Imaging in Functional Posterior Mediastinal Ganglioneuroma Presenting with Catecholamine-Induced Dilated Cardiomyopathy

Imaging in Functional Posterior Mediastinal Ganglioneuroma Presenting with Catecholamine-Induced Dilated Cardiomyopathy | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Imaging in Functional Posterior Mediastinal Ganglioneuroma Presenting with Catecholamine-Induced Dilated Cardiomyopathy Imran Salam Teeli, Irfan Robbani This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7168388/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 18 Apr, 2026 Read the published version in Egyptian Journal of Radiology and Nuclear Medicine → Version 1 posted You are reading this latest preprint version Abstract Ganglioneuromas are rare, benign neural crest-derived tumors capable of secreting catecholamines and clinically mimicking more aggressive neoplasms such as paragangliomas. We present the case of a 35-year-old woman with paroxysmal hypertension, palpitations, and exertional dyspnea, found to have catecholamine-induced dilated cardiomyopathy and a posterior mediastinal mass. Imaging suggested a paraganglioma, but histopathological and immunohistochemical evaluation confirmed a functional ganglioneuroma. Following preoperative alpha-adrenergic blockade, complete surgical excision of the tumor led to resolution of symptoms, normalization of blood pressure, and significant improvement in cardiac function. This case emphasizes the importance of early recognition and multidisciplinary management of functional ganglioneuromas to prevent serious cardiovascular complications and ensure favorable outcomes. ganglioneuroma catecholamine-secreting tumor posterior mediastinum paraganglioma mimic dilated cardiomyopathy sympathetic nervous system Figures Figure 1 Figure 2 Figure 3 Figure 4 Introduction Ganglioneuroma is a rare, benign tumor of neural crest origin that typically arises from the sympathetic chain[ 1 ]. Functional ganglioneuromas, which secrete catecholamines, are exceptionally uncommon and can clinically resemble more aggressive tumors such as paragangliomas[ 2 ]. The estimated prevalence of ganglioneuroma is approximately one per million population. These tumors are usually very slow-growing and often asymptomatic, with many cases discovered incidentally during imaging for unrelated conditions. However, when symptoms do occur, they are typically due to the mass effect of the tumor, such as compression of adjacent structures or, in rare functional cases, due to hormone secretion. Symptomatic cases warrant further evaluation through imaging and biochemical studies to guide appropriate management[ 3 , 4 ]. We report the case of a 35-year-old female presenting with paroxysmal hypertension, palpitations, and progressive exertional dyspnea. Imaging and biochemical evaluation revealed a catecholamine-secreting posterior mediastinal mass initially suspected to be a paraganglioma. However, histopathological and immunohistochemical analyses confirmed the diagnosis of a functional ganglioneuroma. This case highlights the diagnostic complexity and clinical implications of functional ganglioneuromas, emphasizing the importance of early recognition and complete surgical resection for favorable outcomes. Case Presentation A 35-year-old woman presented with progressive exertional dyspnea, episodic palpitations, and paroxysmal hypertension. She had no prior history of cardiovascular disease or significant medical history. On admission, her blood pressure was 180/100 mmHg, and electrocardiography showed sinus tachycardia without ischemic changes. Transthoracic echocardiography revealed global hypokinesia of the left ventricular myocardium with chamber dilatation and a reduced ejection fraction of 40%, consistent with non-ischemic dilated cardiomyopathy. Given her symptoms and the absence of traditional cardiovascular risk factors, a secondary cause of hypertension and cardiac dysfunction was suspected. Biochemical testing showed significantly elevated plasma free metanephrines and 24-hour urinary catecholamines. Chest radiography identified a posterior mediastinal mass with right-sided paravertebral displacement(Fig. 1). Contrast-enhanced computed tomography (CT) of the chest revealed a well-circumscribed, homogenous soft tissue lesion (6 × 4 cm) in the right paravertebral region, with mild progressive post-contrast enhancement and no calcifications or evidence of invasion into adjacent structures(Fig. 2). Magnetic resonance imaging (MRI) further characterized the lesion as hypointense on T1-weighted images and hyperintense on T2-weighted images, with a distinctive whorled internal architecture. These radiologic features were suggestive of a catecholamine-producing tumor, such as a paraganglioma(Fig. 3). A CT-guided core needle biopsy was performed. Histological analysis revealed mature ganglion cells embedded in abundant Schwannian stroma, without atypia or mitotic activity. Immunohistochemistry