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Liver resection for atypical giant focal nodular hyperplasia
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Liver resection for atypical giant focal nodular hyperplasia a,∗
a
b
a
T a
Dmitry Akhaladze , Nikolay Grachev , Denis Kachanov , Sergey Talypov , Nikolay Merkulov , Natalia Uskovaa, Evgeniy Andreeva, Gavriil Rabaeva, Natalia Ivanovaa, Svetlana Varfolomeevac a Department of Oncology and Pediatric Surgery of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia b Department of Clinical Oncology of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia c Institute of Oncology, Radiology and Nuclear Medicine of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia
A R T I C LE I N FO
A B S T R A C T
Keywords: Huge focal nodular hyperplasia Children Liver resection Hepatic vein dilatation
Focal nodular hyperplasia (FNH) usually presents in children with underlying liver disease, in patients undergone chemotherapy and adolescent females. The reports, describing huge FNH with severe dilatation of the right hepatic vein (RHV) in healthy children are absent. A 2,7 years old boy without any comorbidities was presented with intermittent abdominal pain and a huge mass, causing the abdominal asymmetry. The initial imaging showed the giant mass (more than 125 mm in the biggest dimensions), with sings of the atypical FNH It was arising from 5,6 liver segments, and was accompanied with strong expansion of the RHV. According to CT-volumetry, the lesion volume exceeded the right hepatic lobe volume on 350 ml. Despite the all signs of atypical FNH and absence of underlying liver diseases, the surgery without preoperative biopsy was carried out. Postoperative course was uneventful. The pathological examination confirmed the diagnosis - focal nodular hyperplasia of the liver. The postoperative computed tomography showed the RHV, returned to its physiological size. Giant atypical FNH may occur in very young children. Huge size of the tumor can lead to severe dilatation of the RHV, which can be reversible. The surgical resection is the treatment of choice in such cases.
1. Introduction Benign liver tumors occur rare in children, in contrast with adults. After the infantile hemangioma, the focal nodular hyperplasia (FNH) remains the second most frequent lesion and, according to many authors, accounts for less than 2% of liver tumors in children [1–8]. In most FNH are incidentally found, never become malignant and commonly do not require surgery in asymptomatic cases. Despite, this neoplasm has specific computed tomography (CT) and magnetic resonance imaging (MRI) characteristics sometimes it is necessary to provide needle biopsy to exclude malignant liver tumor – first of all fibrolamellar hepatocellular carcinoma and hepatocellular adenoma [9,10]. The pathogenesis of FNH is not completely understood. Among the etiological factors the underlying liver diseases such as biliary athresia, Alagille syndrome, or liver parenchyma injury after chemoand radiotherapy are described [11–16]. A couple of reports describe
FNH in kids, underwent hematopoietic stem cell transplant [17–20] and as a consequence of hepatocyte atrophy, compensatory to liver tissue hyperplasia in cases of vascular abnormalities [21,22]. However, no mentions are found in the literature about liver blood vessels, in particular hepatic veins, changes caused by huge FNH. Etiology and diagnostic algorithms are well described in a literature, but there are limited reports on large FNG in children. In this paper an interesting clinical case of a giant size FNG with a number of previously unspecified vascular features in a small boy is presented. 2. Case report A 2,7 years old boy admitted to our clinic with a large hepatic lesion. It was detected accidently, presented with abdominal asymmetry and right upper quadrant pain. Screening ultrasound 6 months before did not find any pathology. Abdominal CT showed non-encapsulated
∗
Corresponding author. E-mail addresses: [email protected] (D. Akhaladze), [email protected] (N. Grachev), [email protected] (D. Kachanov), [email protected] (S. Talypov), [email protected] (N. Merkulov), [email protected] (N. Uskova), [email protected] (E. Andreev), [email protected] (G. Rabaev), [email protected] (N. Ivanova), [email protected] (S. Varfolomeeva). https://doi.org/10.1016/j.epsc.2019.101202 Received 3 February 2019; Received in revised form 29 March 2019; Accepted 7 April 2019 Available online 09 April 2019 2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
D. Akhaladze, et al.
Fig. 1. CT, showing a large liver right lobe mass (1) in the arterial enhancement phase (A). The lesion became isoattenuated to normal adjacent parenchyma (2) in portal venous (B) and on the delay phase (C). 3 – compressed right kidney; 4 – gallbladder; Ao - aorta.
