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A Rare Case of Intracardiac Extension of Hepatocellular Carcinoma in a Child

A Rare Case of Intracardiac Extension of Hepatocellular Carcinoma in a Child Hindawi Case Reports in Oncological Medicine Volume 2019, Article ID 4783595, 5 pages https://doi.org/10.1155/2019/4783595 Case Report A Rare Case of Intracardiac Extension of Hepatocellular Carcinoma in a Child 1 1 2 Abdulrahman Al Jassmi , Hani Humad, and Said Abou Eida Department of Pediatric Hematology/Oncology, Dubai Hospital, PO Box 92227 Dubai, UAE Department of Pediatrics, Welcare Hospital, Dubai, UAE Correspondence should be addressed to Abdulrahman Al Jassmi; amaljassmi@dha.gov.ae Received 2 April 2019; Accepted 6 September 2019; Published 5 December 2019 Academic Editor: Raffaele Palmirotta Copyright © 2019 Abdulrahman Al Jassmi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hepatocellular carcinoma (HCC) is the fifth most common malignancy found in men and ninth most common in women, out of which 72.5% reported cases are from Asia. In children, it accounts for <2% cases worldwide with even rarer incidence of 1.2% involving intracardial extension. However, it presents with a high mortality rate with mean survival ranging from 1 to 4 months. The present case is an extremely rare case of intracardiac extension of HCC in a 3.5-year-old Asian girl with no history of hepatitis B infection presented at an advanced stage of HCC who succumbed within one month of presentation to the hospital. 1. Introduction incidence rate of 1.2% [9]. In cardiac metastasis, HCC leads to intracavitary involvement of the right atrium through Historically, first being described in the eighteenth century direct tumor invasion of the inferior vena cava (IVC) causing by Gaspard-Laurent Bayle, hepatocellular carcinoma (HCC) cardiac symptoms [9, 10]. is now the most common malignant liver tumor following However, this report is an extremely rare case of intracar- hepatoblastoma [1, 2]. Worldwide, it is the third leading diac extension of HCC in a girl with 80% involvement of the cause of cancer-related mortality accounting for 782,000 right atrium and extension into the right ventricle too, with- (95%) deaths out of 782,000 people in 2012 and 781,631 out any cardiac symptoms at the time of presentation. (93%) out of 841,080 people in 2018. It is the fifth most com- mon malignancy found in men and ninth most common in 2. Case Report women out of which 72.5% cases are reported from Asia [2, 3]. Although it is quite rare in children, accounting At the end of November 2012, a 3.5-year-old child presented for less than 2% cases worldwide, it presents with high mor- to the pediatric department with a progressive abdominal tality with nearly 80% deaths within the first year of diagnosis swelling for the past two and a half months. Earlier, she [1, 4–7]. In most cases, the diagnosis of HCC is made at an was seen in Bangladesh, where antibiotics were prescribed advanced stage with the usual sites of metastases being the yet she did not take them. Initially, she had an erythematous lungs, lymph nodes, and bones [8]. rash that subsided but had no other symptoms of vomiting, Generally, the incidence of cardiac metastasis ranges diarrhea, constipation, or difficulty in breathing. On physical from 2.3 to 18.3% with a greater propensity of metastases examination, the girl has a thin build with an abdominal after the fifth decade of life; the most common secondary car- mass extending from the right hypochondrium downward diac tumors are melanoma, mediastinal primary tumors, (8-10 cm) crossing the midline, which had a lobular irregular mesotheliomas, lung carcinomas, breast carcinoma, and surface; the upper border of the mass was nonpalpable, and renal carcinoma. Albeit found in close proximity to the heart, no bruits were present. Chest, cardiovascular, and central the intracardiac extension of HCC is extremely rare with an nervous system examinations were normal along with the 2 Case Reports in Oncological Medicine Table 1: The results of the laboratory investigations of the patient at different intervals. Tests Normal range Result 1 Results 2 Results 3 ∗ ∗ ∗ Hemoglobin (g/dL) 11-13 15.7 16.4 14 White blood cells (×10 /μL) 5.0-15.0 14.2 11 14.9 3 ∗ ∗ 523 489 Platelets (×10 /μL) 150-450 290 ∗ ∗ Albumin (g/dL) 3.8-5.4 3.9 3.6 2.5 ∗ ∗ 48 1573 Alanine aminotransferase (U/L) 0-33 29 Aspartate aminotransferase (U/L) <281 242 199 HIV AG/AB Negative Negative —— >30,000 Alpha-fetoprotein (ng/mL) 0.5-5.5 —— Calcium oxalate crystals: 3+ Urinalysis White blood cells: 0-5 absence of pallor, jaundice, and lymphadenopathy. No signs of edema were noted. The child was admitted a couple of times for the evalua- tion of the mass, performing investigations and reaching a final diagnosis. Meanwhile, she was treated symptomatically in the ICU with intravenous hydration and maintaining con- stant oxygenation. She was followed up conservatively because of the end-staged metastatic HCC and the increased risks of heart surgery-attributable mortality. During this period, she