The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

Kolomeytseva A.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

Beltsevich D.G.

National Medical Research Center of Endocrinology

Gurevich L.E.

M.F. Vladimirsky Moscow Regional Research and Clinical Institute (MONIKI)

Roslyakova A.A.

National Medical Research Center of Endocrinology of the Ministry of Health of Russia

Sedova M.V.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

Batov M.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

Fedenko A.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

Treatment of metastatic pheochromocytoma and paraganglioma

Authors:

Kolomeytseva A.A., Beltsevich D.G., Gurevich L.E., Roslyakova A.A., Sedova M.V., Batov M.A., Fedenko A.A.

More about the authors

Journal: P.A. Herzen Journal of Oncology. 2024;13(1): 70‑75

Read: 1482 times


To cite this article:

Kolomeytseva AA, Beltsevich DG, Gurevich LE, Roslyakova AA, Sedova MV, Batov MA, Fedenko AA. Treatment of metastatic pheochromocytoma and paraganglioma. P.A. Herzen Journal of Oncology. 2024;13(1):70‑75. (In Russ.)
https://doi.org/10.17116/onkolog20241301170

Recommended articles:
Difficulties of diagnosing a clinically silent pheochromocytoma. Russian Journal of Preventive Medi­cine. 2025;(3):107-111

References:

  1. Fassnacht M, Assie G, Baudin E, Eisenhofer G, de la Fouchardiere C, Haak HR, de Krijger R, Porpiglia F, Terzolo M, Berruti A; ESMO Guidelines Committee. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020;31(11):1476-1490. https://doi.org/10.1016/j.annonc.2020.08.2099
  2. Lam AK. Update on adrenal tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. Endocr Pathol. 2017;28(3):213-227.  https://doi.org/10.1007/s12022-017-9484-5
  3. Kimura N, Capella C, DeLellis RA, Epstein JI, Gill A, Kawashima A. Extra-adrenal paragangliomas. In: Lloyd RV, Osamura RY, Kloppel G, Rosai J, eds. WHO classification of tumours of endocrine organs. 4th ed. Vol. 10. Lyon: IARC; 2017;190-195. 
  4. Tischler AS, de Krijger RR, Gill A, et al. Phaeochromocytoma. In: Lloyd RV, Osamura RY, Kloppel G, Rosai J, eds. WHO classification of tumours of endocrine organs. 4th ed. Vol. 10. IARC, Lyon; 2017;183-189. 
  5. WHO Classification of Tumours Editorial Board. Endocrine and neuroendocrine tumours. Lyon: International Agency for Research on Cancer; 2022. https://tumourclassification.iarc.who.int/chapters/36
  6. Assadipour Y, Sadowski SM, Alimchandani M, Quezado M, Steinberg SM, Nilubol N, Patel D, Prodanov T, Pacak K, Kebebew E. SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma. Surgery. 2017;161(1):230-239.  https://doi.org/10.1016/j.surg.2016.05.050
  7. Papathomas TG, Oudijk L, Persu A, Gill AJ, van Nederveen F, Tischler AS, Tissier F, Volante M, Matias-Guiu X, Smid M, et al. SDHB/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a multinational study of the European Network for the Study of Adrenal Tumors (ENS@T). Mod Pathol. 2015;28(6):807-821.  https://doi.org/10.1038/modpathol.2015.41
  8. Rebrova DV, Vorokhobina NV, Imyanitov EN, Rusakov VF, Krasnov LM, Sleptsov IV, Chernikov RA, Fedorov EA, Semenov AA, Chinchuk IK, Sablin IV, Alekseev MA, Kuleshov OV, Fedotov YuN. Clinical and laboratory features of hereditary pheochromocytoma and paraganglioma. Problemy Endokrinologii. 2022;68(1):8-17. (In Russ.). https://doi.org/10.14341/probl12834
  9. Ayala-Ramirez M, Feng L, Johnson MM, Ejaz S, Habra MA, Rich T, Busaidy N, Cote GJ, Perrier N, Phan A, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab. 2011;96(3):717-725.  https://doi.org/10.1210/jc.2010-1946
  10. Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, Kimura N, Mannelli M, McNicol AM, Tischler AS; International Symposium on Pheochromocytoma. Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab. 2007;3(2):92-102.  https://doi.org/10.1038/ncpendmet0396
  11. Mel’nichenko GA, Troshina EA, Bel’tsevich DG, Kuznetsov NS, Yukina MYu. Russian Association of Endocrinologists clinical practice guidelines for diagnosis and treatment of pheochromocytoma and paraganglioma. Endokrinnaya Khirugiya. 2015;9(3):15-33. (In Russ.). https://doi.org/10.14341/serg2015315-33
  12. Hescot S, Leboulleux S, Amar L, Vezzosi D, Borget I, Bournaud-Salinas C, de la Fouchardiere C, Libé R, Do Cao C, Niccoli P, et al.; French group of Endocrine and Adrenal tumors (Groupe des Tumeurs Endocrines-REseau NAtional des Tumeurs ENdocrines and COrtico-MEdullo Tumeurs Endocrines networks). One-year progression-free survival of therapy-naive patients with malignant pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 2013;98(10):4006-4012. https://doi.org/10.1210/jc.2013-1907
  13. Noda T, Nagano H, Miyamoto A, Wada H, Murakami M, Kobayashi S, Marubashi S, Takeda Y, Dono K, Umeshita K, et al. Successful outcome after resection of liver metastasis arising from an extraadrenal retroperitoneal paraganglioma that appeared 9 years after surgical excision of the primary lesion. Int J Clin Oncol. 2009;14(5):473-477.  https://doi.org/10.1007/s10147-008-0872-1
  14. Strajina V, Dy BM, Farley DR, Richards ML, McKenzie TJ, Bible KC, Que FG, Nagorney DM, Young WF, Thompson GB. Surgical treatment of malignant pheochromocytoma and paraganglioma: retrospective case series. Ann Surg Oncol. 2017;24(6):1546-1550. https://doi.org/10.1245/s10434-016-5739-5
  15. Breen W, Bancos I, Young WF Jr, Bible KC, Laack NN, Foote RL, Hallemeier CL. External beam radiation therapy for advanced/unresectable malignant paraganglioma and pheochromocytoma. Adv Radiat Oncol. 2017;3(1):25-29.  https://doi.org/10.1016/j.adro.2017.11.002