initial signs of biliary hypertension - dilated to 6 mm intrahepatic right lobe bile ducts. 3D CT reconstruction provided additional information about the tumor size and the interrelation between the lesion and anatomical structures, first of all – right hepatic vein. Gadolinium-enhanced MRI demonstrated arterial hyperenhanced lesion, isointensed to the liver on portal venous phase. On MRI, a central scar was not found also. Initial laboratory assessment demonstrated slightly elevated total bilirubin (32 mmol/l), aminotransferase levels (aspartate aminotransferase 61 U/L; alanine aminotransferase 58 U/L), normal alkaline phosphatase and negative hepatitis B and C serologic testing. Serum α-fetoprotein (AFP) measured 3,91 ng/ml. Other system examinations were within normal values. Given the tumor size, moderate biliary hypertension and clinical symptoms (notable abdominal asymmetry and pain), the patient, according to imaging findings and laboratory date was considered to be a candidate for surgery without pathologic confirmation of diagnosis. The preliminary diagnosis before surgery was atypical FNH. Intraoperatively the lesion more than 12 cm involved 5,6 liver segments, displacing the gallbladder, ascending colon and small bowel medially (Fig. 3). No evidence of extrahepatic disease spread and lymphadenopathy was found. As the biopsy before surgery was not performed, to exclude the hepatocellular carcinoma (HCC) or hepatocellular adenoma (HCA), an urgent pathology was sent. Fresh frozen histology did not show any malignancy. Thus, an atypical 5,6 bisegmentectomy was applied. After right liver lobe mobilization the right hepatic vein, supdiaphragmatic and infrahepatic IVC were encircled with vascular loops to control in case of bleeding. Parenchymal transection was carried out using the ultrasonic dissector and bipolar cautery under short hepatic vascular occlusion. Pringle maneuver duration lasted 4 min. All feeding vessels were ligated and dissected out. The dilated right hepatic vein was clamped, than closed with running suture. Postoperative course was uneventful and patient was discharged on POD 7. In a pathological examination, the lesion was represented by liver tissue with a nodular structure due to the presence of fibrous septa of various thickness with coarse scar in the center of the sample.
Fig. 2. CT 3D – reconstruction. The right liver volume came to 195 ml; tumor volume measurement shows 545 ml.
huge liver mass with smooth lobular edges measuring 128 × 92 × 119 mm, originating from hepatic segments 5,6. An intravenous enhancement displayed the strong and homogeneous contrasting of entire mass during arterial phase. The mass became isoattenuated to healthy liver parenchyma on venous and delayed phase. Absence of a central scar in the lesion signed on atypical FNH (Fig. 1). A number of arterial vessels feeding the tumor were visualized. Additionally, an enlarged (17 mm) right hepatic vein was noted (in comparison to the diameter of the retrohepatic inferior vena cava (IVC) which came to 14 mm) (Fig. 4A). According to CT-volumetry, the volume of the lesion exceeded the right hepatic lobe volume on 350 ml (Fig. 2). This explains hepatic vein dilatation up to 17 mm in diameter. Besides, the patient presented the 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