was referred to other centers for investigations, including chest X-ray and angiography and transesophageal echocardiography (TEE) to get insights into not only the location of the mass in relation to the atrial wall or the tricus- pid valve but also its position with respect to the superior and Figure 1: Chest X-ray showing an abdominal mass. inferior vena cava. However, TEE was not performed due to financial constraints. The patient’s conditions worsened and she developed fever, periorbital edema, sacral edema, and of the heart in close contact with IVC. Vessels were pedal edema. Her edema worsened, and later she developed stretched rather than compromised or invaded, and portal difficulty in breathing and had to be put on artificial oxygen and hepatic veins were patent. Chest CT showed slight to maintain saturation. right-sided pleural effusion filling the lateral and posterior Three months later, cardiac auscultation revealed a sinus costophrenic sulci along with mild pleural effusion on the left side too. A large exophytic mass was noted projecting, heart rhythm and 2/6 (quiet) systolic murmur. Additionally, a bilateral pitting edema was noticed in the lower extremities. as an extension from the extensive liver lesion, into the right Laboratory investigations showed abnormally elevated atrium. Bone scintigraphy was unremarkable with no evi- hemoglobin, platelets, albumin, and hepatic enzymes dence of a metastatic lesion. (Table 1). Additionally, chest X-ray showed an ill-defined Liver biopsy was performed at our institution, and the histopathological report was consistent with a malignant infiltrate in the right lower zone with bilateral bronchial wall thickening (Figure 1). Notably, there were no lung metasta- tumor (Figure 2). Immunohistochemistry findings favored ses. On abdominal ultrasonography, the liver was enlarged the diagnosis of a moderately differentiated HCC. In line with nodular heterogeneous echotexture and multiple echo- with the findings of the physical examination, imaging stud- genic patches without biliary dilatation. ies, and histopathological analysis, the patient was finally diagnosed with HCC, which had extended through the infe- A 2D transthoracic echocardiogram demonstrated a large mass in the right atrium extending to the tricuspid valve rior vena cava to the right atrium, occupying 80% of the right without significant obstruction. Computed tomography atrium with an extension to the right ventricle. The Pretreat- (CT) of the abdomen showed a large 9:5×11:5× 13:5cm ment Extension of Disease (PRETEXT) system was used for lobulated, mixed complex solid-cystic mass lesion, which staging, and the tumor was staged as PRETEXT IV, V3. The patient was readmitted for further management after was a predominantly hypodense solid lesion with heteroge- nous cystic components and intervening thick septa occupy- reaching the final diagnosis of HCC. Her condition was ing the left lobe of the liver and a large part of the right lobe reviewed by a pediatric oncology team and cardiothoracic (segments 5 and 6) and caudate lobe. It extended superiorly surgeons, and she was started on chemotherapy with a plan above the diaphragm as a well-demarcated 2:5×3cm mass to resect the mass extending to the right atrium. Neverthe- less, the patient could not sustain chemotherapy owing to at the cardiophrenic angle dipping into the right atrium Case Reports in Oncological Medicine 3 Figure 2: A biopsy showing frequent mitotic figures, pleomorphism, and vesicular nuclei with ill-defined infiltrate in the right lower zone. her comorbidities. Unfortunately, the girl died of cardiore- 18 cases of right atrium extension of tumor and 5 cases of spiratory arrest at the operation table during the induction extension into the right ventricle [20]. HCC has a greater of anesthesia at the end of December 2012. CPR measures propensity for vascular invasion with portal vein thrombosis were undertaken to resuscitate the child, but the child suc- being the most common accounting for 20-65% cases, cumbed to inflow and outflow blockage of the heart. followed by systemic venous thromboembolism at 6% and hepatic vein thrombosis at 1.4-4.9%. However, the tumor thrombus invasion of the inferior vena cava is quite rare 3. Discussion accounting for 0.67-3% of all cases [21]. HCC stages as an abdominal swelling along with discomfort Cardiac metastases occur either via direct extension of a owing to its large size, which is comparable to this case [4]. It tumor thrombus through IVC invasion or through the bloodstream or the lymphatic system; the most common is responsible for 23% of all primary pediatric hepatic malig- nancies with an annual incidence of 0.15 per 100,000 (1975- pathway for HCC is IVC [9, 16]. Isolated cardiac metastasis 1995). Overwhelmingly, 75% of children over the age of 10 of HCC is extremely rare, and a literature search shows 17 years are affected; however, it is quite rare in children < 5 such cases, none of which were pediatric patients [16, 22]. years of age, accounting for <10% cases [4, 7]. In contrast The frequent location