A cardiac mass revealing a thoracic neuroblastoma in a four-years- old: a case report

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Ouafik, S. Tizki, H. Arache, S. Safadi, H. Ouhsousou, I. Bazi, and 8 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4379076/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 3 You are reading this latest preprint version Abstract Neuroblastoma, an embryonal malignant tumor originating from neuroblasts within the sympathetic nervous system, is the third most prevalent pediatric malignancy, following leukemias and central nervous system tumors. The primary sites of metastases from neuroblastoma are the bone marrow, bones, and lymph nodes. Cardiac extension is an exceptionally rare occurrence. We report the case of a four-year-old child, with no prior medical history of cardiopathy, who had been experiencing exertional dyspnea for a few months leading to his admission. The patient presented to the pediatric emergency department for shortness of breath, chest pain, and fever.The clinical findings were: polypnea, signs of respiratory distress, bilateral jugular vein distention, and a systolic heart murmur at all the cardiac auscultation sites. A chest X-ray showed cardiomegaly and alveolar edema. Echocardiography revealed a large atrial septal defect with right heart enlargement, severe pulmonary hypertension, and an echogenic mobile lesion in the left atrium. Further investigations revealed a large left posterior mediastinal mass with calcifications extending to the left atrium. A biopsy was performed and histopathology of the mediastinal mass revealed neuroblastoma. The intracardiac extension of tumors in the pediatric population is rare. Thoracic and cardiac imagery can be used to characterize the mass further and adapt treatment. To the best of our knowledge, this is the first published case of a posterior mediastinal neuroblastoma with direct intracardiac extension. Cardiac mass neuroblastoma thoracic neuroblastoma secondary cardiac tumor case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Introduction Neuroblastoma is an embryonal tumor of the sympathetic nervous system. It most commonly arises from the adrenal glands but can develop in sympathetic chains in other areas of the body. It stands as the most prevalent extracranial solid tumor observed in children and the third most common pediatric malignancy after leukemias and central nervous system tumors in developed countries (7% of childhood cancers) ( 1 ). Thoracic neuroblastomas constitute a minority of all neuroblastoma cases. The prognosis is generally favorable and the overall survival is excellent for patients with low- to intermediate- risk tumors ( 2 ). Cardiac tumors and metastases are extremely rare ( 3 ). To the best of our knowledge, this is the first published case of a mediastinal neuroblastoma extending to the heart in a pediatric population. Case presentation A four-year-old patient presented to the pediatric emergency department of Hassan II Regional Hospital in Agadir with complaints of shortness of breath, chest pain, and fever. He had no significant medical history other than uninvestigated severe failure to thrive and experienced exertional dyspnea in the few months prior to his admission. On physical examination, the patient was conscious and had a low-grade fever of 38,2°C. He was tachycardic at 145 beats per minute and severely dyspneic with a respiratory rate of 54 cycles/minute and central cyanosis. The peripheral capillary saturation was 90% while breathing ambient room air. Thoracic examination revealed a thorax deformity, signs of respiratory distress: suprasternal, intercostal, and subcostal recessions, and diminished vesicular murmurs on the left lung. Cardiovascular examination revealed bilateral distended jugular veins were found and a systolic heart murmur at all the cardiac auscultation sites with no lower limb edema or hepatomegaly. Neurologic examination revealed no abnormalities, with no signs of Horner’s syndrome or nystagmus. All other physical examinations were unremarkable. A chest X-Ray was performed and revealed cardiomegaly and perihilar alveolar edema (bat-wing appearance) ( Fig. 1 ) . Echocardiography found a large atrial septal defect with right heart enlargement and severe pulmonary hypertension (sPap: 90 mmHg). In addition to those findings: an echogenic mobile pedunculated mass in the left atrium of 15mm ( Fig. 2 ) . The electrocardiogram (EKG) findings were sinus tachycardia, right axis deviation, and an S1Q3T3 pattern (the McGinn-White sign). Blood tests were requested and showed neutrophil hyperleukocytosis (23280/µL), elevated C-reactive protein (99mg/dL). Blood cultures were sterile and the serum d-dimer level was high (2320 ng/mL). The patient’s serum electrolyte levels were normal. The patient was admitted to the pediatric department and put on a nasal cannula. Ceftriaxone (50 mg/kg/day) and