was positive for S-100 and synaptophysin, but negative for chromogranin, confirming the diagnosis of a benign ganglioneuroma of sympathetic origin(Fig. 4). The patient was stabilized preoperatively with alpha-adrenergic blockade. Surgical excision of the mass was performed via a right posterolateral thoracotomy. Postoperative recovery was uneventful, and follow-up echocardiography three months later showed significant improvement in cardiac function with an ejection fraction of 55%. Blood pressure normalized, and symptoms resolved completely. Differential Diagnosis In the evaluation of a posterior mediastinal mass with catecholamine secretion, several differential diagnoses were considered. Paragangliomas are extra-adrenal neuroendocrine tumors that commonly secrete catecholamines and closely mimic the presentation of functional ganglioneuromas[ 1 ]. Pheochromocytomas, typically arising from the adrenal medulla, also present with paroxysmal hypertension and elevated catecholamines but are rarely found in the posterior mediastinum[ 5 ]. Neuroblastomas are malignant tumors seen predominantly in children and may present with similar imaging features and catecholamine secretion[ 6 ]. Schwannomas are benign nerve sheath tumors that can occur in the paravertebral region but are usually non-functional and lack biochemical evidence of catecholamine excess[ 7 ]. Radiologic features such as enhancement patterns and tissue architecture, along with histopathology and immunohistochemistry, are critical in distinguishing between these entities. In this case, the presence of mature ganglion cells and Schwannian stroma without atypia confirmed the diagnosis of a benign functional ganglioneuroma. Management Strategy Management of functional ganglioneuroma requires a multidisciplinary approach. Preoperative alpha-adrenergic blockade is essential to prevent intraoperative hypertensive crises due to catecholamine release. In some cases, beta-blockers may be added after adequate alpha blockade to control tachycardia. Surgical resection remains the definitive treatment, with complete excision preferred to minimize the risk of recurrence and symptom persistence. The choice between open or minimally invasive surgery depends on the tumor’s size, location, and proximity to vital structures. Post-operative cardiac monitoring is crucial, especially in patients with catecholamine-induced cardiomyopathy, to evaluate functional recovery and guide further management. Long-term follow-up is recommended to monitor for late recurrence and assess cardiovascular stability. Discussion Ganglioneuromas are rare, benign neoplasms that arise from neural crest-derived cells of the sympathetic nervous system. They typically occur in children and young adults and are often discovered incidentally. However, functional GNs, though rare, may secrete catecholamines and present with features resembling pheochromocytoma or paraganglioma. Clinical manifestations can include paroxysmal hypertension, palpitations, headaches, diaphoresis, and even cardiomyopathy due to catecholamine-induced myocardial dysfunction. In our case, the patient presented with features of sympathetic overdrive and non-ischemic cardiomyopathy, leading to the suspicion of a functional neuroendocrine tumor. Imaging plays a vital role in localization and characterization. On CT, GNs typically appear as well-defined, low-attenuation masses with minimal enhancement. MRI offers superior soft tissue contrast, with GNs demonstrating low signal intensity on T1 and high signal on T2-weighted images, often with a characteristic whorled appearance. Iodine-123 MIBG scintigraphy can further aid in confirming catecholamine activity. Histopathological confirmation remains the gold standard. GNs demonstrate mature ganglion cells within a background of Schwannian stroma. Immunohistochemistry typically shows positivity for S-100 protein and synaptophysin, while chromogranin may be variably expressed. Complete surgical excision is the treatment of choice, and prognosis is excellent with minimal risk of recurrence. Preoperative adrenergic blockade is crucial to avoid perioperative hypertensive crises in functional tumors. Postoperative monitoring is necessary to assess cardiac recovery and detect recurrence. Conclusion Functional ganglioneuroma of the posterior mediastinum is an uncommon but critical diagnosis, especially when complicated by catecholamine-induced dilated cardiomyopathy. Early recognition through multimodal imaging and biochemical testing is essential for appropriate surgical planning and prevention of cardiovascular complications. Complete resection can lead to significant cardiac recovery, underlining the importance of considering this rare but treatable entity. Declarations Funding This study received no financial support from any institution or individual. Ethics Approval and Consent to Participate Not applicable. Patient Consent Informed consent