D. Akhaladze, et al.
Moreover there are no reports of any size FNH in children with so dilated hepatic vein in literature. From our point of view, the right hepatic vein expansion in present case evolved due to giant tumor size. According to the preoperative CT volumetry, the volume of lesion (557 ml) exceeded the volume of the right liver lobe (180 ml). Thus, such increased load on the right hepatic vein could cause its dilatation. This hypothesis is also confirmed by the decreased diameter of the right hepatic vein showed on POD 6 CT. The diagnostic and treatment approaches of FNH and guidelines are well known - in atypical cases on imaging, liver biopsy is indicated [27]. In described child the surgery was carried out without the preoperative needle biopsy for the following reasons: the only sign of an atypical FNH was the absence of central scar, while other findings confirmed the diagnosis. Anyway, the patient had the indications for surgery such as biliary hypertension and the pain. Besides, we planned the intraoperative histological examination to exclude HCC and HCA. However, some authors claim that biopsy is mandatory due to overlapping radiological features of benign and malignant tumors [9,28,29]. At the same time, there are a lot of publications describing the specific CT, MRI and ultrasound visualization data for FNH [6,9,22,30–33]. Non-surgical treatment was not considered as option because it should be reserved for patients unfit for resection [27]. 4. Conclusion Fig. 3. Operative field. 1 – biased gallbladder.
Therefore, this clinical case proves the possibility of giant FNH in very young children under 5 years old. Huge FNH may be accompanied by dilated hepatic vein if it is drained into the system of one hepatic vein. Liver resection in case of it is necessarily leads to decrease the vein diameter. The presence of radiological characteristic features even in case of central scar absence may confirm the FNH and allows to avoid biopsy in selected patients. Conflicts of interest None. Disclosure The authors declare no conflicts of interest. Funding Fig. 4. A - preoperative CT shows dilated right hepatic vein (1); 2 – main trunk of middle hepatic vein; 3 – main trunk of left hepatic vein; 4 – inferior vena cava. B – decreased in diameter right hepatic vein on postoperative CT (1).
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Proliferation of the bile ducts around fibrosis was observed. Hepatocytes were without signs of atypia. The final histopathology was reported as FNG of the liver. The postoperative CT showed reduced to 9 mm diameter of right hepatic vein.
Acknowledgements None. References
3. Discussion
[1] Meyers RL Tumors of the liver in children. Surg Oncol 2007;16:195–203. https:// doi.org/10.1016/j.suronc.2007.07.002. [2] Reymond D, Plaschkes J, Ridolfi Lüthy A, Leibundgut K, Hirt A, Wagner HP. Focal nodular hyperplasia of the liver in children: review of follow-up and outcome. J Pediatr Surg 1995;30(11):1590–3. https://doi.org/10.1016/j.hpb.2017.05.001. [3] Yang Y, Fu S, Li A, Zhou W, Pan Z, Cui L, Huang G, Wu B, Wu M. Management and surgical treatment for focal nodular hyperplasia in children. Pediatr Surg Int 2008;24:699–703. №6. [4] Mathieu D, Vilgrain V, Mahfouz AE, Anglade MC, Vullierme MP, Denys A. Benign liver tumors. Magn Reson Imag Clin N Am 1997;5:255–88. [5] Wanless IR, Mawdsley C, Adams R. On the pathogenesis of focal nodular hyperplasia of the liver. Hepatology 1985;5:1194–200. [6] Cha DI, Yoo SY, Kim JH, Jeon TY, Eo H. Clinical and imaging features of focal nodular hyperplasia in children. AJR Am J Roentgenol 2014 May;202(5):960–5. https://doi.org/10.2214/AJR.13.11856. [7] Mueller BU, Lopez-Terrada D, Finegold MJ. Tumors of the liver. Principles and practice of pediatric oncology. Philadelphia: Lippincott Williams and Wilkins; 2006. p. 887–904.
FNH can be diagnosed at any age but in children the median age of diagnosis was found to be 8.7 years [11,23]. It is difficult to diagnose a benign liver lesion in very young children group (under 5 years old) especially without underlying liver disease, because in this age 75% of the liver tumors are malignant [24]. In the literature review Ma I.T. et al. found 6 patients in this age group with the largest dimension of lesion - 9.4 cm (6.6 ± 3.4 cm) [25]. Zhuang L. et al. reported a case of surgery in 6 years old child with 10.5 × 8x6.5 cm huge FNG as the biggest FNH ever reported among children [26]. We described a huge FNH in 2 years and 7 months old boy. We did not found any mention in the literature, of the size of FNH, which exceeds the size of described patient in this age. 3
