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

Yıl 2025, Cilt: 9 Sayı: 2, 135 - 141, 31.08.2025
https://doi.org/10.29058/mjwbs.1598103

Öz

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.

Kaynakça

  • 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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

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

Yıl 2025, Cilt: 9 Sayı: 2, 135 - 141, 31.08.2025
https://doi.org/10.29058/mjwbs.1598103

Öz

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.

Etik Beyan

Olgu sunumlarında olur/onam formunun alınmış olup buna ilişkin bilgiye makalede yer verilmiştir.

Kaynakça

  • 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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
Toplam 23 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Endokrinoloji, Kulak Burun Boğaz
Bölüm Derleme
Yazarlar

Deniz Baklacı 0000-0001-8449-4965

Duygu Erdem 0000-0002-7973-2719

Seda Nur Saka 0009-0002-2135-0504

Gökhan Furkan Kılıç 0000-0003-3975-3547

Aleyna Şivetoğlu 0009-0006-7262-181X

Yayımlanma Tarihi 31 Ağustos 2025
Gönderilme Tarihi 8 Aralık 2024
Kabul Tarihi 17 Temmuz 2025
Yayımlandığı Sayı Yıl 2025 Cilt: 9 Sayı: 2

Kaynak Göster

Vancouver Baklacı D, Erdem D, Saka SN, Kılıç GF, Şivetoğlu A. Dopamin Sekrete Eden Baş ve Boyun Paragangliomaları: Literatür İncelemesi ve Olgu Sunumu. Med J West Black Sea. 2025;9(2):135-41.

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