of an intracardiac primary or met- astatic tumor is usually the left ventricle and left atrium, with to adults, where HCC occurs predominantly on the back- ground of cirrhotic liver, HCC presents in normal livers in the right ventricle and right atrium being less common. Right the majority (70%) of the pediatric population [11]. Gener- atrium tumors may obstruct the orifice or damage the tricus- ally, these children are positive for hepatitis B serum antigen pid valve leading to tricuspid stenosis or regurgitation caus- (HBsAg), which is in contrast to this case [6]. Other etiolog- ing right heart failure with resulting signs and symptoms of dyspnea and lower extremity edema [23]. Great heterogene- ical factors related to HCC in children constitute congenital and metabolic conditions including biliary atresia, hereditary ity is seen in patients with intracardiac extension of HCC, tyrosinemia type 1, congenital hepatic fibrosis, and progres- from being asymptomatic to heart failure, sudden cardiac sive familial intrahepatic cholestasis [7]. arrest, Budd-Chiari syndrome, and pulmonary embolism In the orient and sub-Saharan African regions, about [24]. The reported patient had no cardiac symptoms at the time of presentation, until at the later stage she developed 75-92% reported cases are positive for serum alpha- fetoprotein level (sAFP). AFP levels are of prognostic value fever and periorbital, sacral, and pedal edema along with dif- as elevated AFP titers are associated with 1.8 times higher ficulty in breathing. The prognosis for intracardiac metastasis mortality [6]. HCC also carries poor prognosis owing to its of HCC is dismal with mean survival ranging from 1 to 4 chemoresistant nature with an estimated cure rate of only months, irrespective of treatment [12, 25]. Cardiac metastasis is best appreciated in transesophageal 25-30% [4, 5]. Advanced stages of HCC have a median sur- vival rate of 4-7 months [12]. Tumor resection is the sole echocardiography (TEE), transthoracic echocardiography curative treatment, and it can be performed either via partial (TTE) along with CT scan, and MRI, which form the main- liver resection or hepatic transplantation. In the recent stud- stay for diagnosing HCC metastasis to the heart. These ies, the overall five-year survival rate ranges between 50% help in identifying the extension of the tumor to the heart, right ventricular outflow obstruction, pulmonary thrombo- and 60% following surgical resection, yet it reaches only 20-30% in pediatric patients with advanced disease at diag- embolism, and pericardial effusion [18]. The only effective nosis who received a combination of surgical resection and treatment option includes surgical extraction of the throm- chemotherapy [13–15]. bus along with tumor resection, although patient manage- Unfortunately, a very large number of pediatric patients ment with advanced HCC with intracardiac extension is difficult and extremely risky. Other management options have advanced or metastatic HCC at the time of diagnosis, with the lung being the most common site for extrahepatic include transarterial chemoembolization (TACE), ablation, metastasis [7, 8]. However, an extension of HCC into the radiation, and chemotherapy [19, 24, 26]. Although the right atrium is possible, albeit it is extremely rare with studies patient in the present report was opted for surgical exci- indicating 1.2-4.1% of extrahepatic cardiac metastasis sion of the tumor, the patient succumbed to inflow and outflow blockage of the heart during anesthesia initiation [16–19]. An autopsy case study of 439 HCC cases showed 4 Case Reports in Oncological Medicine at the operation table. She died in <1 month after being [12] M. Salehi, T. Yee, E. Alatevi, and Y. Thein, “Clinically silent intracardiac metastasis with extremely poor progno- presented to the hospital. sis in a patient with hepatocellular carcinoma,” Case HCC with cardiac metastasis has very poor prognosis, Reports in Gastroenterology, vol. 11, no. 2, pp. 416–421, more so, in pediatric patients. The present case is an extremely rare case of hepatocellular carcinoma extension [13] J. P. McAteer, A. B. Goldin, P. J. Healey, and K. W. Gow, “Sur- to the right atrium along with extension to the right ventricle gical treatment of primary liver tumors in children: outcomes in a 3.5-year-old girl. This is the first literature review involv- analysis of resection and transplantation in the SEER data- ing a child with intracardiac extension of HCC. Awareness in base,” Pediatric Transplantation, vol. 17, no. 8, pp. 744–750, clinicians about such rare cases will affect the overall diagno- sis and management of patients. [14] M. Murawski, V. B. Weeda, R. Maibach et al., “Hepatocellular carcinoma in children: does modified platinum-and doxorubicin-based chemotherapy increase tumor resectability Conflicts of Interest and change outcome? Lessons learned from the SIOPEL 2 and 3 studies,” Journal of Clinical Oncology, vol. 34, no. 10, The authors declare that there is no conflict of interest pp. 1050–1056, 2016. regarding the publication of this