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
D. Akhaladze, et al.
[8] Oliveira C, Gil-Agostinho A, Gonçalves I, Noruegas MJ. Transarterial embolisation of a large focal nodular hyperplasia, using microspheres, in a paediatric patient. BMJ Care Rep 2015. https://doi.org/10.1136/bcr-2014-208879. Jul 10;2015. pii: bcr2014208879. [9] Valentino PL, Ling SC, Ng VL, John P, Bonasoni P, Castro DA, Taylor G, Chavhan GB, Kamath BM. The role of diagnostic imaging and liver biopsy in the diagnosis of focal nodular hyperplasia in children. Liver Int 2014 Feb;34(2):227–34. https://doi. org/10.1111/liv.12241. [10] Pillon M, Carucci NS, Mainardi C, Carraro E, Zuiliani M, Chemello L, Carole E, Tumino M, Varotto S, Toffolutti T, Destro R, Gazzola MV, Alaggio R, Basso G, Messina C. Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children. Bone Marrow Transplant 2015 Mar;50(3):414–9. https://doi.org/10.1038/bmt.2014.276. [11] Towbin AJ, Luo GG, Yin H, Mo JQ. Focal nodular hyperplasia in children, adolescents, and young adults. Pediatr Radiol 2011 Mar;41(3):341–9. https://doi.org/10. 1007/s00247-010-1839-8. [12] Joyner BL, Levin TL, Goyal RK, Newman B. Focal nodular hyperplasia of the liver: a sequela of tumor therapy. Pediatr Radiol 2006;36:729. https://doi.org/10.1007/ s00247-005-0090-1. [13] Bouyn CI, Leclere J, Raimondo G, Le Pointe HD, Couanet D, Valteau-Couanet D, Hartmann O. Hepaticfocal nodular hyperplasia in children previously treated for a solid tumor: incidence, risk factors, and outcome. Cancer 2003;97:3107–13. [14] Marabelle A, Campagne D, Dechelotte P, Chipponi J, Demeocq F, Kanold J. Focal nodular hyperplasia of the liver in patients previously treated for pediatric neoplastic diseases. J Pediatr Hematol Oncol 2008 Jul;30(7):546–9. https://doi.org/10. 1097/MPH.0b013e3181691709. [15] Tajima T, Honda H, Yanaga K, Kuroiwa T, Yoshimitsu K, Irrie H, Kuwabara Y, Taguchi K, Masuda K. Hepatic nodular hyperplasia in a boy with Alagille syndrome: CT and MR appearances. Pediatr Radiol 2001 Aug;31(8):584–8. [16] Rapp JB, Bellah RD, Maya C, Pawel BR, Anupindi SA. Giant hepatic regenerative nodules in Alagille syndrome. Pediatr Radiol 2017 Feb;47(2):197–204. https://doi. org/10.1007/s00247-016-3728-2. [17] Pillon M, Carucci NS, Mainardi C, Carraro E, Zuiliani M, Chemello L, Carole E, Tumino M, Varotto S, Toffolutti T, Destro R, Gazzola MV, Alaggio R, Basso G, Messina C. Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children. Bone Marrow Transplant 2015;50:414–9. https:// doi.org/10.1038/bmt.2014.276. [18] Anderson L, Gregg D, Margolis D, Casper J, Talano J. Focal nodular hyperplasia in pediatric allogeneic hematopoietic cell transplant: case series. Bone Marrow Transplant 2010 Aug;45(8):1357–9. https://doi.org/10.1038/bmt.2009.336. [19] Sudour H, Mainard L, Baumann C, Clement L, Salmon A, Bordigoni P. Focal nodular hyperplasia of the liver following hematopoietic SCT. Bone Marrow Transplant 2009 Jan;43(2):127–32. https://doi.org/10.1038/bmt.2008.304. [20] Masetti R, Zama D, Gasperini P, Morello W, Prete A, Colecchia A, Festi D, Pession A. Focal nodular hyperplasia of the liver in children after hematopoietic stem cell
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4