gentamicin (5mg/kg/day) were introduced due to the initial suspected diagnosis of infective endocarditis. Given the patient’s respiratory distress and pulmonary hypertension, he could not be sedated to undergo a CT pulmonary angiography. Low-molecular-weight heparin was added at 100 U/kg every 12 hours since pulmonary embolism could not be ruled out. The patient exhibited mild improvement in his breathing with persistent signs of respiratory distress but no longer presented with fever. One week later, the follow-up transthoracic echocardiography did not show any modifications: the pedunculated mass was suspected to be a thrombus or an intracardiac tumor. The realization of a chest scanner without sedation presented significant challenges, requiring collaborative efforts from the parents and the radiologists and, more importantly, the cooperation of the patient under strict surveillance from the intensive care team. Computed tomographic pulmonary angiogram (CTPA protocol) revealed a large posterolateral heterogeneous mediastinal mass in the left paravertebral region with central calcification. The adjacent structures were compressed with a mediastinal shift to the right, abutting the thoracic aorta, tracheal carina and esophagus. The intracardiac mass was visualized and exhibited the same characteristics as the mediastinal mass, with no signs of associated pulmonary embolism ( Fig. 3 , 4 , 5 ). Left paravertebral Mediastinal mass, ovoid, molding posteriorly to the posterior chest wall, without any adjacent bone lesions, heterogeneously enhancing after contrast with a central calcified component and hypodense zones,, displacing the mediastinal structures to the right and collapsing most of the left inferior lobe, It measures 97 x 56.8 x 79.4mm (HT x AP x TV) Pediculated round soft tissue lesion in the left atrium (red arrow), appearing in continuity with the mediastinal mass and having the same enhancing after contrast, measuring 14.6 mm in diameter. An ultrasound-guided transthoracic coaxial biopsy was performed by the radiologist, and the sample was sent to the pathologist for cytological assessment, immunohistochemistry, and mutation analyses. The levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine samples, as well as the levels of serum lactate dehydrogenase (LDH) and serum ferritin, were elevated. The alpha-fetoprotein (AFP) and human chorionic gonadotropin (BhCG) levels were normal. Histopathological assessment revealed undifferentiated small round cells compatible with neuroblastoma with a low mitotic index. ( Fig. 6 ) . Spinal cord and cardiac MRI, bone marrow aspiration, and abdominal and pelvic computed tomography were programmed but the patient’s health deteriorated rapidly despite extensive medical intervention. Unfortunately, he died from hypoxemic respiratory failure before further investigations or etiological treatment. Discussion Cardiac masses in children are rare. They are more often benign (fibroma, rhabdomyoma and teratoma) than malignant (sarcomas). Secondary intracardiac tumors are also rare and the primary malignant tumors include sarcomas, lymphomas, and Wilms’ tumor ( 4 ). Neuroblastomas are the third most common pediatric malignancy. Thoracic neuroblastomas (TNBLs) account for a small fraction of all neuroblastomas. Tumors usually develop from the sympathetic chains in the paravertebral area. They constitute close to 15% of childhood cancer fatalities, highlighting their aggressive character ( 5 ). The clinical presentations highly vary. There is a wide range of respiratory or neurologic symptoms, ranging from wheezing and chest pain to ataxia or ptosis, miosis and enophthalmos (the triad of Horner’s syndrome) ( 4 ). In our patient, the symptoms were primarily respiratory with no neurological signs on physical examination. Transthoracic echocardiography revealed a large atrial septal defect, severe pulmonary hypertension and more alarmingly: an echogenic mobile pedunculated mass in the left atrium of 15 mm. Initially, a thrombus or cardiac mass (primarily a myxoma) was suspected. Intracardiac thrombi are the most frequent cardiac tumor mimics. Although rare in children, they can occur in patients with Kawasaki disease, with central veinous lines, or with congenital heart disease. Metastatic tumors, on the other hand, arise more commonly from Wilms’s tumors, sarcomas, and lymphomas via direct extension or hematogenous spread ( 3 ). In 2021, a case-report involving a left atrium metastasis of neuroblastoma (initially thought to be a myxoma) was reported with no known primary site ( 6 ). To the best of our knowledge, no other reports of a mediastinal neuroblastoma extending to the heart by direct extension have been published There are two recognized staging systems for neuroblastomas, the International Neuroblastoma Risk Group Staging System (INRGSS) ( Table 1 ) and the International Neuroblastoma Staging System (INSS). The INSRGSS, which is based on imaging and the INSS uses the results of the resection surgery to determine the cancer stage ( 7 ). Table 1 International Neuroblastoma Risk Group Staging System (INRGSS) ( 7 ) Stage Description L1 Localized tumor not involving vital structures as defined by the list of IDRFs and confined to one body compartment. L2 Locoregional tumor with presence of one or more IDRFs M Distant metastatic disease (except stage MS) MS Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow. The primary tumor can be INSS stage 1, 2, or 3. Our patient’s tumor was staged as L2 (locoregional tumor with the presence of one or more image-defined risk factors). Prognostic factors such as serum lactate dehydrogenase (LDH) and serum ferritin were also elevated, indicating a greater stratified risk ( 8 ). A multidisciplinary meeting was held to determine the treatment plan and schedule the remaining tests, but the patient subsequently died from acute hypoxemic respiratory failure. The prognosis of patients with thoracic neuroblastomas depends on a variety of risk factors such as age at presentation, the tumor stage, histologic appearance, and N-MYC amplification of the tumor. Low-risk patients with localized tumors can undergo complete resection alone with an excellent prognosis. In high-risk tumors, chemotherapy, radiotherapy, and surgery are often needed( 9 ) . Although the cause remains unclear, the prognosis of mediastinal neuroblastomas is reportedly to be better than that of other sites of localized neuroblastomas ( 2 )( 10 ). Conclusion Thoracic neuroblastomas often occur in the posterior mediastinum. They should always be considered as a cause of respiratory or neurological symptoms in children, especially when they are under the age of two. Currently, the intracardiac extension of mediastinal neuroblastomas is an extremely rare occurrence. Further studies and data are needed to improve the understanding of this phenomenon. Declarations Informed consent : Written informed consent was obtained by the patient's parents for the publication of this case report Credit authorship contribution statement I.Ouafik: Writing – review & editing, Writing – original draft, Investigation, Data curation, Conceptualization. S. Tizki: Writing – review & editing, Validation, Supervision, Investigation, Conceptualization. H.Arache: Investigation. S.Safadi : Investigation H. Ouhsousou: Investigation. I.Bazi : Interpreting the scans and writing captions. M.Salek : Performing the biopsy. S.Wakrim : Interpreting the scans and supervising. S.Raqim : performing the echocardiography and interpreting the images. Y.Bouhajeb : Pathology analysis and writing the interpretation. M.Minaoui : Supervising and interpreting the echocardiography findings . H.Nassik : Intensive care and supervision A. Daoudi: Supervision. K. Nainia: Supervision. References Pudela C, Balyasny S, Applebaum MA. Nervous system: Embryonal tumors: Neuroblastoma. Atlas Genet Cytogenet Oncol Haematol. juill 2020;24(7):284‑90. Thoracic neuroblastoma - PMC [Internet]. [cité 30 mars 2024]. Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4901157/ Bussani R, Castrichini M, Restivo L, Fabris E, Porcari A, Ferro F, et al. Cardiac Tumors: Diagnosis, Prognosis, and Treatment. Current Cardiology Reports [Internet]. 2020 [cité 31 mars 2024];22(12). Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7547967/ Bussani R, De-Giorgio F, Abbate A, Silvestri F. Cardiac metastases. J Clin Pathol. janv 2007;60(1):27‑34. Swift CC, Eklund MJ, Kraveka JM, Alazraki AL. Updates in Diagnosis, Management, and Treatment of Neuroblastoma. Radiographics. 2018;38(2):566‑80. Aijaz Z, Durrani HM, Iftikhar P, Khenhrani RR, FaisalUddin M. Metastatic Spread of Neuroblastoma to the Left Atrium Mimicking Atrial Myxoma: A Rare Occurrence in an Adolescent. Cureus [Internet]. janv 2021 [cité 30 mars 2024];13(1). Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7893675/ Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K, et al. The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report. J Clin Oncol. 10 janv 2009;27(2):298‑303. Moroz V, Machin D, Hero B, Ladenstein R, Berthold F, Kao P, et al. The prognostic strength of serum LDH and serum ferritin in children with neuroblastoma: A report from the International Neuroblastoma Risk Group (INRG) project. Pediatr Blood Cancer. août 2020;67(8):e28359. Neuroblastoma: biology, prognosis, and treatment - PubMed [Internet]. [cité 30 mars 2024]. Disponible sur: https://pubmed.ncbi.nlm.nih.gov/18242317/ Häberle B, Hero B, Berthold F, von Schweinitz D. Characteristics and outcome of thoracic neuroblastoma. Eur J Pediatr Surg. juin 2002;12(3):145‑50. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Submission checks completed at journal 08 May, 2024 Editor assigned by journal 08 May, 2024 First submitted to journal 06 May, 2024 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-4379076","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":300698213,"identity":"f3f470d8-f5f5-4231-86b3-073a2e749dae","order_by":0,"name":"I. 