paper. [15] J. de Ville de Goyet, R. L. Meyers, G. M. Tiao, and B. Morland, “Beyond the Milan criteria for liver transplantation in children References with hepatic tumours,” The Lancet Gastroenterology & Hepa- tology, vol. 2, no. 6, pp. 456–462, 2017. [1] P. R. Exelby, R. M. Filler, and J. L. Grosfeld, “Liver tumors in [16] M. Kawakami, M. Koda, M. Mandai et al., “Isolated metastases children in the particular reference to hepatoblastoma and of hepatocellular carcinoma in the right atrium: case report hepatocellular carcinoma: American Academy of Pediatrics and review of the literature,” Oncology Letters, vol. 5, no. 5, Surgical Section Survey – 1974,” Journal of Pediatric Surgery, pp. 1505–1508, 2013. vol. 10, no. 3, pp. 329–337, 1975. [17] M. Barrett, L. Viglianti Benjamin, A. Hanson Christopher, and [2] A. Reuben, “Hepatocellular carcinoma in adults and chil- R. J. Schildhouse, “A case of right atrial obliteration caused by dren,” Clinics in Liver Disease, vol. 19, no. 2, pp. xiii–xxvi, intracardiac extension of hepatocellular carcinoma,” Case Reports in Oncology, vol. 10, no. 1, pp. 8–14, 2017. [3] International Agency for Research on Cancer, GLOBOCAN [18] Y.-C. Liu, Y.-L. Ho, G.-T. Huang, D.-S. Chen, J.-C. Sheu, and 2012: estimated cancer incidence, mortality and prevalence C.-H. Chen, “Clinical manifestations and survival of patients worldwide in 2012 v1.0, IARC, 2012, July 2019, https:// with hepatocellular carcinoma and cardiac metastasis,” Jour- publications.iarc.fr/Databases/Iarc-Cancerbases/ nal of Gastroenterology and Hepatology, vol. 25, no. 1, GLOBOCAN-2012-Estimated-Cancer-Incidence-Mortality- pp. 150–155, 2010. And-Prevalence-Worldwide-In-2012-V1.0-2012. [19] W. Li, Y. Wang, W. Gao, and J. Zheng, “HCC with tumor [4] B. J. Allan, B. Wang, J. S. Davis et al., “A review of 218 pediatric thrombus entering the right atrium and inferior vena cava cases of hepatocellular carcinoma,” Journal of Pediatric Sur- treated by percutaneous ablation,” BMC Surgery, vol. 17, gery, vol. 49, no. 1, pp. 166–171, 2014. no. 1, article 21, 2017. [5] R. Pazdur, B. Bready, and A. Cangir, “Pediatric hepatic tumors: [20] M. Kojiro, H. Nakahara, S. Sugihara, T. Murakami, clinical trials conducted in the United States,” Journal of Surgi- T. Nakashima, and H. Kawasaki, “Hepatocellular carcinoma cal Oncology, vol. 53, no. S3, pp. 127–130, 1993. with intra-atrial tumor growth. A clinicopathologic study of [6] S. W. Moore, P. B. Hesseling, G. Wessels, and J. W. Schneider, 18 autopsy cases,” Archives of Pathology & Laboratory Medi- “Hepatocellular carcinoma in children,” Pediatric Surgery cine, vol. 108, no. 12, pp. 989–992, 1984. International, vol. 12, no. 4, pp. 266–270, 1997. [21] J. Huang, Z.-Y. Pan, L. Li et al., “Hepatocellular carcinoma [7] K. Palaniappan, V. V. Borkar, M. Safwan et al., “Pediatric with inferior vena caval and right atrial tumor thrombi hepatocellular carcinoma in a developing country: is the etiol- and massive pulmonary artery embolism: a case report,” ogy changing?,” Pediatric Transplantation, vol. 20, no. 7, Molecular and Clinical Oncology, vol. 6, no. 1, pp. 111–114, pp. 898–903, 2016. [8] T. Terada and H. Maruo, “Unusual extrahepatic metastatic [22] E. Tastekin, U. Usta, T. Ege, G. Kazindir, and A. K. Kutlu, sites from hepatocellular carcinoma,” International Journal of “Cardiac metastasis of hepatocellular carcinoma in a young Clinical and Experimental Pathology, vol. 6, no. 5, pp. 816– non-cirrhotic patient, to the left ventricle,” Annals of hepatol- 820, 2013. ogy, vol. 11, no. 3, pp. 392–394, 2012. [9] R. Bussani, F. De-Giorgio, A. Abbate, and F. Silvestri, “Cardiac metastases,” Journal of Clinical Pathology, vol. 60, no. 1, [23] I. P. Panidis, M. N. Kotler, G. S. Mintz, and J. Ross, “Clinical and echocardiographic features of right atrial pp. 27–34, 2007. masses,” American Heart Journal, vol. 107, no. 4, pp. 745–758, [10] R. F. Stainback, Y. S. Hamirani, D. A. Cooley, and L. M. Buja, “Tumors of the heart,” in Cardiovascular Medicine, J. T. Will- erson, H. J. J. Wellens, J. N. Cohn, and D. R. Holmes, Eds., [24] M. W. H. Kamal, M. Farshidpour, A. W. Long, S. Farooqui, pp. 2267–2294, Springer London, London, 2007. and S. C. Cunningham, “Hepatocellular carcinoma with intra-atrial extension responding to transarterial chemoembo- [11] R. Angelico, C. Grimaldi, M. C. Saffioti, A. Castellano, and lization via the right hepatic and right inferior phrenic arter- M. Spada, “Hepatocellular carcinoma in children: hepatic ies,” Gastrointestinal Cancer Research, vol. 7, no. 3-4, resection and liver transplantation,” Translational Gastroen- pp. 111–116, 2014. terology and Hepatology, vol. 3, p. 59, 2018. Case Reports in Oncological Medicine 5 [25] M. Oncale and B. Lewis, “Hepatocellular carcinoma with extension to the heart via the inferior vena cava,” Baylor University Medical Center Proceedings, vol. 28, no. 2, pp. 229-230, 2015. [26] S. Jelic and G. C. Sotiropoulos, “Hepatocellular carcinoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up,” Annals of Oncology, vol. 21, Supplement 5, pp. v59–v64, 2010. 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A Rare Case of Intracardiac Extension of Hepatocellular Carcinoma in a Child