  16. Teno S, Tanabe A, Nomura K, Demura H. Acutely exacerbated hypertension and increased inflammatory signs due to radiation treatment for metastatic pheochromocytoma. Endocr J. 1996;43(5):511-516.  https://doi.org/10.1507/endocrj.43.511
  17. van der Harst E, de Herder WW, Bruining HA, Bonjer HJ, de Krijger RR, Lamberts SW, van de Meiracker AH, Boomsma F, Stijnen T, Krenning EP, et al. [(123)I]metaiodobenzylguanidine and [(111)In]octreotide uptake in benign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86(2):685-693.  https://doi.org/10.1210/jcem.86.2.7238
  18. Noto RB, Pryma DA, Jensen J, Lin T, Stambler N, Strack T, Wong V, Goldsmith SJ. Phase 1 study of high-specific-activity I-131 MIBG for metastatic and/or recurrent pheochromocytoma or paraganglioma. J Clin Endocrinol Metab. 2018;103(1):213-220.  https://doi.org/10.1210/jc.2017-02030
  19. Loh KC, Fitzgerald PA, Matthay KK, Yeo PP, Price DC. The treatment of malignant pheochromocytoma with iodine-131 metaiodobenzylguanidine (131I-MIBG): a comprehensive review of 116 reported patients. J Endocrinol Invest. 1997;20(11):648-658.  https://doi.org/10.1007/BF03348026
  20. Mukherjee JJ, Kaltsas GA, Islam N, Plowman PN, Foley R, Hikmat J, Britton KE, Jenkins PJ, Chew SL, Monson JP, et al. Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with (131)I-meta-iodobenzylguanidine [(131)I-mIBG]. Clin Endocrinol (Oxford). 2001;55(1):47-60.  https://doi.org/10.1046/j.1365-2265.2001.01309.x
  21. Pryma DA, Chin BB, Noto RB, Dillon JS, Perkins S, Solnes L, Kostakoglu L, Serafini AN, Pampaloni MH, Jensen J, et al. Efficacy and safety of high-specific-activity 131I-MIBG therapy in patients with advanced pheochromocytoma or paraganglioma. J Nucl Med. 2019;60(5):623-630.  https://doi.org/10.2967/jnumed.118.217463
  22. Gedik GK, Hoefnagel CA, Bais E, Olmos RA. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2008;35(4):725-733.  https://doi.org/10.1007/s00259-007-0652-6
  23. Rachh SH, Abhyankar S, Basu S. [¹³¹I] Metaiodobenzylguanidine therapy in neural crest tumors: varying outcome in different histopathologies. Nucl Med Commun. 2011;32(12):1201-1210. https://doi.org/10.1097/MNM.0b013e32834bad97
  24. Shilkrut M, Bar-Deroma R, Bar-Sela G, Berniger A, Kuten A. Low-dose iodine-131 metaiodobenzylguanidine therapy for patients with malignant pheochromocytoma and paraganglioma: single center experience. Am J Clin Oncol. 2010;33(1):79-82.  https://doi.org/10.1097/COC.0b013e31819e2c28
  25. Chen H, Sippel RS, O’Dorisio MS, Vinik AI, Lloyd RV, Pacak K; North American Neuroendocrine Tumor Society (NANETS). The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39(6):775-783.  https://doi.org/10.1097/MPA.0b013e3181ebb4f0
  26. Hubalewska-Dydejczyk A, Trofimiuk M, Sowa-Staszczak A, Gilis-Januszewska A, Wierzchowski W, Pach D, Budzyński A, Karcz D. Ekspresja receptorów somatostatynowych (SSTR1-sSTR5) w guzach chromochłonnych [Somatostatin receptors expression (SSTR1-SSTR5) in pheochromocytomas]. Przegl Lek. 2008;65(9):405-407. (In Polish).
  27. Saveanu A, Muresan M, De Micco C, Taieb D, Germanetti AL, Sebag F, Henry JF, Brunaud L, Enjalbert A, Weryha G, et al. Expression of somatostatin receptors, dopamine D₂ receptors, noradrenaline transporters, and vesicular monoamine transporters in 52 pheochromocytomas and paragangliomas. Endocr Relat Cancer. 2011;18(2):287-300.  https://doi.org/10.1530/ERC-10-0175
  28. de Herder WW, Hofland LJ. Somatostatin receptors in pheochromocytoma. Front Horm Res. 2004;31:145-154. 
  29. Buchmann I, Henze M, Engelbrecht S, Eisenhut M, Runz A, Schäfer M, Schilling T, Haufe S, Herrmann T, Haberkorn U. Comparison of 68Ga-DOTATOC PET and 111In-DTPAOC (Octreoscan) SPECT in patients with neuroendocrine tumours. Eur J Nucl Med Mol Imaging. 2007;34(10):1617-1626. https://doi.org/10.1007/s00259-007-0450-1
  30. Kowalski J, Henze M, Schuhmacher J, Mäcke HR, Hofmann M, Haberkorn U. Evaluation of positron emission tomography imaging using [68Ga]-DOTA-D Phe(1)-Tyr(3)-Octreotide in comparison to [111In]-DTPAOC SPECT. First results in patients with neuroendocrine tumors. Mol Imaging Biol. 2003;5(1):42-48.  https://doi.org/10.1016/s1536-1632(03)00038-6