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Nainia","email":"","orcid":"","institution":"University ibn Zohr","correspondingAuthor":false,"prefix":"","firstName":"K.","middleName":"","lastName":"Nainia","suffix":""}],"badges":[],"createdAt":"2024-05-06 21:24:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4379076/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4379076/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":56621394,"identity":"877cb715-48c5-468a-8311-a9a52528340a","added_by":"auto","created_at":"2024-05-16 18:07:05","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1067016,"visible":true,"origin":"","legend":"\u003cp\u003eChest X-ray posterior-anterior view showing cardiomegaly, displacement of the mediastinal structures to the right and left mediatinal mass with distinct external limits and blurred internal limits fusing with the heart shadow and left lower lobe collapse.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/215d1f97de526546af44861f.png"},{"id":56622024,"identity":"d066567e-03d6-4405-a3af-28e10820df95","added_by":"auto","created_at":"2024-05-16 18:15:05","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":563838,"visible":true,"origin":"","legend":"\u003cp\u003eTransthoracic echo cardiography revealing a pedunculated echogenic mass (red arrow) a large atrial septal defect with right heart enlargement, severe pulmonary hypertension (sPap: 90mmhg). In addition to those findings: an echogenic mobile pedunculated mass in the left atrium of 15mm\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/d6092a5afcdf3880f6c3fae9.png"},{"id":56621398,"identity":"14b9a402-f2d8-40ec-8d95-b1e25c85047f","added_by":"auto","created_at":"2024-05-16 18:07:05","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":369789,"visible":true,"origin":"","legend":"\u003cp\u003eSagittal(a) pre contrast and sagittal (b) and coronal (c) contrast enhanced chest CT, mediastinal window:\u003c/p\u003e\n\u003cp\u003eLeft paravertebral Mediastinal mass, ovoid, molding posteriorly to the posterior chest wall, without any adjacent bone lesions, heterogeneously enhancing after contrast with a central calcified component and hypodense zones, , displacing the mediastinal structures to the right and collapsing most of the left inferior lobe , It measures 97 x 56.8 x 79.4mm (HT x AP x TV)\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/a76fd7500681692d03a878a6.png"},{"id":56621399,"identity":"da928e78-3c7e-4fea-9c2a-972700157d8b","added_by":"auto","created_at":"2024-05-16 18:07:05","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":409647,"visible":true,"origin":"","legend":"\u003cp\u003eAxial (a) and sagittal (b) post contrast chest CT [ computed tomography pulmonary angiogram 4, 5 and veinous phase]\u003c/p\u003e\n\u003cp\u003ePediculated round soft tissue lesion in the left atrium (red arrow), appearing in continuity with the mediastinal mass and having the same enhancing after contrast, measuring 14.6 mm in diameter.\u003c/p\u003e","description":"","filename":"floatimage4.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/47bc8370f3e60e3d865356e8.png"},{"id":56621397,"identity":"b27c483d-c8bd-4338-86bc-a038d9895c32","added_by":"auto","created_at":"2024-05-16 18:07:05","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":624266,"visible":true,"origin":"","legend":"\u003cp\u003eAxial et coronal chest ct scan, lung windowLeft upper lobe Mosaic attenuation pattern compatible with pulmonary hypertension (a) Left lower lobe collapses due to mass effect (b)\u003c/p\u003e","description":"","filename":"floatimage5.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/79fc3ce56fbed87251bddf8c.png"},{"id":56621395,"identity":"19810ac5-d50e-43ac-9df3-702ef9959c93","added_by":"auto","created_at":"2024-05-16 18:07:05","extension":"png","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":1212971,"visible":true,"origin":"","legend":"\u003cp\u003eProliferation consisting of small to intermediate, undifferentiated cells with very scanty, poorly defined cytoplasm, with rounded, elongated, salt-and-paper nuclei, sometimes nucleolated without a neurofibrillary background. Histological examination shows tumor proliferation separated by fibrous septa. It is made of small to intermediate cells without large atypia and almost without cytoplasm, the nucleus is sometimes hyperchromatic with coarse chromatin and a small nucleolus is often visible. Mitoses are rare.\u003c/p\u003e","description":"","filename":"floatimage6.png","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/2f309cbfec9f3b535607a2c8.png"},{"id":56622314,"identity":"26605ce5-68d4-4819-a278-48553f52a2d3","added_by":"auto","created_at":"2024-05-16 18:23:08","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":6821820,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4379076/v1/66916784-a43c-41c8-be1b-3c1a7caca188.