Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-cd9895bd7-hc48f Total loading time: 0 Render date: 2025-01-04T04:06:07.342Z Has data issue: false hasContentIssue false

Chapter 13 - Tumours

Published online by Cambridge University Press:  19 August 2019

Michael T. Ashworth
Michael T. Ashworth
Affiliation:
Great Ormond Street Hospital for Children, London
Get access

Summary

This chapter surveys the entire spectrum of tumours that are described in the fetus, infant and child. The commoner tumours (rhabdomyoma, fibroma, teratoma, myxoma) are discussed in detail, but there is also extensive discussion of rarer tumours such as fatty tumours and vascular tumours and even rarer entities such as juvenile xanthogranuloma or inflammatory myofibroblastic tumour. A brief section is devoted to primary malignant tumours of the heart and to metastatic tumours. Finally, there is discussion of pseudoneoplasms that may be seen in children.

Keywords

Tumourfibromarhabdomyomateratomamyxomacystic tumour atrioventricular nodemetastasisinflammatory myofibroblastic tumour
Type
Chapter
Information
Pathology of Heart Disease in the Fetus, Infant and Child
Autopsy, Surgical and Molecular Pathology
 , pp. 283 - 299
Publisher: Cambridge University Press
Print publication year: 2019

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Uzun, O, Wilson, DG, Vujanic, GM, Parsons, JM, De Giovanni, JV. Cardiac tumours in children. Orphanet J Rare Dis 2007; 2: 11.Google Scholar
Nadas, AS, Ellison, RC. Cardiac tumors in infancy. Am J Cardiol 1968; 21: 363–366.Google Scholar
Burke, A, Virmani, R. Classification and incidence of cardiac tumors. In Burke, A, Virmani, R (eds) Atlas of Tumor Pathology 3rd series, Fascilce 16, Tumors of the Heart and Great Vessels. Washington DC: AFIP; 1995; pp. 1–11.Google Scholar
Isaacs, H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004; 25: 252–273.CrossRefGoogle ScholarPubMed
Sallee, D, Spector, ML, Heerkeren, DW et al. Primary pediatric cardiac tumors: a 17 year experience. Cardiol Young 1999; 9: 155–162.Google Scholar
Beghetti, M, Gow, RM, Haney, I et al. Pediatric benign primary cardiac tumours: a 15-year review. Am Heart J 1997; 134: 1107–1114.Google Scholar
Tworetzky, W, McElhinney, DB, Margossian, R et al. Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol 2003; 92: 487–489.Google Scholar
van Slegtenhorst, M, Nellist, M, Nagelkerken, B et al. Interaction between hamartin and tuberin, the TSC1 and TSC2 gene products. Hum Mol Genet 1998; 7: 1053–1057.Google Scholar
Curatolo, P. Mechanistic target of rapamycin (mTOR) in tuberous sclerosis complex-associated epilepsy. Pediatr Neurol 2015; 52: 281–289.Google Scholar
Choi, JY, Bae, EJ, Noh, CI, Yoon, YS, Hwang, YS. Cardiac rhabdomyomas in childhood tuberous sclerosis. Cardiol Young 1995; 5: 166–171.CrossRefGoogle Scholar
Isaacs, H. Perinatal (fetal and neonatal) tuberous sclerosis: a review. Am J Perinatol 2009; 26: 755–760.CrossRefGoogle ScholarPubMed
Weeks, DA, Chase, DR, Malott, RL et al. HMB-45 staining in angiomyolipoma, cardiac rhabdomoma, other mesenchymal processes, and tuberous sclerosis-associated brain lesions. Int J Surg Pathol 1994; 1: 191–198.Google Scholar
