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Role of 68Ga-DOTATATE PET/CT in a Case of SDHB-Related Pterygopalatine Fossa Paraganglioma Successfully Controlled with Octreotide

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Abstract

We describe an unusual case of a 42-year-old female with an unresectable succinate dehydrogenase subunit B (SDHB)-related pterygopalatine fossa paraganglioma. She underwent somatostatin receptor imaging with 68Ga-DOTA(0)-Tyr(3)-octreotate (68Ga-DOTATATE) positron emission tomography/computed tomography (PET/CT), which showed uptake in the above mentioned tumor. Hence, the patient was started on octreotide, a cold somatostatin analog, and responded with tumor stabilization and improvement of clinical symptoms for 36 months since initiation of octreotide therapy. This case demonstrates the role of 68Ga-DOTATATE PET/CT in diagnostic localization and its subsequent role in treatment using cold somatostatin analog as a potential choice of therapy in the management of paraganglioma in an unusual location with limited therapeutic options.

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References

  1. Martin TP, Irving RM, Maher ER. The genetics of paragangliomas: a review. Clin Otolaryngol. 2007;32:7–11.

    Article  CAS  Google Scholar 

  2. TaïebD KA, Boedeker CC, Martucci V, Fojo T, Adler JR Jr, et al. Current approaches and recent developments in the management of head and neck paragangliomas. Endocr Rev. 2014;35:795–819.

    Article  CAS  Google Scholar 

  3. Sykes JM, Ossoff RH. Paragangliomas of the head and neck. Otolaryngol Clin N Am. 1986;19:755–67.

    CAS  Google Scholar 

  4. Janssen I, Chen CC, Taieb D, Patronas NJ, Millo CM, Adams KT, et al. 68Ga-DOTATATE PET/CT in the localization of head and neck Paragangliomas compared with other functional imaging modalities and CT/MRI. J Nucl Med. 2016;57:186–91.

    Article  CAS  Google Scholar 

  5. Erickson D, Kudva YC, Ebersold MJ, Thomson GB, Grant CS, van Heerden JA, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001;86:5210–6.

    Article  CAS  Google Scholar 

  6. Capitana C, Ntali G, Karavitaki N, Grossman AB. The management of head-and-neck paragangliomas. Endocr Relat Cancer. 2013;20:R291–305.

    Article  Google Scholar 

  7. McCrary HC, Babajanian E, Calquin M, Carpenter P, Casazza G, Naumer A, et al. Characterization of malignant head and neck Paragangliomas at a single institution across multiple decades. JAMA Otolaryngol Head Neck Surg. 2019;145:641–6.

    Article  Google Scholar 

  8. Jha A, Nilubol N, and Pacak K. Some Considerations in Treating Malignant Head and Neck Paragangliomas at a Single Institution Across Multiple Decades. JAMA Otolaryngol Head Neck Surg. 2019 Nov 14. doi: 10.1001/jamaoto.2019.3444. [Epub ahead of print]

    Article  Google Scholar 

  9. Reubi JC, Waser B, Schaer JC, Laissue JA. Somatostatin receptor sst1-sst5 expression in normal and neoplastic human tissues using receptor autoradiography with subtype-selective ligands. Eur J Nucl Med. 2001;28:836–46.

    Article  CAS  Google Scholar 

  10. Reubi JC, Schar JC, Waser B, Wenger S, Heppeler A, Schmitt JS, et al. Affinity profiles for human somatostatin receptor subtypes SST1-SST5 of somatostatin radiotracers selected for scintigraphic and radiotherapeutic use. Eur J Nucl Med. 2000;27:273–82.

    Article  CAS  Google Scholar 

  11. Han S, Suh CH, Woo S, Kim YJ, Lee JJ. Performance of 68Ga-DOTA-conjugated Somatostatin receptor targeting peptide PET in detection of pheochromocytoma and Paraganglioma: a systematic review and meta-analysis. J Nucl Med. 2019;60:369–76.

    Article  CAS  Google Scholar 

  12. Janssen I, Blanchet EM, Adams K, Chen CC, Millo CM, Herscovitch P, et al. Superiority of [68Ga]-DOTATATE PET/CT to other functional imaging modalities in the localization of SDHB-associated metastatic Pheochromocytoma and Paraganglioma. Clin Cancer Res. 2015;21:3888–95.

    Article  CAS  Google Scholar 

  13. Janssen I, Chen CC, Millo CM, Ling A, Taieb D, Lin FL, et al. PET/CT comparing (68)Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2016;43:1784–91.