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"A cardiac mass revealing a thoracic neuroblastoma in a four-years- old: a case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eNeuroblastoma is an embryonal tumor of the sympathetic nervous system. It most commonly arises from the adrenal glands but can develop in sympathetic chains in other areas of the body. It stands as the most prevalent extracranial solid tumor observed in children and the third most common pediatric malignancy after leukemias and central nervous system tumors in developed countries (7% of childhood cancers) (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThoracic neuroblastomas constitute a minority of all neuroblastoma cases. The prognosis is generally favorable and the overall survival is excellent for patients with low- to intermediate- risk tumors (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eCardiac tumors and metastases are extremely rare (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). To the best of our knowledge, this is the first published case of a mediastinal neuroblastoma extending to the heart in a pediatric population.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA four-year-old patient presented to the pediatric emergency department of Hassan II Regional Hospital in Agadir with complaints of shortness of breath, chest pain, and fever. He had no significant medical history other than uninvestigated severe failure to thrive and experienced exertional dyspnea in the few months prior to his admission.\u003c/p\u003e \u003cp\u003eOn physical examination, the patient was conscious and had a low-grade fever of 38,2\u0026deg;C. He was tachycardic at 145 beats per minute and severely dyspneic with a respiratory rate of 54 cycles/minute and central cyanosis. The peripheral capillary saturation was 90% while breathing ambient room air.\u003c/p\u003e \u003cp\u003eThoracic examination revealed a thorax deformity, signs of respiratory distress: suprasternal, intercostal, and subcostal recessions, and diminished vesicular murmurs on the left lung. Cardiovascular examination revealed bilateral distended jugular veins were found and a systolic heart murmur at all the cardiac auscultation sites with no lower limb edema or hepatomegaly. Neurologic examination revealed no abnormalities, with no signs of Horner\u0026rsquo;s syndrome or nystagmus. All other physical examinations were unremarkable. A chest X-Ray was performed and revealed cardiomegaly and perihilar alveolar edema (bat-wing appearance) \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eEchocardiography found a large atrial septal defect with right heart enlargement and severe pulmonary hypertension (sPap: 90 mmHg). In addition to those findings: an echogenic mobile pedunculated mass in the left atrium of 15mm \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe electrocardiogram (EKG) findings were sinus tachycardia, right axis deviation, and an S1Q3T3 pattern (the McGinn-White sign). Blood tests were requested and showed neutrophil hyperleukocytosis (23280/\u0026micro;L), elevated C-reactive protein (99mg/dL). Blood cultures were sterile and the serum d-dimer level was high (2320 ng/mL). The patient\u0026rsquo;s serum electrolyte levels were normal. The patient was admitted to the pediatric department and put on a nasal cannula. Ceftriaxone (50 mg/kg/day) and gentamicin (5mg/kg/day) were introduced due to the initial suspected diagnosis of infective endocarditis. Given the patient\u0026rsquo;s respiratory distress and pulmonary hypertension, he could not be sedated to undergo a CT pulmonary angiography. Low-molecular-weight heparin was added at 100 U/kg every 12 hours since pulmonary embolism could not be ruled out. The patient exhibited mild improvement in his breathing with persistent signs of respiratory distress but no longer presented with fever. One week later, the follow-up transthoracic echocardiography did not show any modifications: the pedunculated mass was suspected to be a thrombus or an intracardiac tumor. The realization of a chest scanner without sedation presented significant challenges, requiring collaborative efforts from the parents and the radiologists and, more importantly, the cooperation of the patient under strict surveillance from the intensive care team. Computed tomographic pulmonary angiogram (CTPA protocol) revealed a large posterolateral heterogeneous mediastinal mass in the left paravertebral region with central calcification. The adjacent structures were compressed with a mediastinal shift to the right, abutting the thoracic aorta, tracheal carina and esophagus. The intracardiac mass was visualized and exhibited the same characteristics as the mediastinal mass, with no signs of associated pulmonary embolism \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e,\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e,\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e\u003cb\u003e).