  31. Zandee WT, Feelders RA, Smit Duijzentkunst DA, Hofland J, Metselaar RM, Oldenburg RA, van Linge A, Kam BLR, Teunissen JJM, Korpershoek E, et al. Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy using 177Lu-DOTATATE. Eur J Endocrinol. 2019;181(1):45-53.  https://doi.org/10.1530/EJE-18-0901
  32. Tenenbaum F, Schlumberger M, Lumbroso J, Parmentier C. Beneficial effects of octreotide in a patient with a metastatic paraganglioma. Eur J Cancer. 1996;32A(4):737.  https://doi.org/10.1016/0959-8049(95)00617-6
  33. Kau R, Arnold W. Somatostatin receptor scintigraphy and therapy of neuroendocrine (APUD) tumors of the head and neck. Acta Otolaryngol. 1996;116(2):345-349.  https://doi.org/10.3109/00016489609137855
  34. Koriyama N, Kakei M, Yaekura K, Okui H, Yamashita T, Nishimura H, Matsushita S, Tei C. Control of catecholamine release and blood pressure with octreotide in a patient with pheochromocytoma: a case report with in vitro studies. Horm Res. 2000;53(1):46-50.  https://doi.org/10.1159/000023513
  35. Lamarre-Cliche M, Gimenez-Roqueplo AP, Billaud E, Baudin E, Luton JP, Plouin PF. Effects of slow-release octreotide on urinary metanephrine excretion and plasma chromogranin A and catecholamine levels in patients with malignant or recurrent phaeochromocytoma. Clin Endocrinol (Oxford). 2002;57(5):629-634.  https://doi.org/10.1046/j.1365-2265.2002.01658.x
  36. NCCN Clinical Practice Guidelines in Oncology. Neuroendocrine and adrenal tumors. Version 2.2022. 2022.
  37. LACNETS. A Neuroendocrine Tumor Society. LAMPARA: lanreotide in metastatic pheochromocytoma and PARAganglioma. https://clinicaltrials.gov/show/NCT03946527
  38. Huang H, Abraham J, Hung E, Averbuch S, Merino M, Steinberg SM, Pacak K, Fojo T. Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer. 2008;113(8):2020-2028. https://doi.org/10.1002/cncr.23812
  39. Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Déandreis D, Borget I, Loriot C, Chougnet C, et al. SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Int J Cancer. 2014;135(11):2711-2720. https://doi.org/10.1002/ijc.28913
  40. Nozières C, Walter T, Joly MO, Giraud S, Scoazec JY, Borson-Chazot F, Simon C, Riou JP, Lombard-Bohas C. A SDHB malignant paraganglioma with dramatic response to temozolomide-capecitabine. Eur J Endocrinol. 2012;166(6):1107-1111. https://doi.org/10.1530/EJE-11-1098
  41. Tong A, Li M, Cui Y, Ma X, Wang H, Li Y. Temozolomide is a potential therapeutic tool for patients with metastatic pheochromocytoma/paraganglioma — case report and review of the literature. Front Endocrinol (Lausanne). 2020;11:61.  https://doi.org/10.3389/fendo.2020.00061
  42. Mora J, Cruz O, Parareda A, Sola T, de Torres C. Treatment of disseminated paraganglioma with gemcitabine and docetaxel. Pediatr Blood Cancer. 2009;53(4):663-665.  https://doi.org/10.1002/pbc.22006
  43. Felsberg J, Rapp M, Loeser S, Fimmers R, Stummer W, Goeppert M, Steiger HJ, Friedensdorf B, Reifenberger G, Sabel MC. Prognostic significance of molecular markers and extent of resection in primary glioblastoma patients. Clin Cancer Res. 2009;15(21):6683-6693. https://doi.org/10.1158/1078-0432.CCR-08-2801
  44. Quillien V, Lavenu A, Sanson M, Legrain M, Dubus P, Karayan-Tapon L, Mosser J, Ichimura K, Figarella-Branger D. Outcome-based determination of optimal pyrosequencing assay for MGMT methylation detection in glioblastoma patients. J Neurooncol. 2014;116(3):487-496.  https://doi.org/10.1007/s11060-013-1332-y
  45. O’Kane GM, Ezzat S, Joshua AM, Bourdeau I, Leibowitz-Amit R, Olney HJ, Krzyzanowska M, Reuther D, Chin S, Wang L, et al. A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: the SNIPP trial. Br J Cancer. 2019;120(12):1113-1119. https://doi.org/10.1038/s41416-019-0474-x
  46. Baudin E, Goichot B, Berruti A, Hadoux J, Moalla S, Laboureau S, Noelting S, de la Fouchardière C, Kienitz T, Deutschbein T, et al. 567O First International Randomized Study in Malignant Progressive Pheochromocytoma and Paragangliomas (FIRSTMAPPP): an academic double-blind trial investigating sunitinib. Ann Oncol. 2021;32:S621. https://doi.org/10.1016/j.annonc.2021.08.702

Email Confirmation

An email was sent to test@gmail.com with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.