Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate

Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate Infantile hepatic hemangioma (IHH) and mesenchymal hamartoma (MH) form the first and second most common benign hep- atic tumors in children. In this case report, we present a newborn child in whom a growing hepatic mass was discovered at the age of 7 days. She suffered also from anemia, respiratory and renal failure. No signs of heart disease or cutaneous lesions were detected. Alpha-fetoprotein was elevated for her age (3562.2 ng/ml). Imaging studies showed multifocal large cystic lesions asso- ciated with heterogeneous small solid lesions with arterial enhancement of the liver. Non-anatomical liver resection was per- formed initially and synchronous MH and IHH GLUT-1 positive were discovered in the pathological analysis. Segmental IV hepatic resection and later on selective cysts excision were done for persistent hepatic lesion despite medical treatment. After 14 months of beta-blockers treatment, clinical follow-up shows a healthy child with no residual cystic lesions. diagnosed with pre-eclampsia during her pregnancy which INTRODUCTION eventually mandated the preterm delivery. However, neither Benign hepatic tumors form one-third of all hepatic lesions. fetal abnormalities nor hepatic lesions were detected pre- Infantile hepatic hemangioma (IHH) was found to be the most natally. At the age of 7 weeks (42nd week of corrected date), the frequent benign hepatic tumors followed by mesenchymal hamar- child presented with massive abdominal distention reported by toma (MH), focal nodular hyperplasia (FNH), nodular regenerative the family to be progressively growing since birth. Clinical exam- hyperplasia (NRH) and hepatic adenoma (HA) in a decreasing ination revealed pale tachypneic afebrile child with severely order [1]. Although deficiently reported in the literature, the rare distended abdomen with huge hepatomegaly causing severe co-existence of hepatic hemangioma and MH could be found in respiratory distress and bowel obstruction. No infantile jaun- pediatric age group. This rare association might be considered as dice, dysmorphic features, cutaneous lesions nor other stig- incidental or real concurrence between the two pathologies [2]. mata of liver disease were found. The child was admitted in the neonatal intensive care unit. Abdominal ultrasound showed enlarged liver with multi CASE REPORT nodular hypo-hyperechogenic hepatic lesions with complete We present a currently 34-month-old girl, born prematurely at distortion of hepatic vasculature. Computed tomography (CT) 35th week of gestation due to fetal distress. The mother was scan with contrast injection showed co-existing multifocal Received: January 15, 2017. Accepted: March 12, 2017 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 N. Berte et al. Figure 3: Liver parenchyma invaded by multiple enlarging cysts varied in sizes and shapes. Surprisingly, microscopic analysis of two excised cysts among four showed complex lesions of HR containing multi centric zones of infantile hemangioma staining positively for glucose transporter-1 (GLUT-1+). Medical treatment was started immediately with intraven- Figure 1: Large cystic lesions with internal septation associated with heteroge- ous corticoids (2 mg/kg/day) accompanied with beta-blockers neous small solid lesions with arterial enhancement. (propranolol 2 mg/kg/day). Ultrasound and CT scans performed after 6 months showed scattered liver parenchymal invaded by multiple enlarging cysts varied in sizes and shapes and causing severe abdominal compression (Fig. 3). Segmental IV hepatectomy was performed after 8 months of the first operation. Histopathologic analysis of the tumor showed again the same complex lesion of HR containing infantile hem- angioma GLUT-1+ without any signs of malignancy. The remaining lesions of IHH were treated by large dose of beta-blockers and regressed significantly. However, another subcapsular cystic lesion developed after 6 months from the second operation. Selective surgical excision was performed this time for the newly developed cystic lesion and medical treatment was continued. Today, at the age of 34 months, the child carries a good general condition with satisfying tolerance to her beta-blocker treatment. Ultrasonographic follow-up shows normal regenerative liver parenchyma with a small hemangiomatic lesions measuring <1 cm. Figure 2: Intraoperative view of the hepatic cysts before excision. DISCUSSION hepatic solid and macro-cystic lesions with no hepatic calcifica- tions (Fig. 1). Benign