\u003c/b\u003e\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eLeft paravertebral Mediastinal mass, ovoid, molding posteriorly to the posterior chest wall, without any adjacent bone lesions, heterogeneously enhancing after contrast with a central calcified component and hypodense zones,, displacing the mediastinal structures to the right and collapsing most of the left inferior lobe, It measures 97 x 56.8 x 79.4mm (HT x AP x TV)\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003ePediculated round soft tissue lesion in the left atrium (red arrow), appearing in continuity with the mediastinal mass and having the same enhancing after contrast, measuring 14.6 mm in diameter.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eAn ultrasound-guided transthoracic coaxial biopsy was performed by the radiologist, and the sample was sent to the pathologist for cytological assessment, immunohistochemistry, and mutation analyses. The levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine samples, as well as the levels of serum lactate dehydrogenase (LDH) and serum ferritin, were elevated. The alpha-fetoprotein (AFP) and human chorionic gonadotropin (BhCG) levels were normal.\u003c/p\u003e \u003cp\u003eHistopathological assessment revealed undifferentiated small round cells compatible with neuroblastoma with a low mitotic index. \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig6\" class=\"InternalRef\"\u003e6\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eSpinal cord and cardiac MRI, bone marrow aspiration, and abdominal and pelvic computed tomography were programmed but the patient\u0026rsquo;s health deteriorated rapidly despite extensive medical intervention. Unfortunately, he died from hypoxemic respiratory failure before further investigations or etiological treatment.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eCardiac masses in children are rare. They are more often benign (fibroma, rhabdomyoma and teratoma) than malignant (sarcomas). Secondary intracardiac tumors are also rare and the primary malignant tumors include sarcomas, lymphomas, and Wilms\u0026rsquo; tumor (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). Neuroblastomas are the third most common pediatric malignancy. Thoracic neuroblastomas (TNBLs) account for a small fraction of all neuroblastomas. Tumors usually develop from the sympathetic chains in the paravertebral area. They constitute close to 15% of childhood cancer fatalities, highlighting their aggressive character (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThe clinical presentations highly vary. There is a wide range of respiratory or neurologic symptoms, ranging from wheezing and chest pain to ataxia or ptosis, miosis and enophthalmos (the triad of Horner\u0026rsquo;s syndrome) (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). In our patient, the symptoms were primarily respiratory with no neurological signs on physical examination. Transthoracic echocardiography revealed a large atrial septal defect, severe pulmonary hypertension and more alarmingly: an echogenic mobile pedunculated mass in the left atrium of 15 mm. Initially, a thrombus or cardiac mass (primarily a myxoma) was suspected.\u003c/p\u003e \u003cp\u003eIntracardiac thrombi are the most frequent cardiac tumor mimics. Although rare in children, they can occur in patients with Kawasaki disease, with central veinous lines, or with congenital heart disease. Metastatic tumors, on the other hand, arise more commonly from Wilms\u0026rsquo;s tumors, sarcomas, and lymphomas via direct extension or hematogenous spread (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eIn 2021, a case-report involving a left atrium metastasis of neuroblastoma (initially thought to be a myxoma) was reported with no known primary site (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). To the best of our knowledge, no other reports of a mediastinal neuroblastoma extending to the heart by direct extension have been published\u003c/p\u003e \u003cp\u003eThere are two recognized staging systems for neuroblastomas, the International Neuroblastoma Risk Group Staging System (INRGSS) \u003cb\u003e(\u003c/b\u003eTable\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e and the International Neuroblastoma Staging System (INSS). The INSRGSS, which is based on imaging and the INSS uses the results of the resection surgery to determine the cancer stage (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eInternational Neuroblastoma Risk Group Staging System (INRGSS) (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e)\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eStage\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDescription\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eL1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLocalized tumor not involving vital structures as defined by the list of IDRFs\u0026nbsp;and confined to one body compartment.