Tehrani, M, Vettraino, IM, Chang, CH. Localized nodular hypertrophy mimicking rhabdomyoma in the fetal heart: prenatal sonographic and pathology findings. Pediatr Devel Pathol 2004; 7: 192–197.Google Scholar
Burke, AP, Rosada-de-Christenson, M, Templeton, PA, Virmani, R. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994; 108: 862–870.Google Scholar
Coffin, CM. Congenital cardiac fibroma associated with Gorlin Syndrome. Pediatr Pathol 1992; 12: 255–262.Google Scholar
Farndon, PA, Del Mastro, RG, Evans, DG, Kilpatrick, MW. Location of gene for Gorlin syndrome. Lancet 1992; 339: 581–582.Google Scholar
Scanlan, D, Radio, SJ, Nelson, M et al. Loss of the PTCH1 gene locus in cardiac fibroma. Cardiovasc Pathol 2008; 17: 93–97.Google Scholar
Becker, AE. Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 2000; 21: 317–323.CrossRefGoogle ScholarPubMed
Freedom, RM, Lee, KJ, McDonald, C, Taylor, G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol 2000; 21: 299–316.Google Scholar
de Montpréville, VT, Serraf, A, Aznag, H et al. Fibroma and inflammatory myofibroblastic tumor of the heart. Ann Diagn Pathol 2001; 5: 335–342.Google Scholar
Heerema-McKenney, A, Harrison, MR, Bratton, B et al. Congenital teratoma: a clinicopathologic study of 22 fetal and neonatal tumors. Am J Surg Pathol 2005; 29: 29–38.CrossRefGoogle ScholarPubMed
Kahlbau, H, Gomes, I, Pinto, F, Fragata, JIG. Uncommon multicystic lesion of the interventricular septum in a 7-year-old boy: unusual presentation of an intracardiac teratoma. Ann Thorac Surg 2016; 101: 2375–2377.Google Scholar
Rychik, J, Khalek, N, Gaynor, JW et al. Fetal intrapericardial teratoma: natural history and management including successful in utero surgery. Am J Obstet Gynecol 2016; 215: 780e1–780e7.Google Scholar
Cetrano, E, Polito, A, Carotti, A. Primitive intrapericardial teratoma associated with yolk sac tumour. Cardiol Young 2015; 25: 158–160.Google Scholar
Paladini, D, Tartaglione, A, Vassallo, M, Martinelli, P. Prenatal ultrasonographic findings of a cardiac myxoma. Obstet Gynecol 2003; 102: 1174–1176.Google Scholar
Amano, J, Kono, T, Wada, Y et al. Cardiac myxoma: its origin and tumor characteristics. Ann Thorac Cardiovasc Surg 2003; 9: 215–221.Google Scholar
DiVito, A, Mignogna, C, Donato, G. The mysterious pathways of cardiac myxomas: a review of histogenesis, pathogenesis and pathology. Histopathology 2015; 66: 321–332.CrossRefGoogle Scholar
Terracciano, LM, Mhawech, P, Suess, K et al. Calretinin as a marker for cardiac myxoma. Diagnostic and histogenetic considerations. Am J Clin Pathol 2000; 114: 754–759.Google Scholar
Wassef, M, Blei, F, Adams, D et al.; ISSVA Board and Scientific Committee. Vascular Anomalies Classification: Recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015; 136: e203–214.Google Scholar
Mackie, AS, Kozakewich, HP, Geva, T, Perez-Atayde, AR, Mulliken, JB. Vascular tumors of the heart in infants and children: case series and review of the literature. Pediatr Cardiol 2005; 26: 344–349.Google Scholar
Burke, A, Johns, JP, Virmani, R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol 1990; 3: 283–290.Google Scholar
Burke, A, Tavora, F. Miscellaneous benign cardiac tumors and tumor-like lesions of the heart. In Burke, A, Tavora, F (eds) Practical Cardiovascular Pathology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2011, pp. 390–397.Google Scholar
