Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report

Abstract Background Coexistence of a catecholamine-secreting tumor and an adrenal cortical tumor is quite rare which makes both diagnosis and management challenging. The purpose of this article is to describe the presence of this condition, share a stepwise approach for preoperative evaluation, and...

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Main Authors: Yu-Chen Hsu, Cheng-Han Lee, Chen-Yu Chen, Chung-Jye Hung
Format: Article
Language:English
Published: BMC 2022-11-01
Series:BMC Endocrine Disorders
Subjects:
Online Access:https://doi.org/10.1186/s12902-022-01181-6
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author Yu-Chen Hsu
Cheng-Han Lee
Chen-Yu Chen
Chung-Jye Hung
author_facet Yu-Chen Hsu
Cheng-Han Lee
Chen-Yu Chen
Chung-Jye Hung
author_sort Yu-Chen Hsu
collection DOAJ
description Abstract Background Coexistence of a catecholamine-secreting tumor and an adrenal cortical tumor is quite rare which makes both diagnosis and management challenging. The purpose of this article is to describe the presence of this condition, share a stepwise approach for preoperative evaluation, and review the related literature. Case presentation A 44-year-old male patient had a history of hypertension and aggravating hypokalemia for years. Abdominal computed tomography incidentally found concomitant bilateral adrenal and left para-aortic tumors. Comprehensive adrenal hormone tests revealed a high aldosterone renin ratio and mildly elevated 24-h urine vanillylmandelic acid and norepinephrine levels. Subsequently, a metaiodobenzylguanidine scan showed uptake over the left para-aortic tumor, and NP-59 adrenal scintigraphy showed uptake over the left adrenal tumor. Further confirmatory tests, including captopril suppression, irbesartan suppression, and saline infusion, all confirmed the diagnosis of hyperaldosteronism. Adrenal venous sampling following 2 months of preparation with an alpha blocker demonstrated a left aldosterone-producing adrenal adenoma. Combining hormonal analysis, imaging studies, and adrenal venous sampling, the patient was diagnosed with left adrenal aldosteronoma, right adrenal nonfunctional tumor, and left para-aortic paraganglioma (PGL). Accordingly, laparoscopic left adrenalectomy and left PGL excision were performed smoothly under alpha blocker maintenance. The pathology report confirmed left adrenal cortical adenoma and left para-aortic PGL. Postoperatively, the blood pressure, biochemical tests, and adrenal hormone assays returned to normal, and related symptoms disappeared and were relatively stable during the follow-up period of two years. Conclusions This is the first case of left para-aortic PGL coexisting with an ipsilateral aldosterone-producing adenoma presenting as a left para-aortic tumor associated with bilateral adrenal tumors. Awareness of the rarity of this coexistence can avoid unexpected disasters during the process of evaluation and management.
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spelling doaj.art-c6dc68ab0f824df6b6e2ffdbd505381a2022-12-22T04:39:17ZengBMCBMC Endocrine Disorders1472-68232022-11-012211810.1186/s12902-022-01181-6Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case reportYu-Chen Hsu0Cheng-Han Lee1Chen-Yu Chen2Chung-Jye Hung3Department of Obstetrics and Gynecology, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung UniversityDepartment of Surgery, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung UniversityDepartment of Surgery, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung UniversityDepartment of Surgery, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung UniversityAbstract Background Coexistence of a catecholamine-secreting tumor and an adrenal cortical tumor is quite rare which makes both diagnosis and management challenging. The purpose of this article is to describe the presence of this condition, share a stepwise approach for preoperative evaluation, and review the related literature. Case presentation A 44-year-old male patient had a history of hypertension and aggravating hypokalemia for years. Abdominal computed tomography incidentally found concomitant bilateral adrenal and left para-aortic tumors. Comprehensive adrenal hormone tests revealed a high aldosterone renin ratio and mildly elevated 24-h urine vanillylmandelic acid and norepinephrine levels. Subsequently, a metaiodobenzylguanidine scan showed uptake over the left para-aortic tumor, and NP-59 adrenal scintigraphy showed uptake over the left adrenal tumor. Further confirmatory tests, including captopril suppression, irbesartan suppression, and saline infusion, all confirmed the diagnosis of hyperaldosteronism. Adrenal venous sampling following 2 months of preparation with an alpha blocker demonstrated a left aldosterone-producing adrenal adenoma. Combining hormonal analysis, imaging studies, and adrenal venous sampling, the patient was diagnosed with left adrenal aldosteronoma, right adrenal nonfunctional tumor, and left para-aortic paraganglioma (PGL). Accordingly, laparoscopic left adrenalectomy and left PGL excision were performed smoothly under alpha blocker maintenance. The pathology report confirmed left adrenal cortical adenoma and left para-aortic PGL. Postoperatively, the blood pressure, biochemical tests, and adrenal hormone assays returned to normal, and related symptoms disappeared and were relatively stable during the follow-up period of two years. Conclusions This is the first case of left para-aortic PGL coexisting with an ipsilateral aldosterone-producing adenoma presenting as a left para-aortic tumor associated with bilateral adrenal tumors. Awareness of the rarity of this coexistence can avoid unexpected disasters during the process of evaluation and management.https://doi.org/10.1186/s12902-022-01181-6ParagangliomaHyperaldosteronismMetaiodobenzylguanidine scanAdrenal venous samplingCase report
spellingShingle Yu-Chen Hsu
Cheng-Han Lee
Chen-Yu Chen
Chung-Jye Hung
Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
BMC Endocrine Disorders
Paraganglioma
Hyperaldosteronism
Metaiodobenzylguanidine scan
Adrenal venous sampling
Case report
title Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
title_full Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
title_fullStr Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
title_full_unstemmed Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
title_short Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report
title_sort left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors comprehensive adrenal evaluation aiding perfect management a case report
topic Paraganglioma
Hyperaldosteronism
Metaiodobenzylguanidine scan
Adrenal venous sampling
Case report
url https://doi.org/10.1186/s12902-022-01181-6
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AT chenyuchen leftadrenalaldosteronismcoexistingwithleftparaaorticparagangliomapresentingasbilateraladrenalandleftparaaortictumorscomprehensiveadrenalevaluationaidingperfectmanagementacasereport
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