\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eL2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLocoregional tumor with presence of one or more IDRFs\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDistant metastatic disease (except stage MS)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMetastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow. The primary tumor can be INSS stage 1, 2, or 3.\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eOur patient\u0026rsquo;s tumor was staged as L2 (locoregional tumor with the presence of one or more image-defined risk factors).\u003c/p\u003e \u003cp\u003ePrognostic factors such as serum lactate dehydrogenase (LDH) and serum ferritin were also elevated, indicating a greater stratified risk (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). A multidisciplinary meeting was held to determine the treatment plan and schedule the remaining tests, but the patient subsequently died from acute hypoxemic respiratory failure.\u003c/p\u003e \u003cp\u003eThe prognosis of patients with thoracic neuroblastomas depends on a variety of risk factors such as age at presentation, the tumor stage, histologic appearance, and N-MYC amplification of the tumor. Low-risk patients with localized tumors can undergo complete resection alone with an excellent prognosis. In high-risk tumors, chemotherapy, radiotherapy, and surgery are often needed(\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e) .\u003c/p\u003e \u003cp\u003eAlthough the cause remains unclear, the prognosis of mediastinal neuroblastomas is reportedly to be better than that of other sites of localized neuroblastomas (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e)(\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e).\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThoracic neuroblastomas often occur in the posterior mediastinum. They should always be considered as a cause of respiratory or neurological symptoms in children, especially when they are under the age of two. Currently, the intracardiac extension of mediastinal neuroblastomas is an extremely rare occurrence. Further studies and data are needed to improve the understanding of this phenomenon.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003e\u003cu\u003eInformed consent :\u0026nbsp;\u003c/u\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained by the patient\u0026apos;s parents for the publication of this case report\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cu\u003eCredit authorship contribution statement\u003c/u\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eI.Ouafik:\u003c/strong\u003e Writing \u0026ndash; review \u0026amp; editing, Writing \u0026ndash; original draft, Investigation, Data curation, Conceptualization. \u003cstrong\u003eS. Tizki:\u003c/strong\u003e Writing \u0026ndash; review \u0026amp; editing, Validation, Supervision, Investigation, Conceptualization. \u003cstrong\u003eH.Arache:\u003c/strong\u003e Investigation. \u003cstrong\u003eS.Safadi\u003c/strong\u003e : Investigation \u003cstrong\u003eH. Ouhsousou:\u003c/strong\u003e Investigation. \u003cstrong\u003eI.Bazi\u003c/strong\u003e : Interpreting the scans and writing captions.\u003cstrong\u003eM.Salek\u003c/strong\u003e : Performing the biopsy. S.Wakrim : Interpreting the scans and supervising. \u003cstrong\u003eS.Raqim :\u003c/strong\u003e performing the echocardiography and interpreting the images. \u003cstrong\u003eY.Bouhajeb\u003c/strong\u003e : Pathology analysis and writing the interpretation. \u003cstrong\u003eM.Minaoui\u003c/strong\u003e : Supervising and interpreting the echocardiography findings\u003cstrong\u003e. H.Nassik\u003c/strong\u003e : Intensive care and supervision \u003cstrong\u003eA. Daoudi:\u003c/strong\u003e Supervision.\u0026nbsp;\u003cstrong\u003eK. Nainia:\u003c/strong\u003e Supervision.\u003c/p\u003e\n"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003ePudela C, Balyasny S, Applebaum MA. Nervous system: Embryonal tumors: Neuroblastoma. Atlas Genet Cytogenet Oncol Haematol. juill 2020;24(7):284‑90. \u003c/li\u003e\n\u003cli\u003eThoracic neuroblastoma - PMC [Internet]. [cit\u0026eacute; 30 mars 2024]. Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4901157/\u003c/li\u003e\n\u003cli\u003eBussani R, Castrichini M, Restivo L, Fabris E, Porcari A, Ferro F, et al. Cardiac Tumors: Diagnosis, Prognosis, and Treatment. Current Cardiology Reports [Internet]. 2020 [cit\u0026eacute; 31 mars 2024];22(12). Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7547967/\u003c/li\u003e\n\u003cli\u003eBussani R, De-Giorgio F, Abbate A, Silvestri F. Cardiac metastases. J Clin Pathol. janv 2007;60(1):27‑34. \u003c/li\u003e\n\u003cli\u003eSwift CC, Eklund MJ, Kraveka JM, Alazraki AL. Updates in Diagnosis, Management, and Treatment of Neuroblastoma. Radiographics. 