Beaton, A, Kuttler, T, Hassan, A et al. Hemangioendothelioma: a rare case of a primary intracardiac tumor. Pediatr Cardiol 2013; 34: 194–197.CrossRefGoogle ScholarPubMed
Li, W, Teng, P, Xu, H, Ma, L, Ni, Y. Cardiac hemangioma: a comprehensive analysis of 200 cases. Ann Thorac Surg 2015; 99: 2246–2252.CrossRefGoogle Scholar
Dasgupta, R, Fishman, SJ. ISSVA classification. Semin Pediatr Surg 2014; 23: 158–161.Google Scholar
Cook, AL, Williams, DA, Bergman, S, Hines, MH. Atypically located cardiac haemangioma of the mitral valve. Cardiol Young 2011; 21: 598–600.Google Scholar
Daubeney, PE, Ogilvie, BC, Moore, IE, Webber, SA. Intrapericardial lymphangioma presenting as neonatal cardiac tamponade. Pediatr Cardiol 1996; 17: 129–131.CrossRefGoogle ScholarPubMed
Cameselle-Teijeiro, J, Abdulkader, I, Soares, P et al. Cystic tumor of the atrioventricular node of the heart appears to be the heart equivalent of the solid cell nests (ultimobranchial rests) of the thyroid. Am J Clin Pathol 2005; 123: 369–375.CrossRefGoogle ScholarPubMed
Evans, CA, Suvarna, SK. Cystic atrioventricular node tumour: not a mesothelioma. J Clin Pathol 2005; 58: 1232.Google Scholar
Ali, SZ, Susin, M, Kahn, E, Hajdu, SI. Intracardiac teratoma in a child simulating an atrioventricular nodal tumor. Pediatr Pathol 1994; 14: 913–917.Google Scholar
Coffin, CM, Hornick, JL, Fletcher, CD. Inflammatory myofibroblastic tumour: comparison of clinicopathologic, histologic and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 2007; 31: 509–520.Google Scholar
Eilers, AL, Nazarullah, AN, Shipper, ES et al. Cardiac inflammatory myofibroblastic tumor: a comprehensive review of the literature. World J Pediatr Congenit Heart Surg 2014; 5: 556–564.CrossRefGoogle ScholarPubMed
Xu, B, Fraser, RS, Renaud, C et al. Inflammatory myofibroblastic tumor of the aortic valves causing sudden cardiac death: a case report and review of the literature. Pediatr Dev Pathol 2014; 17: 231–239.CrossRefGoogle ScholarPubMed
Sebire, NJ, Ramsay, A, Sheppard, M, et al. Intravascular inflammatory myofibroblastic tumours of infancy. Pediatr Dev Pathol 2002; 5: 400–404.Google Scholar
Kobayashi, D, Delius, RE, Debelenko, LV, Aggarwal, S. Cardiac juvenile xanthogranuloma in an infant presenting with pericardial effusion. Congenit Heart Dis 2013; 8: E106–110.CrossRefGoogle Scholar
Vadmal, MS, Hajdu, SI. Lipoma of the mitral valve in a child. Ann Clin Lab Sci 1998; 28: 242–245.Google Scholar
Barberger-Gateau, P, Paquet, M, Desaulniers, D, Chenard, J. Fibrolipoma of the mitral valve in a child. Clinical and echocardiographic features. Circulation 1978; 58: 955–958.Google Scholar
Benhan, R, Williams, G, Gerlis, L, Scott, O. Lipoma of the mitral valve and papillary muscle. Am J Cardiol 1983; 51: 1459–1460.Google Scholar
Harada, K, Seki, I, Kobayashi, H, Okuni, M, Sakurai, I. Lipoma of the heart in a child. Clinical, echocardiographic, angiographic, and pathological features. Jpn Heart J 1980; 21: 903–910.Google Scholar
Gan, C, An, Q, Tao, K, Tang, H, Li, W. An asymptomatic lipoma of the right atrium in a neonate. J Pediatr Surg 2008; 43: 1920–1922.Google Scholar
Dishop, MK, O’Connor, WN, Abraham, S, Cottrill, CM. Primary cardiac lipoblastoma. Pediatr Dev Pathol 2001; 4: 276–280.CrossRefGoogle ScholarPubMed