was obtained from the patient for the publication of this case. Consent for Publication Written informed consent was obtained from the patient for publication of this case report and accompanying images. Author Contribution Imran the main author wrote the manuscript References Iacobone, M., Torresan, F., Citton, M., Schiavone, D., Viel, G., & Favia, G. (2017). Adrenal ganglioneuroma: The Padua Endocrine Surgery Unit experience. International journal of surgery (London, England), 41 Suppl 1, S103–S108. https://doi.org/10.1016/j.ijsu.2017.03.086 Ikram A, Rehman A. Paraganglioma. [Updated 2024 Feb 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK549834/ Duffy, S., Jhaveri, M., Scudierre, J., Cochran, E., & Huckman, M. (2005). MR imaging of a posterior mediastinal ganglioneuroma: fat as a useful diagnostic sign. AJNR. American journal of neuroradiology, 26(10), 2658–2662. Dąbrowska-Thing, A., Rogowski, W., Pacho, R., Nawrocka-Laskus, E., & Nitek, Ż. (2017). Retroperitoneal Ganglioneuroma Mimicking a Kidney Tumor. Case Report. Polish journal of radiology, 82, 283–286. https://doi.org/10.12659/PJR.899633 Gupta PK, Marwaha B. Pheochromocytoma. [Updated 2024 Nov 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK589700/ Mahapatra S, Challagundla KB. Neuroblastoma. [Updated 2023 Jul 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK448111/ Hilton, D. A., & Hanemann, C. O. (2014). Schwannomas and their pathogenesis. Brain pathology (Zurich, Switzerland), 24(3), 205–220. https://doi.org/10.1111/bpa.12125 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 18 Apr, 2026 Read the published version in Egyptian Journal of Radiology and Nuclear Medicine → Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7168388","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":499380042,"identity":"d5fbb680-74f8-4d33-bd2c-2f9feeb3e2bd","order_by":0,"name":"Imran Salam Teeli","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABCklEQVRIiWNgGAWjYFACHhAhAWYe+FAhIQdmPCBSC+PBGWcsjMFaEghrAQPmw5xtFYkNICY+LebtZw8++FBmEQ1kHDjMwCaRPj/s8EOgLXZyug3YtcicyUs2nHFOInfOmbyEwwU8Erkbb6cZALUkG5sdwK5FgiHHTJq3TSJ3BkOOweEZEkAtsxNAWg4kbsOlhf+N+e+/IC38bwwO8xhIpBvOTv+AX4tEjhkzI0iLBNAWngSJBHnpHAK2SLwxluw5B9LyxuDgjAMShhukcwoOJBjg8Qt/juGHH2V1QIflGH/4+K9OXn52+uYPHyrs5HBpgQA2JLYBWKUBPuXoWuQbCKkeBaNgFIyCkQYAxhlirme6vd0AAAAASUVORK5CYII=","orcid":"","institution":"Sher-i-Kashmir Institute of Medical Sciences","correspondingAuthor":true,"prefix":"","firstName":"Imran","middleName":"Salam","lastName":"Teeli","suffix":""},{"id":499380043,"identity":"f20214d1-5555-4505-8a66-eab3e76a2b34","order_by":1,"name":"Irfan Robbani","email":"","orcid":"","institution":"Sher-i-Kashmir Institute of Medical Sciences","correspondingAuthor":false,"prefix":"","firstName":"Irfan","middleName":"","lastName":"Robbani","suffix":""}],"badges":[],"createdAt":"2025-07-20 08:23:18","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7168388/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7168388/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s43055-025-01667-y","type":"published","date":"2026-04-18T15:58:16+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":89262210,"identity":"a69ecc07-9962-4a30-a243-f47a3bea3bc5","added_by":"auto","created_at":"2025-08-18 07:11:49","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":90174,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eInitial chest radiograph showing a posterior mediastinal mass.\u003c/em\u003e The frontal chest X-ray demonstrates a well-defined right-sided paravertebral opacity with displacement of the mediastinal contour, suggestive of a posterior mediastinal mass. The appearance raises suspicion for a neurogenic or catecholamine-secreting tumor.\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7168388/v1/778de5e7f9daeae16c9573c2.jpg"},{"id":89261458,"identity":"09695c48-3b4c-496b-97dc-658f837f7440","added_by":"auto","created_at":"2025-08-18 07:03:50","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":88141,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eContrast-enhanced computed tomography (CT) of the chest characterizing the mediastinal mass.\u003c/em\u003e Axial contrast-enhanced CT image reveals a well-circumscribed, homogenous soft tissue lesion measuring approximately 6 × 4 cm in the right paravertebral region. The lesion exhibits mild progressive post-contrast enhancement without internal calcifications, vascular invasion, or bony erosion, consistent with a likely neurogenic origin.\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7168388/v1/05f8b9570e87af8e1fcb4085.jpg"},{"id":89261455,"identity":"dade5626-275a-434d-9b7d-7c7301c59ff6","added_by":"auto","created_at":"2025-08-18 07:03:50","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":107877,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eMagnetic resonance imaging (MRI) of the thorax demonstrating characteristic features of the posterior mediastinal lesion.