2018;38(2):566‑80. \u003c/li\u003e\n\u003cli\u003eAijaz Z, Durrani HM, Iftikhar P, Khenhrani RR, FaisalUddin M. Metastatic Spread of Neuroblastoma to the Left Atrium Mimicking Atrial Myxoma: A Rare Occurrence in an Adolescent. Cureus [Internet]. janv 2021 [cit\u0026eacute; 30 mars 2024];13(1). Disponible sur: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7893675/\u003c/li\u003e\n\u003cli\u003eMonclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K, et al. The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report. J Clin Oncol. 10 janv 2009;27(2):298‑303. \u003c/li\u003e\n\u003cli\u003eMoroz V, Machin D, Hero B, Ladenstein R, Berthold F, Kao P, et al. The prognostic strength of serum LDH and serum ferritin in children with neuroblastoma: A report from the International Neuroblastoma Risk Group (INRG) project. Pediatr Blood Cancer. ao\u0026ucirc;t 2020;67(8):e28359. \u003c/li\u003e\n\u003cli\u003eNeuroblastoma: biology, prognosis, and treatment - PubMed [Internet]. [cit\u0026eacute; 30 mars 2024]. Disponible sur: https://pubmed.ncbi.nlm.nih.gov/18242317/\u003c/li\u003e\n\u003cli\u003eH\u0026auml;berle B, Hero B, Berthold F, von Schweinitz D. Characteristics and outcome of thoracic neuroblastoma. Eur J Pediatr Surg. juin 2002;12(3):145‑50. \u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-cardiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pedc","sideBox":"Learn more about [Pediatric Cardiology](http://link.springer.com/journal/246)","snPcode":"246","submissionUrl":"https://submission.nature.com/new-submission/246/3","title":"Pediatric Cardiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Cardiac mass, neuroblastoma, thoracic neuroblastoma, secondary cardiac tumor, case report","lastPublishedDoi":"10.21203/rs.3.rs-4379076/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4379076/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eNeuroblastoma, an embryonal malignant tumor originating from neuroblasts within the sympathetic nervous system, is the third most prevalent pediatric malignancy, following leukemias and central nervous system tumors.\u003c/p\u003e \u003cp\u003eThe primary sites of metastases from neuroblastoma are the bone marrow, bones, and lymph nodes. Cardiac extension is an exceptionally rare occurrence.\u003c/p\u003e \u003cp\u003eWe report the case of a four-year-old child, with no prior medical history of cardiopathy, who had been experiencing exertional dyspnea for a few months leading to his admission. The patient presented to the pediatric emergency department for shortness of breath, chest pain, and fever.The clinical findings were: polypnea, signs of respiratory distress, bilateral jugular vein distention, and a systolic heart murmur at all the cardiac auscultation sites. A chest X-ray showed cardiomegaly and alveolar edema. Echocardiography revealed a large atrial septal defect with right heart enlargement, severe pulmonary hypertension, and an echogenic mobile lesion in the left atrium. Further investigations revealed a large left posterior mediastinal mass with calcifications extending to the left atrium. A biopsy was performed and histopathology of the mediastinal mass revealed neuroblastoma.\u003c/p\u003e \u003cp\u003eThe intracardiac extension of tumors in the pediatric population is rare. Thoracic and cardiac imagery can be used to characterize the mass further and adapt treatment.\u003c/p\u003e \u003cp\u003eTo the best of our knowledge, this is the first published case of a posterior mediastinal neuroblastoma with direct intracardiac extension.\u003c/p\u003e","manuscriptTitle":"A cardiac mass revealing a thoracic neuroblastoma in a four-years- old: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-05-16 18:07:00","doi":"10.21203/rs.3.rs-4379076/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"checksComplete","content":"","date":"2024-05-08T06:19:21+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-05-08T06:19:21+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Cardiology","date":"2024-05-06T21:12:17+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-cardiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pedc","sideBox":"Learn more about [Pediatric Cardiology](http://link.springer.com/journal/246)","snPcode":"246","submissionUrl":"https://submission.nature.com/new-submission/246/3","title":"Pediatric Cardiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"c94ad325-f885-47ea-8cd6-ede5e9a8289d","owner":[],"postedDate":"May 16th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2024-05-16T18:07:00+00:00","versionOfRecord":[],"versionCreatedAt":"2024-05-16 18:07:00","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4379076","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4379076","identity":"rs-4379076","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}