Surgical removal of retroperitoneal paraganglioma with aortic bifurcation resection and replacement: a case report
https://doi.org/10.21294/1814-4861-2025-24-4-155-161
Abstract
Background. Paragangliomas are rare neuroendocrine tumors that often located paravertebrally in the retroperitoneal space and near major abdominal blood vessels. Surgical resection is the primary treatment for paragangliomas. A close adjacency between a tumor and the main vessels requires a combined treatment approach: a surgical resection of a tumor along with a portion of the vascular trunk, ensuring that the removed tissue is within the healthy surrounding areas. Separation of the tumor from the vessel may potentially leave a microscopic margin of tumor cells on the vessel wall (R1 resection) and usually results in damage to the vessel and significant blood loss.
Case presentation. We present here the case of surgical treatment of paraganglioma of the organ of Zuckerkandl in a 52-year-old female patient. A close adjacency of the tumor to the aortic bifurcation required tumor resection involving the aortic bifurcation and subsequent reconstruction of the main blood flow using a Polytetrafluoroethylene (PtFe) bifurcation aorto-common iliac alloprosthetic graft (PtFe conduit). The postoperative period was uneventful. The patient is stable and is currently under follow-up care.
Conclusion. This case report demonstrates the feasibility of performing radical surgery in patients with retroperitoneal paragangliomas closely adjacent to the major vessels.
About the Authors
R. I. RasulovRussian Federation
Rodion I. Rasulov, MD, DSc, Professor, Head of the Department of Oncology; Deputy Chief Physician
100, Yubileyniy, Irkutsk, 664049
32, Frunze st., Irkutsk, 664035
A. A. Muratov
Russian Federation
Andrey A. Muratov, MD, PhD, Assistant, Department of Oncology; Oncologist, Department of Abdominal Oncology
100, Yubileyniy, Irkutsk, 664049
32, Frunze st., Irkutsk, 664035
A. G. Kuvshinov
Russian Federation
Artem G. Kuvshinov, MD, PhD, Head of the Department of Abdominal Oncology
32, Frunze st., Irkutsk, 664035
A. P. Ponomarenko
Russian Federation
Anna P. Ponomarenko, MD, Anesthesiologist, Anesthesiology and Resuscitation Department № 1
32, Frunze st., Irkutsk, 664035
A. M. Bulytov
Russian Federation
Alexey M. Bulytov, MD, Radiologist, X-ray Diagnostics Department
32, Frunze st., Irkutsk, 664035
D. O. Malykh
Russian Federation
Dmitry O. Malykh, MD, Anesthesiologist-Resuscitator, Anesthesiology and Resuscitation Department № 1
32, Frunze st., Irkutsk, 664035
References
1. Mete O., Asa S.L., Gill A.J., Kimura N., de Krijger R.R., Tischler A. Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas. Endocr Pathol. 2022; 33(1): 90–114. doi: 10.1007/s12022-022-09704-6.
2. Eid M., Foukal J., Sochorová D., Tuček Š., Starý K., Kala Z., Mayer J., Němeček R., Trna J., Kunovský L. Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options. Cancer Med. 2023; 12(13): 13942–57. doi: 10.1002/cam4.6010.
3. Krishnamurthy S.S., Moorthy S., Muralidharan K., Raja A. Surgical Management of Paraganglioma of the Organ of Zuckerkandl with Combined Resection of the Aorta and Inferior Vena Cava with Bifurcation: A Case Report. Vasc Specialist Int. 2025; 41: 3. doi: 10.5758/vsi.240099.
4. Neumann H.P.H., Young W.F. Jr., Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med. 2019; 381(6): 552–65. doi: 10.1056/NEJMra1806651.
5. Baron E., Wu C.C., Gupta K., Wernberg J.A., Sheehan M.T., Sharma R. Robotic Resection in Succinate Dehydrogenase Subunit B (SDHB)- Mutated Hereditary Paraganglioma: A Case Report of Two Patients and A Literature Review. Cureus. 2024; 16(3): e56336. doi: 10.7759/cureus.56336.