\u003c/em\u003eThe lesion appears hypointense on T1-weighted sequences and hyperintense on T2-weighted images, with a distinctive whorled internal architecture. Post-contrast images show heterogeneous but gradual enhancement. These imaging features are radiologically suggestive of a catecholamine-producing tumor, such as a paraganglioma.\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7168388/v1/7b1cbb7c051f60e09fe942c9.jpg"},{"id":89261462,"identity":"3250d5e9-f48c-4bad-a541-6ed8039f23e7","added_by":"auto","created_at":"2025-08-18 07:03:50","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":117243,"visible":true,"origin":"","legend":"\u003cp\u003eHistopathological examination of CT-guided core needle biopsy specimen. The section shows mature ganglion cells (black arrows) embedded in abundant Schwannian stroma (asterisks), characteristic of a benign ganglioneuroma. No cytological atypia or mitotic activity is observed.\u003c/p\u003e","description":"","filename":"4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7168388/v1/ac946ecc90bcac0a40db6081.jpg"},{"id":107350755,"identity":"1d703cd1-4484-4d40-9181-85cd667dc00e","added_by":"auto","created_at":"2026-04-20 16:03:15","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":536948,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7168388/v1/18059a4d-2c18-42ae-aa75-c1c082a86930.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Imaging in Functional Posterior Mediastinal Ganglioneuroma Presenting with Catecholamine-Induced Dilated Cardiomyopathy","fulltext":[{"header":"Introduction","content":"\u003cp\u003eGanglioneuroma is a rare, benign tumor of neural crest origin that typically arises from the sympathetic chain[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Functional ganglioneuromas, which secrete catecholamines, are exceptionally uncommon and can clinically resemble more aggressive tumors such as paragangliomas[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The estimated prevalence of ganglioneuroma is approximately one per million population. These tumors are usually very slow-growing and often asymptomatic, with many cases discovered incidentally during imaging for unrelated conditions. However, when symptoms do occur, they are typically due to the \u003cb\u003emass effect\u003c/b\u003e of the tumor, such as compression of adjacent structures or, in rare functional cases, due to hormone secretion. Symptomatic cases warrant further evaluation through imaging and biochemical studies to guide appropriate management[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eWe report the case of a 35-year-old female presenting with paroxysmal hypertension, palpitations, and progressive exertional dyspnea. Imaging and biochemical evaluation revealed a catecholamine-secreting posterior mediastinal mass initially suspected to be a paraganglioma. However, histopathological and immunohistochemical analyses confirmed the diagnosis of a functional ganglioneuroma. This case highlights the diagnostic complexity and clinical implications of functional ganglioneuromas, emphasizing the importance of early recognition and complete surgical resection for favorable outcomes.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 35-year-old woman presented with progressive exertional dyspnea, episodic palpitations, and paroxysmal hypertension. She had no prior history of cardiovascular disease or significant medical history. On admission, her blood pressure was 180/100 mmHg, and electrocardiography showed sinus tachycardia without ischemic changes. Transthoracic echocardiography revealed global hypokinesia of the left ventricular myocardium with chamber dilatation and a reduced ejection fraction of 40%, consistent with non-ischemic dilated cardiomyopathy. Given her symptoms and the absence of traditional cardiovascular risk factors, a secondary cause of hypertension and cardiac dysfunction was suspected.\u003c/p\u003e\u003cp\u003eBiochemical testing showed significantly elevated plasma free metanephrines and 24-hour urinary catecholamines. Chest radiography identified a posterior mediastinal mass with right-sided paravertebral displacement(Fig.\u0026nbsp;1). Contrast-enhanced computed tomography (CT) of the chest revealed a well-circumscribed, homogenous soft tissue lesion (6 \u0026times; 4 cm) in the right paravertebral region, with mild progressive post-contrast enhancement and no calcifications or evidence of invasion into adjacent structures(Fig.\u0026nbsp;2).\u003c/p\u003e\u003cp\u003eMagnetic resonance imaging (MRI) further characterized the lesion as hypointense on T1-weighted images and hyperintense on T2-weighted images, with a distinctive whorled internal architecture. These radiologic features were suggestive of a catecholamine-producing tumor, such as a paraganglioma(Fig.