    Article  CAS  Google Scholar 

  14. Jha A, Ling A, Millo C, Gupta G, Viana B, Lin FI, et al. Superiority of (68)Ga-DOTATATE over (18)F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx)-related pheochromocytoma and paraganglioma in the pediatric population. Eur J Nucl Med Mol Imaging. 2018;45:787–97.

    Article  CAS  Google Scholar 

  15. Jha A, de Luna K, Balili CA, Millo C, Paraiso CA, Ling A, et al. Clinical, diagnostic, and treatment characteristics of SDHA-related metastatic Pheochromocytoma and Paraganglioma. Front Oncol. 2019;9:53.

    Article  Google Scholar 

  16. Jha A, Ling A, Millo C, Chen C, Gupta G, Viana B, et al. Superiority of 68Ga-DOTATATE PET/CT to other functional and anatomic imaging modalities in the detection of SDHD-related pheochromocytoma and paraganglioma – A comparative prospective study. J Nucl Med. May 1, 2018 vol. 59 no. supplement 1 46

  17. Taieb D, Hicks RJ, Hindie E, Guillet BA, Avrram A, Ghedini P, et al. European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2019;46:2112–37.

    Article  Google Scholar 

  18. *Taïeb D, *Jha A, Treglia G, and Pacak K. Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma. Endocr Relat Cancer. 2019;27:R627–R652.

  19. Puranik AD, Kulkarni HR, Singh A, et al. Peptide receptor radionuclide therapy with 90Y/177Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours). Eur J Nucl Med Mol Imaging. 2015;42:1223–30.

    Article  CAS  Google Scholar 

  20. Tenenbaum F, Schlumberger M, Lumbroso J, Parmentier C. Beneficial effects of octreotide in a patient with a metastatic paraganglioma. Eur J Cancer. 1996;32A:737.

    Article  CAS  Google Scholar 

  21. Tonyukuk V, Emral R, Temizkan S, Sertcelik A, Erden I, Corapcioglu D. Case report: patient with multiple paragangliomas treated with long acting somatostatin analogue. Endocr J. 2003;50:507–13.

    Article  Google Scholar 

  22. Duet M, Guichard JP, Rizzo N, Boudiaf M, Herman P. Tran Ba Huy P. are somatostatin analogs therapeutic alternatives in the management of head and neck paragangliomas? Laryngoscope. 2005;115:1381–4.

    Article  CAS  Google Scholar 

  23. van Hulsteijn LT, van Duinen N, Verbist BM, Jansen JC, van der Klaauw AA, Smit JW, et al. Effects of octreotide therapy in progressive head and neck paragangliomas: case series. Head Neck. 2013;35:E391–6.

    Article  Google Scholar 

  24. Rinke A, Muller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, et al. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID study group. J Clin Oncol. 2009;27(28):4656–63.

    Article  CAS  Google Scholar 

  25. Caplin ME, Pavel M, Cwikla JB, Phan AT, Raderer M, Sedlackova E, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371(3):224–33.

    Article  CAS  Google Scholar 

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Acknowledgments

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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Authors and Affiliations

  1. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC, 1E-3140, 10 Center Drive, MSC-1109, Bethesda, MD, 20892-1109, USA

    Abhishek Jha, Mayank Patel, Melissa K. Gonzales, Marianne Knue & Karel Pacak

  2. Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, 20892, USA

    Eva Baker & Alexander Ling

  3. Positron Emission Tomography Department, Radiology and Imaging Sciences, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, 20892, USA

    Corina Millo

  4. Division of Nuclear Medicine and Molecular Imaging, Department of Radiology and Radiological Sciences, Johns Hopkins Medicine, Baltimore, MD, USA

    Ali Cahid Civelek

Authors
  1. Abhishek Jha
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  2. Mayank Patel
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  3. Eva Baker
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  4. Melissa K. Gonzales
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  5. Alexander Ling
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  6. Corina Millo
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  7. Marianne Knue
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  8. Ali Cahid Civelek
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  9. Karel Pacak
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Corresponding author

Correspondence to Karel Pacak.

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Conflict of Interest

Abhishek Jha, Mayank Patel, Eva Baker, Melissa K. Gonzales, Alexander Ling, Corina Millo, Marianne Knue, Ali Cahid Civelek, and Karel Pacak declares 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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Informed consent was obtained from the participant included in the study.

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Jha, A., Patel, M., Baker, E. et al. Role of 68Ga-DOTATATE PET/CT in a Case of SDHB-Related Pterygopalatine Fossa Paraganglioma Successfully Controlled with Octreotide. Nucl Med Mol Imaging 54, 48–52 (2020). https://doi.org/10.1007/s13139-019-00629-3

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  • DOI: https://doi.org/10.1007/s13139-019-00629-3

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