Dopamine-Secreting Head and Neck Paragangliomas: Literature Review and A Case Report

Abstract

This study aims to review the cases of dopamine-secreting paragangliomas of the head and neck in the literature and to present a patient diagnosed with a jugular and carotid body paraganglioma secreting only dopamine. It is considered that dopamine excess is common in head and neck paragangliomas and dopamine measurement may be useful in the follow-up of patients. Between 1983 and 2024, 16 cases of predominantly dopamine-secreting paragangliomas located in the head and neck region were reviewed in the literature and one case is presented to contribute to the existing knowledge. Hypertension is the most common symptom in dopamine-secreting paragangliomas of the head and neck, followed by hearing loss, dysphagia, dysphonia and tinnitus. The median age was 46.3 years and the male to female ratio was 1.6:1. Treatment modalities included surgery in 10 cases, radiotherapy in 2 cases, conservative treatment in 1 case and no treatment record was available in 3 cases. In our case, the patient presented with hypertension, hearing loss, dysphonia, tinnitus and facial paralysis and was treated with radiotherapy. Dopamine-secreting head and neck paragangliomas are usually symptomatic due to compression, and there may be various morbidities associated with the disease at the time of diagnosis. Dopamine measurements may be useful for the diagnosis and follow-up of these tumors.

Keywords

• Head and neck
• dopamine
• paraganglioma

Dopamin Sekrete Eden Baş ve Boyun Paragangliomaları: Literatür İncelemesi ve Olgu Sunumu

Abstract

Bu çalışmanın amacı, literatürde baş boyunda dopamin salgılayan paraganglioma olgularını incelemek ve yalnızca dopamin salgılayan juguler ve karotis cisim paraganglioma tanılı bir hastayı sunmaktır. Dopamin fazlalığının baş boyun paragangliomalarında sık görüldüğü ve dopamin ölçümünün hastaların takibinde faydalı olabileceği düşünülmektedir 1983-2024 yılları arasında literatürde baş-boyun bölgesinde yer alan ve ağırlıklı olarak dopamin salgılayan 16 paraganglioma olgusu incelenmiş ve bir olgu sunularak literatüre katkıda bulunulmuştur. Baş boyunda dopamin salgılayan paragangliomalarda en sık görülen semptom hipertansiyon olarak bulunmuş; bunu işitme kaybı, disfaji, disfoni, tinnitus izlemiştir. Olguların medyan yaşı 46,3 olup, erkek-kadın oranı 1,6:1’dir. Tedavi yöntemleri arasında 10 olguda cerrahi, 2 olguda radyoterapi, 1 olguda konservatif tedavi uygulanmış, 3 olguda tedavi kayıtlarına ulaşılamamıştır. Olgumuzda da hipertansiyon, işitme kaybı, disfoni, tinnitus ve fasiyal paralizi mevcuttu ve radyoterapi tedavisi aldı. Dopamin salgılayan baş boyun paragangliomaları genellikle basıya bağlı semptom verir, tanı anında hastalığa bağlı çeşitli morbiditeler olabilir. Dopamin ölçümleri, bu tümörlerin tanı ve takibi için faydalı olabilir.

Keywords

• Baş ve boyun
• dopamin
• paraganglioma

References

1. 1. Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med. 2019;381(6):552–565. doi:10.1056/NEJMra1806651
2. 2. Mustafa K, Zadeh S, Culver SA. Dopamine-Secreting Carotid Body Paraganglioma in a Patient With SDHB Mutation. AACE Clin Case Rep. 2024;10(3):109–112. doi:10.1016/j. aace.2024.03.003
3. 3. Strauss M, Nicholas GG, Abt AB, Harrison TS, Seaton JF. Malignant catecholamine-secreting carotid body paraganglioma. Otolaryngol Head Neck Surg. 1983;91(3):315–321. doi:10.1177/019459988309100323
4. 4. Schwaber MK, Glasscock ME, Nissen AJ, Jackson CG, Smith PG. Diagnosis and management of catecholamine secreting glomus tumors. Laryngoscope. 1984;94(8):1008–1015. doi:10.1288/00005537-198408000-00002
5. 5. Azzarelli B, Felten S, Muller J, Miyamoto R, Purvin V. Dopamine in paragangliomas of the glomus jugulare. Laryngoscope. 1988;98(5):573–578. doi:10.1288/00005537-198805000- 00020
6. 6. Troughton RW, Fry D, Allison RS, Nicholls MG. Depression, palpitations, and unilateral pulsatile tinnitus due to a dopamine-secreting glomus jugulare tumor. Am J Med. 1998;104(3):310–311. doi:10.1016/s0002-9343(98)00019-9
7. 7. Levin RJ, Hamill NJ, Grenko RT, Huang MY, Fedok FG. Dopamine- secreting glomus vagale: a case report and histopathologic correlation. Head Neck. 1998;20(8):753–757. doi:10.1002/ (sici)1097-0347(199812)20:8<753::aid-hed15>3.0.co;2-v
8. 8. Hirano S, Shoji K, Kojima H, Omori K. Dopamine-secreting carotid body tumor. Am J Otolaryngol. 1998;19(6):412–416. doi:10.1016/s0196-0709(98)90048-5