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Hindawi Case Reports in Oncological Medicine Volume 2019, Article ID 4783595, 5 pages https://doi.org/10.1155/2019/4783595 Case Report A Rare Case of Intracardiac Extension of Hepatocellular Carcinoma in a Child 1 1 2 Abdulrahman Al Jassmi , Hani Humad, and Said Abou Eida Department of Pediatric Hematology/Oncology, Dubai Hospital, PO Box 92227 Dubai, UAE Department of Pediatrics, Welcare Hospital, Dubai, UAE Correspondence should be addressed to Abdulrahman Al Jassmi; amaljassmi@dha.gov.ae Received 2 April 2019; Accepted 6 September 2019; Published 5 December 2019 Academic Editor: Raffaele Palmirotta Copyright © 2019 Abdulrahman Al Jassmi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hepatocellular carcinoma (HCC) is the fifth most common malignancy found in men and ninth most common in women, out of which 72.5% reported cases are from Asia. In children, it accounts for <2% cases worldwide with even rarer incidence of 1.2% involving intracardial extension. However, it presents with a high mortality rate with mean survival ranging from 1 to 4 months. The present case is an extremely rare case of intracardiac extension of HCC in a 3.5-year-old Asian girl with no history of hepatitis B infection presented at an advanced stage of HCC who succumbed within one month of presentation to the hospital. 1. Introduction incidence rate of 1.2% [9]. In cardiac metastasis, HCC leads to intracavitary involvement of the right atrium through Historically, first being described in the eighteenth century direct tumor invasion of the inferior vena cava (IVC) causing by Gaspard-Laurent Bayle, hepatocellular carcinoma (HCC) cardiac symptoms [9, 10]. is now the most common malignant liver tumor following However, this report is an extremely rare case of intracar- hepatoblastoma [1, 2]. Worldwide, it is the third leading diac extension of HCC in a girl with 80% involvement of the cause of cancer-related mortality accounting for 782,000 right atrium and extension into the right ventricle too, with- (95%) deaths out of 782,000 people in 2012 and 781,631 out any cardiac symptoms at the time of presentation. (93%) out of 841,080 people in 2018. It is the fifth most com- mon malignancy found in men and ninth most common in 2. Case Report women out of which 72.5% cases are reported from Asia [2, 3]. Although it is quite rare in children, accounting At the end of November 2012, a 3.5-year-old child presented for less than 2% cases worldwide, it presents with high mor- to the pediatric department with a progressive abdominal tality with nearly 80% deaths within the first year of diagnosis swelling for the past two and a half months. Earlier, she [1, 4–7]. In most cases, the diagnosis of HCC is made at an was seen in Bangladesh, where antibiotics were prescribed advanced stage with the usual sites of metastases being the yet she did not take them. Initially, she had an erythematous lungs, lymph nodes, and bones [8]. rash that subsided but had no other symptoms of vomiting, Generally, the incidence of cardiac metastasis ranges diarrhea, constipation, or difficulty in breathing. On physical from 2.3 to 18.3% with a greater propensity of metastases examination, the girl has a thin build with an abdominal after the fifth decade of life; the most common secondary car- mass extending from the right hypochondrium downward diac tumors are melanoma, mediastinal primary tumors, (8-10 cm) crossing the midline, which had a lobular irregular mesotheliomas, lung carcinomas, breast carcinoma, and surface; the upper border of the mass was nonpalpable, and renal carcinoma. Albeit found in close proximity to the heart, no bruits were present. Chest, cardiovascular, and central the intracardiac extension of HCC is extremely rare with an nervous system examinations were normal along with the 2 Case Reports in Oncological Medicine Table 1: The results of the laboratory investigations of the patient at different intervals. Tests Normal range Result 1 Results 2 Results 3 ∗ ∗ ∗ Hemoglobin (g/dL) 11-13 15.7 16.4 14 White blood cells (×10 /μL) 5.0-15.0 14.2 11 14.9 3 ∗ ∗ 523 489 Platelets (×10 /μL) 150-450 290 ∗ ∗ Albumin (g/dL) 3.8-5.4 3.9 3.6 2.5 ∗ ∗ 48 1573 Alanine aminotransferase (U/L) 0-33 29 Aspartate aminotransferase (U/L) <281 242 199 HIV AG/AB Negative Negative —— >30,000 Alpha-fetoprotein (ng/mL) 0.5-5.5 —— Calcium oxalate crystals: 3+ Urinalysis White blood cells: 0-5 absence of pallor, jaundice, and lymphadenopathy. No signs of edema were noted. The child was admitted a couple of times for the evalua- tion of the mass, performing investigations and reaching a final diagnosis. Meanwhile, she was treated symptomatically in the ICU with intravenous hydration and maintaining con- stant oxygenation. She was followed up conservatively because of the end-staged metastatic HCC and the increased risks of heart surgery-attributable mortality. During this period, she was referred to other centers for