\u0026nbsp;3). A CT-guided core needle biopsy was performed. Histological analysis revealed mature ganglion cells embedded in abundant Schwannian stroma, without atypia or mitotic activity. Immunohistochemistry was positive for S-100 and synaptophysin, but negative for chromogranin, confirming the diagnosis of a benign ganglioneuroma of sympathetic origin(Fig.\u0026nbsp;4).\u003c/p\u003e\u003cp\u003eThe patient was stabilized preoperatively with alpha-adrenergic blockade. Surgical excision of the mass was performed via a right posterolateral thoracotomy. Postoperative recovery was uneventful, and follow-up echocardiography three months later showed significant improvement in cardiac function with an ejection fraction of 55%. Blood pressure normalized, and symptoms resolved completely.\u003c/p\u003e\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eDifferential Diagnosis\u003c/h2\u003e\u003cp\u003eIn the evaluation of a posterior mediastinal mass with catecholamine secretion, several differential diagnoses were considered. Paragangliomas are extra-adrenal neuroendocrine tumors that commonly secrete catecholamines and closely mimic the presentation of functional ganglioneuromas[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Pheochromocytomas, typically arising from the adrenal medulla, also present with paroxysmal hypertension and elevated catecholamines but are rarely found in the posterior mediastinum[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Neuroblastomas are malignant tumors seen predominantly in children and may present with similar imaging features and catecholamine secretion[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Schwannomas are benign nerve sheath tumors that can occur in the paravertebral region but are usually non-functional and lack biochemical evidence of catecholamine excess[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Radiologic features such as enhancement patterns and tissue architecture, along with histopathology and immunohistochemistry, are critical in distinguishing between these entities. In this case, the presence of mature ganglion cells and Schwannian stroma without atypia confirmed the diagnosis of a benign functional ganglioneuroma.\u003c/p\u003e\u003c/div\u003e\n\u003ch3\u003eManagement Strategy\u003c/h3\u003e\n\u003cp\u003eManagement of functional ganglioneuroma requires a multidisciplinary approach. Preoperative alpha-adrenergic blockade is essential to prevent intraoperative hypertensive crises due to catecholamine release. In some cases, beta-blockers may be added after adequate alpha blockade to control tachycardia. Surgical resection remains the definitive treatment, with complete excision preferred to minimize the risk of recurrence and symptom persistence. The choice between open or minimally invasive surgery depends on the tumor\u0026rsquo;s size, location, and proximity to vital structures. Post-operative cardiac monitoring is crucial, especially in patients with catecholamine-induced cardiomyopathy, to evaluate functional recovery and guide further management. Long-term follow-up is recommended to monitor for late recurrence and assess cardiovascular stability.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eGanglioneuromas are rare, benign neoplasms that arise from neural crest-derived cells of the sympathetic nervous system. They typically occur in children and young adults and are often discovered incidentally. However, functional GNs, though rare, may secrete catecholamines and present with features resembling pheochromocytoma or paraganglioma.\u003c/p\u003e\u003cp\u003eClinical manifestations can include paroxysmal hypertension, palpitations, headaches, diaphoresis, and even cardiomyopathy due to catecholamine-induced myocardial dysfunction. In our case, the patient presented with features of sympathetic overdrive and non-ischemic cardiomyopathy, leading to the suspicion of a functional neuroendocrine tumor.\u003c/p\u003e\u003cp\u003eImaging plays a vital role in localization and characterization. On CT, GNs typically appear as well-defined, low-attenuation masses with minimal enhancement. MRI offers superior soft tissue contrast, with GNs demonstrating low signal intensity on T1 and high signal on T2-weighted images, often with a characteristic whorled appearance. Iodine-123 MIBG scintigraphy can further aid in confirming catecholamine activity. Histopathological confirmation remains the gold standard. GNs demonstrate mature ganglion cells within a background of Schwannian stroma. Immunohistochemistry typically shows positivity for S-100 protein and synaptophysin, while chromogranin may be variably expressed.\u003c/p\u003e\u003cp\u003eComplete surgical excision is the treatment of choice, and prognosis is excellent with minimal risk of recurrence. Preoperative adrenergic blockade is crucial to avoid perioperative hypertensive crises in functional tumors. Postoperative monitoring is necessary to assess cardiac recovery and detect recurrence.