9. 9. Koch CA, Rodbard JS, Brouwers FM, Eisenhofer G, Pacak K. Hypotension in a woman with a metastatic dopamine-secreting carotid body tumor. Endocr Pract. 2003;9(4):310–314. doi:10.4158/EP.9.4.310
10. 10. Eisenhofer G, Goldstein DS, Sullivan P, Izbicki G, Martiniova L, Hiroi M, Miesel T, Adams KT, Pacak K. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 2005;90(4):2068–2075. doi:10.1210/jc.2004-2025
11. 11. Jeffery, J., Devendra, D., Farrugia, J., Gardner, D., Murphy, M. J., Williams, R., Ayling, R. M., & Wilkin, T. J. Increased urinary dopamine excretion in association with bilateral carotid body tumours: clinical, biochemical and genetic findings. Ann Clin Biochem. 2006;43(Pt 2):156–160. doi:10.1258/000456306776021517
12. 12. Benarroch EE, McKeon A. Recurrent syncope as a manifestation of a dopamine-secreting glomus vagale tumor. Neurology. 2011;76(11):1021–1022. doi:10.1212/WNL. 0b013e31821043e3
13. 13. Soh AW, Kek PC. Dopamine-secreting carotid body paragangliomas - biochemical control with radiotherapy. Intern Med. 2012;51(6):613–618. doi:10.2169/internalmedicine.51.6324
14. 14. Tuleasca, C., Jaquet, Y., Schweizer, V., Negretti, L., Magaddino, V., Maeder, P., Abid, K. A., Lhermitte, B., Grouzmann, E., & Levivier, M. Clinical and biochemical responses after Gamma Knife surgery for a dopamine-secreting paraganglioma: case report. Hormones (Athens). 2016;15(1):106–112. doi:10.14310/horm.2002.1605
15. 15. Abdul Sater, Z., Jha, A., Mandl, A., Mangelen, S. K., Carrasquillo, J. A., Ling, A., Gonzales, M. K., Lopes Abath Neto, O., Miettinen, M., Adams, K. T., Nockel, P., El Lakis, M., & Pacak, K. Gallbladder Paraganglioma Associated with SDHD Mutation: a Potential Pitfall on 18F-FDOPA PET Imaging. Nucl Med Mol Imaging. 2019;53(2):144–147. doi:10.1007/s13139-018- 0558-1
16. 16. Hussein Z, Korbonits M, Baldeweg SE, Chung TT. Cabergoline reduces 3-methoxytyramine in a SDHC patient with metastatic paraganglioma and prolactinoma. Endocrinol Diabetes Metab Case Rep. 2021;2021:21-0003. doi:10.1530/EDM-21-0003
17. 17. Sandow L, Thawani R, Kim MS, Heinrich MC. Paraganglioma of the Head and Neck: A Review. Endocr Pract. 2023;29(2):141– 147. doi:10.1016/j.eprac.2022.10.002
18. 18. Hickman PE, Leong M, Chang J, Wilson SR, McWhinney B. Plasma free metanephrines are superior to urine and plasma catecholamines and urine catecholamine metabolites for the investigation of pheochromocytoma. Pathology. 2009;41(2):173– 177. doi:10.1080/00313020802579284
19. 19. E Erickson, D., Kudva, Y. C., Ebersold, M. J., Thompson, G. B., Grant, C. S., van Heerden, J. A., & Young, W. F., Jr. Benign Paragangliomas: Clinical Presentation and Treatment Outcomes in 236 Patients. J Clin Endocrinol Metab. 2001;86(11):5210– 5216. doi:10.1210/jcem.86.11.8034
20. 20. Vimawala, S. N., Graboyes, A. Z., Bennett, B., Bonanni, M., Abbasi, A., Oliphant, T., Alonso-Basanta, M., Rassekh, C., Cohen, D., Brant, J. A., & Huan, Y. Head and Neck Paragangliomas: Overview of Institutional Experience. Cancers (Basel). 2024;16(8):1523. doi:10.3390/cancers16081523
21. 21. Miyamoto, S., Yoshida, Y., Ozeki, Y., Okamoto, M., Gotoh, K., Masaki, T., Nishida, H., Shibuya, T., Shin, T., Daa, T., Mimata, H., Kimura, N., & Shibata, H. Dopamine-Secreting Pheochromocytoma and Paraganglioma. J Endocr Soc. 2021;5(12):bvab163. doi:10.1210/jendso/bvab163
22. 22. Van Der Horst-Schrivers AN, Osinga TE, Kema IP, Van Der Laan BF, Dullaart RP. Dopamine excess in patients with head and neck paragangliomas. Anticancer Res. 2010;30(12):5153– 5158.
23. 23. Rao, D., Peitzsch, M., Prejbisz, A., Hanus, K., Fassnacht, M., Beuschlein, F., Brugger, C., Fliedner, S., Langton, K., Pamporaki, C., Gudziol, V., Stell, A., Januszewicz, A., Timmers, H. J. L. M., Lenders, J. W. M., & Eisenhofer, G. Plasma methoxytyramine: clinical utility with metanephrines for diagnosis of pheochromocytoma and paraganglioma. Eur J Endocrinol. 2017;177(2):103–113. doi:10.1530/EJE-17-0077