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Krishnamurthy SS, 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. [PMID: 40033739 DOI: 10.5758/vsi.240099] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2024] [Revised: 12/29/2024] [Accepted: 12/31/2024] [Indexed: 03/05/2025] Open
Abstract
Paragangliomas are rare neuroendocrine tumors that frequently arise in the organ of Zuckerkandl (OZ). Surgical resection remains the cornerstone of treatment. Although vascular resection and reconstruction have been reported in a few cases, no prior reports have documented the simultaneous resection of both the aorta and inferior vena cava (IVC), along with their bifurcation. We present the case of a 50-year-old man with a large paraganglioma of the OZ, successfully managed through combined resection of the aorta, IVC, their bifurcation, and the psoas muscle. Dacron Y-grafts were used to reconstruct both the aorta and IVC. This case underscores the importance of detailed anatomical knowledge of major vessels, meticulous vascular control, and the technical intricacies of vascular reconstruction. We emphasize the significance of comprehensive perioperative management and provide a literature review on en bloc vascular resection for paraganglioma, offering insights into current surgical practices and outcomes.
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Affiliation(s)
| | - Saravanan Moorthy
- Department of Surgical Oncology, Cancer Institute (WIA), Chennai, India
| | - Krishna Muralidharan
- Department of Vascular Surgery, Tamil Nadu Government Multi Super Speciality Hospital, Chennai, India
| | - Anand Raja
- Department of Surgical Oncology, Cancer Institute (WIA), Chennai, India
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Ghajar D, Khana F, Zakkor MD, AlHussein H, Kayyali A. Portocaval paraganglioma: A second case report. Ann Med Surg (Lond) 2022; 80:104236. [PMID: 36045812 PMCID: PMC9422283 DOI: 10.1016/j.amsu.2022.104236] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/04/2022] [Revised: 07/18/2022] [Accepted: 07/20/2022] [Indexed: 11/29/2022] Open
Abstract
Study design Case Report. Introduction and importance To report a case of a paraganglioma presenting in an uncommon location in the abdomen. Case presentation A 24-year-old man with an abdominal lesion presented with one-year history of severe headaches and palpitations. Interventions and outcome The tumor was surgically resected and was later diagnosed as an extra-adrenal paraganglioma. Conclusion The unique location of a paraganglioma could prove misleading, making it easier to confuse it with other malignant lesions, but it still can't be excluded, and with proper techniques it can be surgically excised.
Paragangliomas (PGLs) are slow-growing tumors of neuroendocrine origin. PGLs in the portocaval region are considered extremely rare. Their unusual location has its own implications that must be carefully considered. Clinical findings with imaging techniques and laboratory tests are key to diagnosis. Surgical resection remains the gold standard of treatment.
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Affiliation(s)
- Duaa Ghajar
- Department of Endocrinology Medicine, Aleppo University Hospital (AUH), Aleppo, Syria
| | - Firas Khana
- Department of Medical Imaging and Diagnostic Radiology, Aleppo University Hospital (AUH), Aleppo, Syria
- Corresponding author.
| | - Mohammed Deeb Zakkor
- Department of Endocrinology Medicine, Aleppo University Hospital (AUH), Aleppo, Syria
| | - Hachem AlHussein
- Department of Endocrinology Medicine, Aleppo University Hospital (AUH), Aleppo, Syria
| | - Alae Kayyali
- Department of Medical Imaging and Diagnostic Radiology, Aleppo University Hospital (AUH), Aleppo, Syria
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Kramme K, Fountain R, Leinwand M. Laparoscopic resection of pheochromocytoma (paraganglioma) of the organ of Zuckerkandl in a pediatric patient. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2021.102165] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
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Constantinescu G, Preda C, Constantinescu V, Siepmann T, Bornstein SR, Lenders JWM, Eisenhofer G, Pamporaki C. Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology. Front Endocrinol (Lausanne) 2022; 13:1021420. [PMID: 36325453 PMCID: PMC9618947 DOI: 10.3389/fendo.2022.1021420] [Citation(s) in RCA: 30] [Impact Index Per Article: 10.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2022] [Accepted: 09/28/2022] [Indexed: 11/16/2022] Open
Abstract
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term "silent" PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, "clinically silent" is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term "silent" and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication.