liver tumors count for one-third of all hepatic lesions [3] Measurement of human chorionic gonadotropin was within the while malignant lesions, primarily metastasis, form the other normal range while alpha-fetoprotein was elevated for her age two-thirds. Many studies showed that among these benign (3562.2 ng/ml). Random needle transcutaneous biopsies were done lesions, IHH represent the most common benign tumor of the but unfortunately, histopathological examination was inconclusive. liver in children mostly in the first 6 months of life and Three days later, the patient developed a sudden abdominal accounts for 12% of all pediatric lesions. MH of the liver form compartment syndrome (hemodynamic instability, respiratory 8% of benign lesions and presents frequently as an enlarging and renal failure). Urgent laparotomy and decompressing non- mass in the young children. Nearly 85% of hamartoma lesions anatomical resection through a bilateral subcostal incision was occur in children under 2 years of age and rarely above the age performed. Four hepatic cysts were excised, two from the right of 5 [1]. However, the association between these two lesions is and two from the left liver lobe while many other small non- exceptional and extremely rare in pediatric age group [2]. The compressive cysts were not excised. The removed cysts mea- lesion in these three patients presented differently than our sured between 4 and 9 cm in diameter and between 46 and case. The first child had synchronous lesions of IHH and MH 300 g in weight (Fig. 2). but was separately located in the liver while in the second case; Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Co-existing infantile hepatic hemangioma and mesenchymal hamartoma 3 IHH appeared 6 months after of the surgical treatment of MH. even in clinically unstable patients and programmed secondary In the third case, they were located in the same extensive resection for persistent non-responsive lesions in optimal clin- tumor, but IHH was GLUT-1 stain was negative. ical situation could be a considerable option permitting good Bejarano et al.[4] reported a case of a newborn girl who preservation and regeneration of liver parenchyma in case of underwent surgical resection or large lesion of MH while IHH benign extended tumors. developed lately after 4 months of the excision. Kuang-Hung et al.[5] also conducted a case of a female new- born that had a hepatic mass detected prenatally. Segmental CONFLICT OF INTEREST STATEMENT resection was performed initially and complete resection of None declared. these tumors and left lobectomy were performed 21 days later. Pathological examination showed synchronous lesions of MH and IHH of the same lobe. REFERENCES This case report shows a lot of similarity to our case but managed differently. We performed repeated non-anatomical 1. Stocker JT. Hepatic tumors in children. Clin Liver Dis. 1 févr resections in association with medical treatment for IHH (ster- 2001;5:259–81. oids and beta-blockers in large doses) while Kuang-Hung et al. 2. Behr GG, Fishman SJ, Caty MG, Kulungowski AM, Paltiel HJ, performed only anatomical lobectomy without medical treat- Alomari AI. Hepatic mesenchymal hamartoma and infantile ment reported. Furthermore, no information was given about hemangioma: a rare association. J Pediatr Surg 2012;47: histochemical staining for GLUT-1 in their study. 448–52. Our management composed of both surgical and medical 3. Chung EM, Cube R, Lewis RB, Conran RM. From the archives treatment. First surgery permitted us to obtain decompression of the AFIP: pediatric liver masses: radiologic-pathologic cor- and histopathological diagnosis. Discovery of IHH lesions in relation part 1. Benign tumors. Radiographics 2010;30:801–26. histopathological analysis indicated medical treatment with 4. Bejarano PA, Serrano MF, Casillas J, Dehner LP, Kato T, Mitral beta-blockers. The second and third tumor resections were N, et al. Concurrent infantile hemangioendothelioma and mainly performed for the recurrence of lesions and non- mesenchymal hamartoma in a developmentally arrested responsive to medical treatment. This management helped in liver of an infant requiring hepatic transplantation. Pediatr minimizing the advanced liver resection and regeneration of Dev Pathol 2003;6:552–7. hepatic parenchyma. 5. Hsiao K-H, Lin L-H, Chen D-F, Huang S-H. Hepatic mesenchy- Synchronous IHH and MH are exceptional situations. Its mal hamartoma combined with infantile hepatic heman- treatment could include both medical (beta-blockers) and surgi- gioendothelioma in an infant. J Formos Med Assoc 2007;106: cal excision. Non-anatomical tumor resection in large lesions, S1–4. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate