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eFunctional ganglioneuroma of the posterior mediastinum is an uncommon but critical diagnosis, especially when complicated by catecholamine-induced dilated cardiomyopathy. Early recognition through multimodal imaging and biochemical testing is essential for appropriate surgical planning and prevention of cardiovascular complications. Complete resection can lead to significant cardiac recovery, underlining the importance of considering this rare but treatable entity.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study received no financial support from any institution or individual.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics Approval and Consent to Participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePatient Consent\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eInformed consent was obtained from the patient for the publication of this case.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for Publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for publication of this case report and accompanying images.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eImran the main author wrote the manuscript\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eIacobone, M., Torresan, F., Citton, M., Schiavone, D., Viel, G., \u0026amp; Favia, G. (2017). Adrenal ganglioneuroma: The Padua Endocrine Surgery Unit experience. International journal of surgery (London, England), 41 Suppl 1, S103\u0026ndash;S108. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.ijsu.2017.03.086\u003c/span\u003e\u003cspan address=\"10.1016/j.ijsu.2017.03.086\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eIkram A, Rehman A. Paraganglioma. [Updated 2024 Feb 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.ncbi.nlm.nih.gov/books/NBK549834/\u003c/span\u003e\u003cspan address=\"https://www.ncbi.nlm.nih.gov/books/NBK549834/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDuffy, S., Jhaveri, M., Scudierre, J., Cochran, E., \u0026amp; Huckman, M. (2005). MR imaging of a posterior mediastinal ganglioneuroma: fat as a useful diagnostic sign. AJNR. American journal of neuroradiology, 26(10), 2658\u0026ndash;2662.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDąbrowska-Thing, A., Rogowski, W., Pacho, R., Nawrocka-Laskus, E., \u0026amp; Nitek, Ż. (2017). Retroperitoneal Ganglioneuroma Mimicking a Kidney Tumor. Case Report. 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Brain pathology (Zurich, Switzerland), 24(3), 205\u0026ndash;220. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1111/bpa.12125\u003c/span\u003e\u003cspan address=\"10.1111/bpa.12125\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"ganglioneuroma, catecholamine-secreting tumor, posterior mediastinum, paraganglioma mimic, dilated cardiomyopathy, sympathetic nervous system","lastPublishedDoi":"10.21203/rs.3.rs-7168388/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7168388/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eGanglioneuromas are rare, benign neural crest-derived tumors capable of secreting catecholamines and clinically mimicking more aggressive neoplasms such as paragangliomas. We present the case of a 35-year-old woman with paroxysmal hypertension, palpitations, and exertional dyspnea, found to have catecholamine-induced dilated cardiomyopathy and a posterior mediastinal mass. Imaging suggested a paraganglioma, but histopathological and immunohistochemical evaluation confirmed a functional ganglioneuroma. Following preoperative alpha-adrenergic blockade, complete surgical excision of the tumor led to resolution of symptoms, normalization of blood pressure, and significant improvement in cardiac function. This case emphasizes the importance of early recognition and multidisciplinary management of functional ganglioneuromas to prevent serious cardiovascular complications and ensure favorable outcomes.\u003c/p\u003e","manuscriptTitle":"Imaging in Functional Posterior Mediastinal Ganglioneuroma Presenting with Catecholamine-Induced Dilated Cardiomyopathy","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-08-18 07:03:45","doi":"10.21203/rs.3.rs-7168388/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"3317df2e-555a-4808-8346-31a9e7afbebc","owner":[],"postedDate":"August 18th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-04-20T16:01:21+00:00","versionOfRecord":{"articleIdentity":"rs-7168388","link":"https://doi.org/10.1186/s43055-025-01667-y","journal":{"identity":"egyptian-journal-of-radiology-and-nuclear-medicine","isVorOnly":false,"title":"Egyptian Journal of Radiology and Nuclear Medicine"},"publishedOn":"2026-04-18 15:58:16","publishedOnDateReadable":"April 18th, 2026"},"versionCreatedAt":"2025-08-18 07:03:45","video":"","vorDoi":"10.1186/s43055-025-01667-y","vorDoiUrl":"https://doi.org/10.1186/s43055-025-01667-y","workflowStages":[]},"version":"v1","identity":"rs-7168388","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7168388","identity":"rs-7168388","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}