolf, Abhishek Jha, Anouk van Berkel, Damian Wild, Ingo Janssen, Corina M. Millo, M. J. R. Janssen, Melissa Gonzales, Henri J.K.M. Timmers, and Karel Pacak have no conflict of interest.

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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.

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Wolf, K.I., Jha, A., van Berkel, A. et al. Eruption of Metastatic Paraganglioma After Successful Therapy with 177Lu/90Y-DOTATOC and 177Lu-DOTATATE. Nucl Med Mol Imaging 53, 223–230 (2019). https://doi.org/10.1007/s13139-019-00579-w

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