transplantation. Pediatr Transplant 2013 Aug;17(5):479–86. https://doi.org/10. 1111/petr.12091. Kumagai H, Masuda T, Oikawa H, Endo K, Endo M, Takano T. Focal nodular hyperplasia of the liver: direct evidence of circulatory disturbances. J Gastroenterol Hepatol 2000;15:1344–7. Ji Y, Chen S, Xiang B, Wen T, Yang J, Zhong L, Li F, Li F. Clinical features of focal nodular hyperplasia of the liver in children. J Pediatr Gastroenterol Nutr 2016 Jun;62(6):813–8. https://doi.org/10.1097/MPG.0000000000001094. Emre S, Umman V, Rodriguez-Davalos M. Current concepts in pediatric liver tumors. Pediatr Transplant 2012;16:549–63. https://doi.org/10.1111/j.1399-3046. 2012.01704.x. Meyers RL. Tumors of the liver in children. Surg Oncol 2007;16:195–203. https:// doi.org/10.1016/j.suronc.2007.07.002. Ma IT, Rojas Y, Masand PM, Castro EC, Himes RW, Kim ES, Goss JA, Nuchtern JG, Finegold MJ, Thompson PA, Vasudevan SA. Focal nodular hyperplasia in children: an institutional experience with review of the literature. J Pediatr Surg 2015 Mar;50(3):382–7. https://doi.org/10.1016/j.jpedsurg.2014.06.016. Zhuang L, Ni C, Din W, Zhang F, Sun Y, Xi D. Huge focal nodular hyperplasia presenting in a 6-year-old child: a case presentation. Int J Surg Case Rep 2016;29:76–9. https://doi.org/10.1016/j.ijscr.2016.10.053. Columbo M, Forner A, Ijzermans J, Paradis VL, Reeves H, Vilgrain VL, ZucmanRossi J. EASL Clinical Practice Guidelines on the management of benign liver tumours. J Hepatol 2016 Aug;65(2):386–98. https://doi.org/10.1016/j.jhep.2016.04. 001. Chow LT, Ng AW, Wong SK. Focal nodular and diffuse haematopoietic marrow hyperplasia in patients with underlying malignancies: a radiological mimic of malignancy in need of recognition. Clin Radiol 2017 Mar;72(3). https://doi.org/10. 1016/j.crad.2016.10.015. 265.e7-265.e23. Sannier A, Cazejust J, Leguoy M, Carvera P, Scatton O, Rusmorduc O, Wendum D. Liver biopsy for diagnosis of presumed benign hepatocellular lesions lacking magnetic resonance imaging diagnostic features of focal nodular hyperplasia. Liver Int 2016 Nov;36(11):1668–76. https://doi.org/10.1111/liv.13113. Kuo YH, Wang JH, Lu SN, Hung CH, Wei YC, Hu CH, Yen YH, Lee CM, Eng HL. Natural course of hepatic focal nodular hyperplasia: a long-term follow-up study with sonography. J Clin Ultrasound 2009 Mar-Apr;37(3):132–7. https://doi.org/10. 1002/jcu.20533. Leconte I, Van Beers BE, Lacrosse M, Sempoux C, Jamart J, Marterne R, Baudrez V, Horsmans Y. Focal nodular hyperplasia: natural course observed with CT and MRI. J Comput Assist Tomogr 2000 Jan-Feb;24(1):61–6. /abs/10.2214/AJR.13.11856. Alqatie A, Mann E, Moineddin R, Kamath BM, Chavhan JB. Solitary liver lesions in children: interobserver agreement and accuracy of MRI diagnosis. Clin Imaging 2015 May-Jun;39(3):442–8. https://doi.org/10.1016/j.clinimag.2014.11.014. Ronot M, Vilgrain V. Imaging of benign hepatocellular lesions: current concepts and recent updates. Clin Res Hepatol Gastroenterol 2014 Dec;38(6):681–8. https://doi. org/10.1016/j.clinre.2014.01.014.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Liver resection for atypical giant focal nodular hyperplasia a,∗
a
b
a
T a
Dmitry Akhaladze , Nikolay Grachev , Denis Kachanov , Sergey Talypov , Nikolay Merkulov , Natalia Uskovaa, Evgeniy Andreeva, Gavriil Rabaeva, Natalia Ivanovaa, Svetlana Varfolomeevac a Department of Oncology and Pediatric Surgery of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia b Department of Clinical Oncology of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia c Institute of Oncology, Radiology and Nuclear Medicine of Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela Str., 117997, Moscow, Russia
A R T I C LE I N FO
A B S T R A C T
Keywords: Huge focal nodular hyperplasia Children Liver resection Hepatic vein dilatation