Heifetz, SA, Parikh, SR, Brown, JW. Hibernoma of the pericardium presenting as pericardial effusion in a child. Pediatr Pathol 1990; 10: 575–580.CrossRefGoogle ScholarPubMed
Miyake, CY, Del Nido, PJ, Alexander, ME et al. Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia. J Am Coll Cardiol 2011; 58: 1903–1909.Google Scholar
Sharma, P, Shakya, U, Sayami, G, Rajbanshi, BG. Lipoblastoma: an unusual tumour of the left ventricle. Eur J Cardiothorac Surg 2016; 49: e147–148.Google Scholar
Bois, MC, Bois, JP, Anavekar, NS, Oliveira, AM, Maleszewski, JJ. Benign lipomatous masses of the heart: a comprehensive series of 47 cases with cytogenetic evaluation. Hum Pathol 2014; 45: 1859–1865.Google Scholar
Davis, JS, Allan, BJ, Perez, EA, Neville, HL, Sola, JE. Primary pediatric cardiac malignancies: the SEER experience. Pediatr Surg Int 2013; 29: 425–429.Google Scholar
Simpson, L, Kumar, SK, Okuno, SH et al. Malignant primary cardiac tumors: review of a single institution experience. Cancer 2008; 112: 2440–2446.CrossRefGoogle ScholarPubMed
Schaffer, LR, Caltharp, SA, Milla, SS et al. Rare presentation of four primary pediatric cardiac tumors. Cardiovasc Pathol 2016; 25: 72–77.Google Scholar
Zhang, PJ, Brooks, JS, Goldblum, JR et al. Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol 2008; 39: 1385–1395.Google Scholar
Prifti, E, Veshti, A, Ikonomi, M, Demiraj, A. Primary cardiac synovial sarcoma originating from the mitral valve causing left ventricular outflow tract obstruction. World J Pediatr Congenit Heart Surg 2015; 6: 650–653.Google Scholar
Eisenstat, J, Gilson, T, Reimann, J, Sampson, B. Low-grade myofibroblastic sarcoma of the heart causing sudden death. Cardiovasc Pathol 2008; 17: 55–59.Google Scholar
McElhinney, DB, Carpentieri, DF, Bridges, ND et al. Sarcoma of the mitral valve causing coronary arterial occlusion in children. Cardiol Young 2001; 11: 539–542.Google Scholar
Bussani, R, De-Giorgio, F, Abbate, A, Silvestri, F. Cardiac metastases. J Clin Pathol 2006; 60: 27–34.CrossRefGoogle ScholarPubMed
Chan, HS, Sonley, MJ, Moës, CA et al. Primary and secondary tumors of childhood involving the heart, pericardium, and great vessels. A report of 75 cases and review of the literature. Cancer 1985; 56: 825–836.Google Scholar
Ritchey, ML, Kelalis, PP, Breslow, N et al. Intracaval and atrial involvement with nephroblastoma: review of National Wilms Tumor Study-3. J Urol 1988; 140: 1113–1118.Google Scholar
Miller, DV, Tazelaar, HD. Cardiovascular pseudoneoplasms. Arch Pathol Lab Med 2010; 134: 362–368.CrossRefGoogle ScholarPubMed
Fealey, ME, Edwards, WD, Miller, DV, Menon, SC, Dearani, JA. Hamartomas of mature cardiac myocytes: report of seven new cases and review of the literature. Hum Pathol 2008; 39: 1064–1071.Google Scholar
Raffa, GM, Malvindi, PG, Settepani, F et al. Hamartoma of mature cardiac myocytes in adults and young: case report and literature review. Int J Cardiol 2013; 163: e28–30.Google Scholar
Reynolds, C, Tazelaar, HD, Edwards, WD. Calcified amorphous tumour of the heart. Hum Pathol 1997; 28: 601–606.Google Scholar
de Hemptinnea, Q, de Cannièreb, D, Vandenbosschea, JL, Ungera, P. Cardiac calcified amorphous tumor: A systematic review of the literature. Int J Cardiol Heart Vasc 2015; 7: 1–5.Google Scholar