investigations, including chest X-ray and angiography and transesophageal echocardiography (TEE) to get insights into not only the location of the mass in relation to the atrial wall or the tricus- pid valve but also its position with respect to the superior and Figure 1: Chest X-ray showing an abdominal mass. inferior vena cava. However, TEE was not performed due to financial constraints. The patient’s conditions worsened and she developed fever, periorbital edema, sacral edema, and of the heart in close contact with IVC. Vessels were pedal edema. Her edema worsened, and later she developed stretched rather than compromised or invaded, and portal difficulty in breathing and had to be put on artificial oxygen and hepatic veins were patent. Chest CT showed slight to maintain saturation. right-sided pleural effusion filling the lateral and posterior Three months later, cardiac auscultation revealed a sinus costophrenic sulci along with mild pleural effusion on the left side too. A large exophytic mass was noted projecting, heart rhythm and 2/6 (quiet) systolic murmur. Additionally, a bilateral pitting edema was noticed in the lower extremities. as an extension from the extensive liver lesion, into the right Laboratory investigations showed abnormally elevated atrium. Bone scintigraphy was unremarkable with no evi- hemoglobin, platelets, albumin, and hepatic enzymes dence of a metastatic lesion. (Table 1). Additionally, chest X-ray showed an ill-defined Liver biopsy was performed at our institution, and the histopathological report was consistent with a malignant infiltrate in the right lower zone with bilateral bronchial wall thickening (Figure 1). Notably, there were no lung metasta- tumor (Figure 2). Immunohistochemistry findings favored ses. On abdominal ultrasonography, the liver was enlarged the diagnosis of a moderately differentiated HCC. In line with nodular heterogeneous echotexture and multiple echo- with the findings of the physical examination, imaging stud- genic patches without biliary dilatation. ies, and histopathological analysis, the patient was finally diagnosed with HCC, which had extended through the infe- A 2D transthoracic echocardiogram demonstrated a large mass in the right atrium extending to the tricuspid valve rior vena cava to the right atrium, occupying 80% of the right without significant obstruction. Computed tomography atrium with an extension to the right ventricle. The Pretreat- (CT) of the abdomen showed a large 9:5×11:5× 13:5cm ment Extension of Disease (PRETEXT) system was used for lobulated, mixed complex solid-cystic mass lesion, which staging, and the tumor was staged as PRETEXT IV, V3. The patient was readmitted for further management after was a predominantly hypodense solid lesion with heteroge- nous cystic components and intervening thick septa occupy- reaching the final diagnosis of HCC. Her condition was ing the left lobe of the liver and a large part of the right lobe reviewed by a pediatric oncology team and cardiothoracic (segments 5 and 6) and caudate lobe. It extended superiorly surgeons, and she was started on chemotherapy with a plan above the diaphragm as a well-demarcated 2:5×3cm mass to resect the mass extending to the right atrium. Neverthe- less, the patient could not sustain chemotherapy owing to at the cardiophrenic angle dipping into the right atrium Case Reports in Oncological Medicine 3 Figure 2: A biopsy showing frequent mitotic figures, pleomorphism, and vesicular nuclei with ill-defined infiltrate in the right lower zone. her comorbidities. Unfortunately, the girl died of cardiore- 18 cases of right atrium extension of tumor and 5 cases of spiratory arrest at the operation table during the induction extension into the right ventricle [20]. HCC has a greater of anesthesia at the end of December 2012. CPR measures propensity for vascular invasion with portal vein thrombosis were undertaken to resuscitate the child, but the child suc- being the most common accounting for 20-65% cases, cumbed to inflow and outflow blockage of the heart. followed by systemic venous thromboembolism at 6% and hepatic vein thrombosis at 1.4-4.9%. However, the tumor thrombus invasion of the inferior vena cava is quite rare 3. Discussion accounting for 0.67-3% of all cases [21]. HCC stages as an abdominal swelling along with discomfort Cardiac metastases occur either via direct extension of a owing to its large size, which is comparable to this case [4]. It tumor thrombus through IVC invasion or through the bloodstream or the lymphatic system; the most common is responsible for 23% of all primary pediatric hepatic malig- nancies with an annual incidence of 0.15 per 100,000 (1975- pathway for HCC is IVC [9, 16]. Isolated cardiac metastasis 1995). Overwhelmingly, 75% of children over the age of 10 of HCC is extremely rare, and a literature search shows 17 years are affected; however, it is quite rare in children < 5 such cases, none of which were pediatric patients [16, 22]. years of age, accounting for <10% cases [4, 7]. In contrast The frequent location of an intracardiac primary or met- astatic