Focal nodular hyperplasia (FNH) usually presents in children with underlying liver disease, in patients undergone chemotherapy and adolescent females. The reports, describing huge FNH with severe dilatation of the right hepatic vein (RHV) in healthy children are absent. A 2,7 years old boy without any comorbidities was presented with intermittent abdominal pain and a huge mass, causing the abdominal asymmetry. The initial imaging showed the giant mass (more than 125 mm in the biggest dimensions), with sings of the atypical FNH It was arising from 5,6 liver segments, and was accompanied with strong expansion of the RHV. According to CT-volumetry, the lesion volume exceeded the right hepatic lobe volume on 350 ml. Despite the all signs of atypical FNH and absence of underlying liver diseases, the surgery without preoperative biopsy was carried out. Postoperative course was uneventful. The pathological examination confirmed the diagnosis - focal nodular hyperplasia of the liver. The postoperative computed tomography showed the RHV, returned to its physiological size. Giant atypical FNH may occur in very young children. Huge size of the tumor can lead to severe dilatation of the RHV, which can be reversible. The surgical resection is the treatment of choice in such cases.
1. Introduction Benign liver tumors occur rare in children, in contrast with adults. After the infantile hemangioma, the focal nodular hyperplasia (FNH) remains the second most frequent lesion and, according to many authors, accounts for less than 2% of liver tumors in children [1–8]. In most FNH are incidentally found, never become malignant and commonly do not require surgery in asymptomatic cases. Despite, this neoplasm has specific computed tomography (CT) and magnetic resonance imaging (MRI) characteristics sometimes it is necessary to provide needle biopsy to exclude malignant liver tumor – first of all fibrolamellar hepatocellular carcinoma and hepatocellular adenoma [9,10]. The pathogenesis of FNH is not completely understood. Among the etiological factors the underlying liver diseases such as biliary athresia, Alagille syndrome, or liver parenchyma injury after chemoand radiotherapy are described [11–16]. A couple of reports describe
FNH in kids, underwent hematopoietic stem cell transplant [17–20] and as a consequence of hepatocyte atrophy, compensatory to liver tissue hyperplasia in cases of vascular abnormalities [21,22]. However, no mentions are found in the literature about liver blood vessels, in particular hepatic veins, changes caused by huge FNH. Etiology and diagnostic algorithms are well described in a literature, but there are limited reports on large FNG in children. In this paper an interesting clinical case of a giant size FNG with a number of previously unspecified vascular features in a small boy is presented. 2. Case report A 2,7 years old boy admitted to our clinic with a large hepatic lesion. It was detected accidently, presented with abdominal asymmetry and right upper quadrant pain. Screening ultrasound 6 months before did not find any pathology. Abdominal CT showed non-encapsulated
∗
Corresponding author. E-mail addresses: [email protected] (D. Akhaladze), [email protected] (N. Grachev), [email protected] (D. Kachanov), [email protected] (S. Talypov), [email protected] (N. Merkulov), [email protected] (N. Uskova), [email protected] (E. Andreev), [email protected] (G. Rabaev), [email protected] (N. Ivanova), [email protected] (S. Varfolomeeva). https://doi.org/10.1016/j.epsc.2019.101202 Received 3 February 2019; Received in revised form 29 March 2019; Accepted 7 April 2019 Available online 09 April 2019 2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
D. Akhaladze, et al.
Fig. 1. CT, showing a large liver right lobe mass (1) in the arterial enhancement phase (A). The lesion became isoattenuated to normal adjacent parenchyma (2) in portal venous (B) and on the delay phase (C). 3 – compressed right kidney; 4 – gallbladder; Ao - aorta.