Choi, EK, Ro, JY, Ayala, AG. Calcified amorphous tumor of the heart: case report and review of the literature. Methodist Debakey Cardiovasc J 2014; 10: 38–40.CrossRefGoogle ScholarPubMed
Veinot, JP, Tazelaar, HD, Edwards, WD, Colby, TV. Mesothelial/monocytic incidental cardiac excrescences: cardiac MICE. Mod Pathol 1994; 7: 9–16.Google Scholar
Hu, ZL, Lü, H, Yin, HL, Wen, JF, Jin, O. A case of mesothelial/monocytic incidental cardiac excrescence and literature review. Diagn Pathol 2010; 5: 40.Google Scholar
Jiao, N, Zhang, W, Wang, W et al. Mesothelial/monocytic incidental cardiac excrescence: a case report and review of literature. Int J Clin Exp Pathol 2014; 7: 6219–6224.Google Scholar
Courtice, RW, Stinson, WA, Walley, VM. Tissue fragments recovered at cardiac surgery masquerading as tumoral proliferations. Evidence suggesting iatrogenic or artefactual origin and common occurrence. Am J Surg Pathol 1994; 18: 167–174.Google Scholar
Ton, O, Polat, N, Mansuroglu, D, Kabukcuoglu, F. Mesothelial/monocytic incidental cardiac excrescence in a patient with antiphospholipid syndrome. Interact Cardiovasc Thorac Surg 2011; 13: 657–659.Google Scholar
Luthringer, DJ, Virmani, R, Weiss, SW, Rosai, J. A distinctive cardiovascular lesion resembling histiocytoid (epithelioid) hemangioma. Evidence suggesting mesothelial participation. Am J Surg Pathol 1990; 14: 993–1000.Google Scholar
Chikkamuniyappa, S, Herrick, J, Jagirdar, JS. Nodular histiocytic/mesothelial hyperplasia: a potential pitfall. Ann Diagn Pathol 2004; 8: 115–120.Google Scholar
Gowda, RM, Khan, IA, Nair, CK et al. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003; 146: 404–410.Google Scholar
Xu, BJ, Xie, XY, Tan, LH, Shu, Q. Papillary fibroelastoma of the tricuspid valve in a 1-mo-old child. Indian J Pediatr 2013; 80: 353–354.Google Scholar
Sun, JP, Asher, CR, Yang, XS et al.. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001; 103: 2687–2693.Google Scholar
Karimi, M, Vining, M, Pellenberg, R, Jajosky, R. Papillary fibroelastoma of tricuspid valve in a pediatric patient. Ann Thorac Surg 2013; 96: 1078–1080.Google Scholar
Deodhar, AP, Tometzki, AJ, Hudson, IN, Mankad, PS. Aortic valve tumor causing acute myocardial infarction in a child. Ann Thorac Surg 1997; 64: 1482–1484.Google Scholar
Somers, GR, Smith, CR, Perrin, DG, Wilson, GJ, Taylor, GP. Sudden unexpected death in infancy and childhood due to undiagnosed neoplasia: an autopsy study. Am J Forensic Med Pathol 2006; 27: 64–69.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

  • Tumours
  • Michael T. Ashworth
  • Book: Pathology of Heart Disease in the Fetus, Infant and Child
  • Online publication: 19 August 2019
  • Chapter DOI: https://doi.org/10.1017/9781316337073.013
Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

  • Tumours
  • Michael T. Ashworth
  • Book: Pathology of Heart Disease in the Fetus, Infant and Child
  • Online publication: 19 August 2019
  • Chapter DOI: https://doi.org/10.1017/9781316337073.013
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Tumours
  • Michael T. Ashworth
  • Book: Pathology of Heart Disease in the Fetus, Infant and Child
  • Online publication: 19 August 2019
  • Chapter DOI: https://doi.org/10.1017/9781316337073.013
Available formats
×