tumor is usually the left ventricle and left atrium, with to adults, where HCC occurs predominantly on the back- ground of cirrhotic liver, HCC presents in normal livers in the right ventricle and right atrium being less common. Right the majority (70%) of the pediatric population [11]. Gener- atrium tumors may obstruct the orifice or damage the tricus- ally, these children are positive for hepatitis B serum antigen pid valve leading to tricuspid stenosis or regurgitation caus- (HBsAg), which is in contrast to this case [6]. Other etiolog- ing right heart failure with resulting signs and symptoms of dyspnea and lower extremity edema [23]. Great heterogene- ical factors related to HCC in children constitute congenital and metabolic conditions including biliary atresia, hereditary ity is seen in patients with intracardiac extension of HCC, tyrosinemia type 1, congenital hepatic fibrosis, and progres- from being asymptomatic to heart failure, sudden cardiac sive familial intrahepatic cholestasis [7]. arrest, Budd-Chiari syndrome, and pulmonary embolism In the orient and sub-Saharan African regions, about [24]. The reported patient had no cardiac symptoms at the time of presentation, until at the later stage she developed 75-92% reported cases are positive for serum alpha- fetoprotein level (sAFP). AFP levels are of prognostic value fever and periorbital, sacral, and pedal edema along with dif- as elevated AFP titers are associated with 1.8 times higher ficulty in breathing. The prognosis for intracardiac metastasis mortality [6]. HCC also carries poor prognosis owing to its of HCC is dismal with mean survival ranging from 1 to 4 chemoresistant nature with an estimated cure rate of only months, irrespective of treatment [12, 25]. Cardiac metastasis is best appreciated in transesophageal 25-30% [4, 5]. Advanced stages of HCC have a median sur- vival rate of 4-7 months [12]. Tumor resection is the sole echocardiography (TEE), transthoracic echocardiography curative treatment, and it can be performed either via partial (TTE) along with CT scan, and MRI, which form the main- liver resection or hepatic transplantation. In the recent stud- stay for diagnosing HCC metastasis to the heart. These ies, the overall five-year survival rate ranges between 50% help in identifying the extension of the tumor to the heart, right ventricular outflow obstruction, pulmonary thrombo- and 60% following surgical resection, yet it reaches only 20-30% in pediatric patients with advanced disease at diag- embolism, and pericardial effusion [18]. The only effective nosis who received a combination of surgical resection and treatment option includes surgical extraction of the throm- chemotherapy [13–15]. bus along with tumor resection, although patient manage- Unfortunately, a very large number of pediatric patients ment with advanced HCC with intracardiac extension is difficult and extremely risky. Other management options have advanced or metastatic HCC at the time of diagnosis, with the lung being the most common site for extrahepatic include transarterial chemoembolization (TACE), ablation, metastasis [7, 8]. However, an extension of HCC into the radiation, and chemotherapy [19, 24, 26]. Although the right atrium is possible, albeit it is extremely rare with studies patient in the present report was opted for surgical exci- indicating 1.2-4.1% of extrahepatic cardiac metastasis sion of the tumor, the patient succumbed to inflow and outflow blockage of the heart during anesthesia initiation [16–19]. An autopsy case study of 439 HCC cases showed 4 Case Reports in Oncological Medicine at the operation table. She died in <1 month after being [12] M. Salehi, T. Yee, E. Alatevi, and Y. Thein, “Clinically silent intracardiac metastasis with extremely poor progno- presented to the hospital. sis in a patient with hepatocellular carcinoma,” Case HCC with cardiac metastasis has very poor prognosis, Reports in Gastroenterology, vol. 11, no. 2, pp. 416–421, more so, in pediatric patients. The present case is an extremely rare case of hepatocellular carcinoma extension [13] J. P. McAteer, A. B. Goldin, P. J. Healey, and K. W. Gow, “Sur- to the right atrium along with extension to the right ventricle gical treatment of primary liver tumors in children: outcomes in a 3.5-year-old girl. This is the first literature review involv- analysis of resection and transplantation in the SEER data- ing a child with intracardiac extension of HCC. Awareness in base,” Pediatric Transplantation, vol. 17, no. 8, pp. 744–750, clinicians about such rare cases will affect the overall diagno- sis and management of patients. [14] M. Murawski, V. B. Weeda, R. Maibach et al., “Hepatocellular carcinoma in children: does modified platinum-and doxorubicin-based chemotherapy increase tumor resectability Conflicts of Interest and change outcome? Lessons learned from the SIOPEL 2 and 3 studies,” Journal of Clinical Oncology, vol. 34, no. 10, The authors declare that there is no conflict of interest pp. 1050–1056, 2016. regarding the publication of this paper. [15] J. de Ville de