initial signs of biliary hypertension - dilated to 6 mm intrahepatic right lobe bile ducts. 3D CT reconstruction provided additional information about the tumor size and the interrelation between the lesion and anatomical structures, first of all – right hepatic vein. Gadolinium-enhanced MRI demonstrated arterial hyperenhanced lesion, isointensed to the liver on portal venous phase. On MRI, a central scar was not found also. Initial laboratory assessment demonstrated slightly elevated total bilirubin (32 mmol/l), aminotransferase levels (aspartate aminotransferase 61 U/L; alanine aminotransferase 58 U/L), normal alkaline phosphatase and negative hepatitis B and C serologic testing. Serum α-fetoprotein (AFP) measured 3,91 ng/ml. Other system examinations were within normal values. Given the tumor size, moderate biliary hypertension and clinical symptoms (notable abdominal asymmetry and pain), the patient, according to imaging findings and laboratory date was considered to be a candidate for surgery without pathologic confirmation of diagnosis. The preliminary diagnosis before surgery was atypical FNH. Intraoperatively the lesion more than 12 cm involved 5,6 liver segments, displacing the gallbladder, ascending colon and small bowel medially (Fig. 3). No evidence of extrahepatic disease spread and lymphadenopathy was found. As the biopsy before surgery was not performed, to exclude the hepatocellular carcinoma (HCC) or hepatocellular adenoma (HCA), an urgent pathology was sent. Fresh frozen histology did not show any malignancy. Thus, an atypical 5,6 bisegmentectomy was applied. After right liver lobe mobilization the right hepatic vein, supdiaphragmatic and infrahepatic IVC were encircled with vascular loops to control in case of bleeding. Parenchymal transection was carried out using the ultrasonic dissector and bipolar cautery under short hepatic vascular occlusion. Pringle maneuver duration lasted 4 min. All feeding vessels were ligated and dissected out. The dilated right hepatic vein was clamped, than closed with running suture. Postoperative course was uneventful and patient was discharged on POD 7. In a pathological examination, the lesion was represented by liver tissue with a nodular structure due to the presence of fibrous septa of various thickness with coarse scar in the center of the sample.
Fig. 2. CT 3D – reconstruction. The right liver volume came to 195 ml; tumor volume measurement shows 545 ml.
huge liver mass with smooth lobular edges measuring 128 × 92 × 119 mm, originating from hepatic segments 5,6. An intravenous enhancement displayed the strong and homogeneous contrasting of entire mass during arterial phase. The mass became isoattenuated to healthy liver parenchyma on venous and delayed phase. Absence of a central scar in the lesion signed on atypical FNH (Fig. 1). A number of arterial vessels feeding the tumor were visualized. Additionally, an enlarged (17 mm) right hepatic vein was noted (in comparison to the diameter of the retrohepatic inferior vena cava (IVC) which came to 14 mm) (Fig. 4A). According to CT-volumetry, the volume of the lesion exceeded the right hepatic lobe volume on 350 ml (Fig. 2). This explains hepatic vein dilatation up to 17 mm in diameter. Besides, the patient presented the 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101202
