Laparoscopic resection of a large clinically silent paraganglioma at the organ of Zuckerkandl: a rare case report and review of the literature

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Abstract

Background

Large paraganglioma of the Zuckerkandl organ (POZ) is extremely rare. The patient can occasionally be paucisymptomatic, further obscuring the diagnosis and carrying high mortality. Recommended treatment for large paraganglioma (PGL) is open surgical removal. We report a case of successful laparoscopic resection of a large POZ with normal blood pressure in a 45-year-old man.

Case presentation

A 45-year-old man was hospitalized because of hyperglycemia. Computed tomography of the abdomen and the serum and urinary catecholamine levels confirmed the diagnosis of large POZ. But his blood pressure was normal and he underwent laparoscopic tumor excision successfully. During 6 months follow-up after laparoscopy, serum and urinary catecholamines were normal but glycaemia remained high level. DNA analysis of the succinate dehydrogenase complex subunits B (SDHB) and SDHD revealed no mutation.

Conclusions

POZ is an unusual mass and preoperative diagnosis can be difficult in clinically silent cases. PGL cannot be excluded in patients with normal blood pressure. Even a large POZ can be excised laparoscopically by following proper techniques.

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Most cited references 21

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The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer.

Karel Pacak, Herbert Chen, Ricardo V. Lloyd … (2010)

Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal chromaffin cells or similar cells in extra-adrenal sympathetic and parasympathetic paraganglia, respectively. Serious morbidity and mortality rates associated with these tumors are related to the potent effects of catecholamines on various organs, especially those of the cardiovascular system. Before any surgical procedure is done, preoperative blockade is necessary to protect the patient against significant release of catecholamines due to anesthesia and surgical manipulation of the tumor. Treatment options vary with the extent of the disease, with laparoscopic surgery being the preferred treatment for removal of primary tumors. Medullary thyroid cancer (MTC) is a malignancy of the thyroid C cells or parafollicular cells. Thyroid C cells elaborate a number of peptides and hormones, such as calcitonin, carcinoembryonic antigen, and chromogranin A. Some or all of these markers are elevated in patients with MTC and can be used to confirm the diagnosis as well as to follow patients longitudinally for recurrence. Medullary thyroid cancer consists of a spectrum of diseases that ranges from extremely indolent tumors that are stable for many years to aggressive types associated with a high mortality rate. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure. The only curative treatment is complete surgical resection.

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Diagnosis and management of pheochromocytoma.

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A case of clinically silent giant right pheochromocytoma and review of literature.

Changfu Li, Yongsheng Chen, Wentao Wang … (2012)

Clinically silent giant cystic pheochromocytoma is a rare adrenal disease; complete resection is the only curative treatment. Due to the serious surgical risk, successful resection is very difficult. We report a case of a patient with giant cystic pheochromocytoma, which was successfully resected. There were no intraoperative and postoperative complications. We analyze and report our experience.

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Author and article information

Contributors

Jiwen Shang:

ORCID: http://orcid.org/0000-0003-2971-4683

sjw139@126.com

Journal

Journal ID (nlm-ta): BMC Urol

Journal ID (iso-abbrev): BMC Urol

Title: BMC Urology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2490

Publication date (Electronic): 7 October 2020

Publication date PMC-release: 7 October 2020

Publication date Collection: 2020

Volume: 20

Electronic Location Identifier: 156

Affiliations

[1 ]GRID grid.263452.4, ISNI 0000 0004 1798 4018, Graduate School, , Shanxi Medical University, ; Taiyuan, 030000 China

[2 ]Department of Urology, Shanxi Bethune Hospital, No. 99 Longcheng Street, Taiyuan, 030032 Shanxi China

Author information

Jiwen Shang http://orcid.org/0000-0003-2971-4683

Article

Publisher ID: 732

DOI: 10.1186/s12894-020-00732-0

PMC ID: 7542907

PubMed ID: 33028271

SO-VID: b1fdfd5e-7697-4b14-b659-84b629ae73c6

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 21 August 2020

Date accepted : 29 September 2020

Funding

Funded by: FundRef http://dx.doi.org/10.13039/501100013318, ShanXi Science and Technology Department;

Award ID: 201903D321130

Award Recipient : Jiwen Shang

Custom metadata

ScienceOpen disciplines: Urology

Keywords: paraganglioma,large,silent,organ of zuckerkandl,laparoscopy,genetic testing

Data availability:

ScienceOpen disciplines: Urology

Keywords: paraganglioma, large, silent, organ of zuckerkandl, laparoscopy, genetic testing

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Laparoscopic resection of a large clinically silent paraganglioma at the organ of Zuckerkandl: a rare case report and review of the literature

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Most cited references 21

The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer.

Diagnosis and management of pheochromocytoma.

A case of clinically silent giant right pheochromocytoma and review of literature.

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