6. Bacalbasa N., Balescu I., Tanase A., Brezean I., Vilcu M., Brasoveanu V. Successful Resection of a Non-functional Paraganglioma with Celiac Trunk Invasion Followed by Common Hepatic Artery Reimplantation – A Case Report and Literature Review. In Vivo. 2018; 32(4): 911–14. doi: 10.21873/invivo.11328.
7. Tzikos G., Menni A., Cheva A., Pliakos I., Tsakona A., Apostolidis S., Iakovou I., Michalopoulos A., Papavramidis T. Composite Paraganglioma of the Celiac Trunk: A Case Report and a Comprehensive Review of the Literature. Front Surg. 2022; 9: 824076. doi: 10.3389/fsurg.2022.824076.
8. Hakariya T., Shida Y., Ito H., Ueda Y., Kurata H., Ohtsubo A., Miyata Y., Sakai H., Furusato B. Successful laparoscopic resection of a paraganglioma immediately behind the inferior vena cava and bilateral renal veins. IJU Case Rep. 2019; 2(5): 261–64. doi: 10.1002/iju5.12097.
9. Mannina E.M., Xiong Z., Self R., Kandil E. Resection of a catecholamine-elaborating retroperitoneal paraganglioma invading the inferior vena cava. Case Rep Surg. 2014: 837054. doi: 10.1155/2014/837054.
10. Srirangalingam U., Gunganah K., Carpenter R., Bhattacharya S., Edmondson S.J., Drake W.M. Phaeochromocytoma and Paraganglioma Excision Involving the Great Vessels. EJVES Short Rep. 2017; 35: 1–6. doi: 10.1016/j.ejvssr.2017.02.002.
11. Rasulov R.I., Muratov A.A., Kuvshinov A.G., Songolov G.I. The experience of surgical treatment of leiomyosarcoma of the inferior vena cava and its tributaries. Grekov’s Bulletin of Surgery. 2022; 181(4): 52–60. (in Russian). doi: 10.24884/0042-4625-2022-181-4-52-60. EDN: MGDHSE.
12. Le H.D.T., Nguyen T.T.M.H., Pham A.V., Dang C.T., Nguyen T.T. Pheochromocytoma of the organ of Zuckerkandl. Radiol Case Rep. 2020; 16(2): 268–72. doi: 10.1016/j.radcr.2020.11.024. Erratum in: Radiol Case Rep. 2023; 18(4): 1643–44. doi: 10.1016/j.radcr.2023.01.017.
13. Parisien-La Salle S., Chbat J., Lacroix A., Perrotte P., Karakiewicz P., Saliba I., Le X.K., Olney H.J., Bourdeau I. Postoperative Recurrences in Patients Operated for Pheochromocytomas and Paragangliomas: New Data Supporting Lifelong Surveillance. Cancers (Basel). 2022; 14(12): 2942. doi: 10.3390/cancers14122942.
Supplementary files
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1. Fig. 1. CT image. Non-organ retroperitoneal tumor before surgery, arterial phase, frontal projection. Note: created by the authors | |
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2. Fig. 2. Intraoperative photo. Mobilization of a non-organ retroperitoneal tumor. Abdominal aorta and iliac vessels on silicone tourniquets. Note: created by the authors | |
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3. Fig. 3. Intraoperative photo. View of the abdominal cavity after removal of a non-organ retroperitoneal tumor. Note: created by the authors | |
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4. Fig. 4. Intraoperative photo. View of the surgical wound after vascular reconstruction. Note: created by the authors | |
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5. Fig. 5. Microphoto. Paraganglioma. Hematoxylin and eosin staining, ×400. Note: created by the authors | |
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6. Fig. 6. CT image of abdominal cavity after surgery, arterial phase, frontal projection. Note: created by the authors | |
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Review
For citations:
Rasulov R.I., Muratov A.A., Kuvshinov A.G., Ponomarenko A.P., Bulytov A.M., Malykh D.O. Surgical removal of retroperitoneal paraganglioma with aortic bifurcation resection and replacement: a case report. Siberian journal of oncology. 2025;24(4):155-161. (In Russ.) https://doi.org/10.21294/1814-4861-2025-24-4-155-161