D. Akhaladze, et al.
Moreover there are no reports of any size FNH in children with so dilated hepatic vein in literature. From our point of view, the right hepatic vein expansion in present case evolved due to giant tumor size. According to the preoperative CT volumetry, the volume of lesion (557 ml) exceeded the volume of the right liver lobe (180 ml). Thus, such increased load on the right hepatic vein could cause its dilatation. This hypothesis is also confirmed by the decreased diameter of the right hepatic vein showed on POD 6 CT. The diagnostic and treatment approaches of FNH and guidelines are well known - in atypical cases on imaging, liver biopsy is indicated [27]. In described child the surgery was carried out without the preoperative needle biopsy for the following reasons: the only sign of an atypical FNH was the absence of central scar, while other findings confirmed the diagnosis. Anyway, the patient had the indications for surgery such as biliary hypertension and the pain. Besides, we planned the intraoperative histological examination to exclude HCC and HCA. However, some authors claim that biopsy is mandatory due to overlapping radiological features of benign and malignant tumors [9,28,29]. At the same time, there are a lot of publications describing the specific CT, MRI and ultrasound visualization data for FNH [6,9,22,30–33]. Non-surgical treatment was not considered as option because it should be reserved for patients unfit for resection [27]. 4. Conclusion Fig. 3. Operative field. 1 – biased gallbladder.
Therefore, this clinical case proves the possibility of giant FNH in very young children under 5 years old. Huge FNH may be accompanied by dilated hepatic vein if it is drained into the system of one hepatic vein. Liver resection in case of it is necessarily leads to decrease the vein diameter. The presence of radiological characteristic features even in case of central scar absence may confirm the FNH and allows to avoid biopsy in selected patients. Conflicts of interest None. Disclosure The authors declare no conflicts of interest. Funding Fig. 4. A - preoperative CT shows dilated right hepatic vein (1); 2 – main trunk of middle hepatic vein; 3 – main trunk of left hepatic vein; 4 – inferior vena cava. B – decreased in diameter right hepatic vein on postoperative CT (1).
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Proliferation of the bile ducts around fibrosis was observed. Hepatocytes were without signs of atypia. The final histopathology was reported as FNG of the liver. The postoperative CT showed reduced to 9 mm diameter of right hepatic vein.
Acknowledgements None. References
3. Discussion
[1] Meyers RL Tumors of the liver in children. Surg Oncol 2007;16:195–203. https:// doi.org/10.1016/j.suronc.2007.07.002. [2] Reymond D, Plaschkes J, Ridolfi Lüthy A, Leibundgut K, Hirt A, Wagner HP. Focal nodular hyperplasia of the liver in children: review of follow-up and outcome. J Pediatr Surg 1995;30(11):1590–3. https://doi.org/10.1016/j.hpb.2017.05.001. [3] Yang Y, Fu S, Li A, Zhou W, Pan Z, Cui L, Huang G, Wu B, Wu M. Management and surgical treatment for focal nodular hyperplasia in children. Pediatr Surg Int 2008;24:699–703. №6. [4] Mathieu D, Vilgrain V, Mahfouz AE, Anglade MC, Vullierme MP, Denys A. Benign liver tumors. Magn Reson Imag Clin N Am 1997;5:255–88. [5] Wanless IR, Mawdsley C, Adams R. On the pathogenesis of focal nodular hyperplasia of the liver. Hepatology 1985;5:1194–200. [6] Cha DI, Yoo SY, Kim JH, Jeon TY, Eo H. Clinical and imaging features of focal nodular hyperplasia in children. AJR Am J Roentgenol 2014 May;202(5):960–5. https://doi.org/10.2214/AJR.13.11856. [7] Mueller BU, Lopez-Terrada D, Finegold MJ. Tumors of the liver. Principles and practice of pediatric oncology. Philadelphia: Lippincott Williams and Wilkins; 2006. p. 887–904.
FNH can be diagnosed at any age but in children the median age of diagnosis was found to be 8.7 years [11,23]. It is difficult to diagnose a benign liver lesion in very young children group (under 5 years old) especially without underlying liver disease, because in this age 75% of the liver tumors are malignant [24]. In the literature review Ma I.T. et al. found 6 patients in this age group with the largest dimension of lesion - 9.4 cm (6.6 ± 3.4 cm) [25]. Zhuang L. et al. reported a case of surgery in 6 years old child with 10.5 × 8x6.5 cm huge FNG as the biggest FNH ever reported among children [26]. We described a huge FNH in 2 years and 7 months old boy. We did not found any mention in the literature, of the size of FNH, which exceeds the size of described patient in this age. 3
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