Goyet, R. L. Meyers, G. M. Tiao, and B. Morland, “Beyond the Milan criteria for liver transplantation in children References with hepatic tumours,” The Lancet Gastroenterology & Hepa- tology, vol. 2, no. 6, pp. 456–462, 2017. [1] P. R. Exelby, R. M. Filler, and J. L. Grosfeld, “Liver tumors in [16] M. Kawakami, M. Koda, M. Mandai et al., “Isolated metastases children in the particular reference to hepatoblastoma and of hepatocellular carcinoma in the right atrium: case report hepatocellular carcinoma: American Academy of Pediatrics and review of the literature,” Oncology Letters, vol. 5, no. 5, Surgical Section Survey – 1974,” Journal of Pediatric Surgery, pp. 1505–1508, 2013. vol. 10, no. 3, pp. 329–337, 1975. [17] M. Barrett, L. Viglianti Benjamin, A. Hanson Christopher, and [2] A. Reuben, “Hepatocellular carcinoma in adults and chil- R. J. Schildhouse, “A case of right atrial obliteration caused by dren,” Clinics in Liver Disease, vol. 19, no. 2, pp. xiii–xxvi, intracardiac extension of hepatocellular carcinoma,” Case Reports in Oncology, vol. 10, no. 1, pp. 8–14, 2017. [3] International Agency for Research on Cancer, GLOBOCAN [18] Y.-C. Liu, Y.-L. Ho, G.-T. Huang, D.-S. Chen, J.-C. Sheu, and 2012: estimated cancer incidence, mortality and prevalence C.-H. Chen, “Clinical manifestations and survival of patients worldwide in 2012 v1.0, IARC, 2012, July 2019, https:// with hepatocellular carcinoma and cardiac metastasis,” Jour- publications.iarc.fr/Databases/Iarc-Cancerbases/ nal of Gastroenterology and Hepatology, vol. 25, no. 1, GLOBOCAN-2012-Estimated-Cancer-Incidence-Mortality- pp. 150–155, 2010. And-Prevalence-Worldwide-In-2012-V1.0-2012. [19] W. Li, Y. Wang, W. Gao, and J. Zheng, “HCC with tumor [4] B. J. Allan, B. Wang, J. S. Davis et al., “A review of 218 pediatric thrombus entering the right atrium and inferior vena cava cases of hepatocellular carcinoma,” Journal of Pediatric Sur- treated by percutaneous ablation,” BMC Surgery, vol. 17, gery, vol. 49, no. 1, pp. 166–171, 2014. no. 1, article 21, 2017. [5] R. Pazdur, B. Bready, and A. Cangir, “Pediatric hepatic tumors: [20] M. Kojiro, H. Nakahara, S. Sugihara, T. Murakami, clinical trials conducted in the United States,” Journal of Surgi- T. Nakashima, and H. Kawasaki, “Hepatocellular carcinoma cal Oncology, vol. 53, no. S3, pp. 127–130, 1993. with intra-atrial tumor growth. A clinicopathologic study of [6] S. W. Moore, P. B. Hesseling, G. Wessels, and J. W. Schneider, 18 autopsy cases,” Archives of Pathology & Laboratory Medi- “Hepatocellular carcinoma in children,” Pediatric Surgery cine, vol. 108, no. 12, pp. 989–992, 1984. International, vol. 12, no. 4, pp. 266–270, 1997. [21] J. Huang, Z.-Y. Pan, L. Li et al., “Hepatocellular carcinoma [7] K. Palaniappan, V. V. Borkar, M. Safwan et al., “Pediatric with inferior vena caval and right atrial tumor thrombi hepatocellular carcinoma in a developing country: is the etiol- and massive pulmonary artery embolism: a case report,” ogy changing?,” Pediatric Transplantation, vol. 20, no. 7, Molecular and Clinical Oncology, vol. 6, no. 1, pp. 111–114, pp. 898–903, 2016. [8] T. Terada and H. Maruo, “Unusual extrahepatic metastatic [22] E. Tastekin, U. Usta, T. Ege, G. Kazindir, and A. K. Kutlu, sites from hepatocellular carcinoma,” International Journal of “Cardiac metastasis of hepatocellular carcinoma in a young Clinical and Experimental Pathology, vol. 6, no. 5, pp. 816– non-cirrhotic patient, to the left ventricle,” Annals of hepatol- 820, 2013. ogy, vol. 11, no. 3, pp. 392–394, 2012. [9] R. Bussani, F. De-Giorgio, A. Abbate, and F. Silvestri, “Cardiac metastases,” Journal of Clinical Pathology, vol. 60, no. 1, [23] I. P. Panidis, M. N. Kotler, G. S. Mintz, and J. Ross, “Clinical and echocardiographic features of right atrial pp. 27–34, 2007. masses,” American Heart Journal, vol. 107, no. 4, pp. 745–758, [10] R. F. Stainback, Y. S. Hamirani, D. A. Cooley, and L. M. Buja, “Tumors of the heart,” in Cardiovascular Medicine, J. T. Will- erson, H. J. J. Wellens, J. N. Cohn, and D. R. Holmes, Eds., [24] M. W. H. Kamal, M. Farshidpour, A. W. Long, S. Farooqui, pp. 2267–2294, Springer London, London, 2007. and S. C. Cunningham, “Hepatocellular carcinoma with intra-atrial extension responding to transarterial chemoembo- [11] R. Angelico, C. Grimaldi, M. C. Saffioti, A. Castellano, and lization via the right hepatic and right inferior phrenic arter- M. Spada, “Hepatocellular carcinoma in children: hepatic ies,” Gastrointestinal Cancer Research, vol. 7, no. 3-4, resection and liver transplantation,” Translational Gastroen- pp. 111–116, 2014. terology and Hepatology, vol. 3, p. 59, 2018. Case Reports in Oncological Medicine 5 [25] M. Oncale and B. Lewis, “Hepatocellular carcinoma with extension to the heart via the inferior vena cava,” Baylor University Medical Center Proceedings, vol. 28, no. 2, pp. 229-230, 2015. [26] S. Jelic and G. C. Sotiropoulos, “Hepatocellular carcinoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up,” Annals of Oncology, vol. 21, Supplement 5, pp. v59–v64, 2010. 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