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Affiliation(s)
- Georgiana Constantinescu
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Endocrinology, Grigore T. Popa University, Iasi, Romania
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, Germany
- *Correspondence: Christina Pamporaki, ; Georgiana Constantinescu,
| | - Cristina Preda
- Department of Endocrinology, Grigore T. Popa University, Iasi, Romania
| | - Victor Constantinescu
- Center of Clinical Neuroscience, University Clinic Carl-Gustav Carus, Dresden University of Technology, Dresden, Germany
| | - Timo Siepmann
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, Germany
- Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
| | - Stefan R. Bornstein
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, Germany
- Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King's College London, London, United Kingdom
| | - Jacques W. M. Lenders
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, Netherlands
| | - Graeme Eisenhofer
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Institute of Clinical Chemistry and Laboratory Medicine, University of Dresden, Dresden, Germany
| | - Christina Pamporaki
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- *Correspondence: Christina Pamporaki, ; Georgiana Constantinescu,
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Liu C, Wen J, Li HZ, Ji ZG. Combined thoracoscopic and laparoscopic approach to remove a large retroperitoneal compound paraganglioma: A case report. World J Clin Cases 2021; 9:6935-6942. [PMID: 34447845 PMCID: PMC8362544 DOI: 10.12998/wjcc.v9.i23.6935] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2021] [Revised: 06/14/2021] [Accepted: 06/22/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Paragangliomas (PGLs) are rare catecholamine-secreting neuroendocrine tumors, which often present with secondary hypertension. The most common location is the retroperitoneal space. For the first time, we report a rare case of large retroperitoneal compound PGL, and we have innovatively applied a new surgical plan to completely remove the tumor.
CASE SUMMARY A 55-year-old middle-aged man was admitted to the hospital for fluctuating blood pressure for more than 1 year with intermittent headache. He suffered dozens of attacks every day. Blood and urine catecholamines were elevated, somatostatin receptor imaging was positive, and the diagnosis of PGL was clear. The imaging examination revealed a large tumor on the right front of the mediastinal spine at the level of T10-L1 (the posterior space of the right phrenic foot). For the first time in our department, a combined thoracoscopic and laparoscopic operation was used to detect and remove large tumors.
CONCLUSION This is the first reported case of using a thoracoscopic and laparoscopic approach simultaneously to remove a large retroperitoneal compound PGL, which may provide a new surgical approach for similar cases.
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Affiliation(s)
- Chang Liu
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Jin Wen
- Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Han-Zhong Li
- Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Zhi-Gang Ji
- Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
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Yoshii H, Izumi H, Tajiri T, Mukai M, Nomura E, Makuuchi H. A patient with paraganglioma undergoing laparoscopic resection: A case report. Clin Case Rep 2021; 9:e04145. [PMID: 34136230 PMCID: PMC8190555 DOI: 10.1002/ccr3.4145] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2021] [Revised: 03/28/2021] [Accepted: 03/29/2021] [Indexed: 11/09/2022] Open
Abstract
Paraganglioma is a very rare extraadrenal nonepithelial tumor. The number of cases of laparoscopic surgery in Paraganglioma is small and controversial. This study encountered a case of successful transperitoneal laparoscopic surgery for a 56-mm paraganglioma in a 53-year-old female. Moreover, previous reports on laparoscopic surgery for paraganglioma are reviewed.
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Affiliation(s)
- Hisamichi Yoshii
- Department of SurgeryTokai University School of MedicineIseharaJapan
| | - Hideki Izumi
- Department of SurgeryTokai University School of MedicineIseharaJapan
| | - Takuma Tajiri
- Department of PathologyTokai University School of MedicineIseharaJapan
| | - Masaya Mukai
- Department of SurgeryTokai University School of MedicineIseharaJapan
| | - Eiji Nomura
- Department of SurgeryTokai University School of MedicineIseharaJapan
| | - Hiroyasu Makuuchi
- Department of SurgeryTokai University School of MedicineIseharaJapan
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