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Abstract

Infantile hepatic hemangioma (IHH) and mesenchymal hamartoma (MH) form the first and second most common benign hep- atic tumors in children. In this case report, we present a newborn child in whom a growing hepatic mass was discovered at the age of 7 days. She suffered also from anemia, respiratory and renal failure. No signs of heart disease or cutaneous lesions were detected. Alpha-fetoprotein was elevated for her age (3562.2 ng/ml). Imaging studies showed multifocal large cystic lesions asso- ciated with heterogeneous small solid lesions with arterial enhancement of the liver. Non-anatomical liver resection was per- formed initially and synchronous MH and IHH GLUT-1 positive were discovered in the pathological analysis. Segmental IV hepatic resection and later on selective cysts excision were done for persistent hepatic lesion despite medical treatment. After 14 months of beta-blockers treatment, clinical follow-up shows a healthy child with no residual cystic lesions. diagnosed with pre-eclampsia during her pregnancy which INTRODUCTION eventually mandated the preterm delivery. However, neither Benign hepatic tumors form one-third of all hepatic lesions. fetal abnormalities nor hepatic lesions were detected pre- Infantile hepatic hemangioma (IHH) was found to be the most natally. At the age of 7 weeks (42nd week of corrected date), the frequent benign hepatic tumors followed by mesenchymal hamar- child presented with massive abdominal distention reported by toma (MH), focal nodular hyperplasia (FNH), nodular regenerative the family to be progressively growing since birth. Clinical exam- hyperplasia (NRH) and hepatic adenoma (HA) in a decreasing ination revealed pale tachypneic afebrile child with severely order [1]. Although deficiently reported in the literature, the rare distended abdomen with huge hepatomegaly causing severe co-existence of hepatic hemangioma and MH could be found in respiratory distress and bowel obstruction. No infantile jaun- pediatric age group. This rare association might be considered as dice, dysmorphic features, cutaneous lesions nor other stig- incidental or real concurrence between the two pathologies [2]. mata of liver disease were found. The child was admitted in the neonatal intensive care unit. Abdominal ultrasound showed enlarged liver with multi CASE REPORT nodular hypo-hyperechogenic hepatic lesions with complete We present a currently 34-month-old girl, born prematurely at distortion of hepatic vasculature. Computed tomography (CT) 35th week of gestation due to fetal distress. The mother was scan with contrast injection showed co-existing multifocal Received: January 15, 2017. Accepted: March 12, 2017 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 N. Berte et al. Figure 3: Liver parenchyma invaded by multiple enlarging cysts varied in sizes and shapes. Surprisingly, microscopic analysis of two excised cysts among four showed complex lesions of HR containing multi centric zones of infantile hemangioma staining positively for glucose transporter-1 (GLUT-1+). Medical treatment was started immediately with intraven- Figure 1: Large cystic lesions with internal septation associated with heteroge- ous corticoids (2 mg/kg/day) accompanied with beta-blockers neous small solid lesions with arterial enhancement. (propranolol 2 mg/kg/day). Ultrasound and CT scans performed after 6 months showed scattered liver parenchymal invaded by multiple enlarging cysts varied in sizes and shapes and causing severe abdominal compression (Fig. 3). Segmental IV hepatectomy was performed after 8 months of the first operation. Histopathologic analysis of the tumor showed again the same complex lesion of HR containing infantile hem- angioma GLUT-1+ without any signs of malignancy. The remaining lesions of IHH were treated by large dose of beta-blockers and regressed significantly. However, another subcapsular cystic lesion developed after 6 months from the second operation. Selective surgical excision was performed this time for the newly developed cystic lesion and medical treatment was continued. Today, at the age of 34 months, the child carries a good general condition with satisfying tolerance to her beta-blocker treatment. Ultrasonographic follow-up shows normal regenerative liver parenchyma with a small hemangiomatic lesions measuring <1 cm. Figure 2: Intraoperative view of the hepatic cysts before excision. DISCUSSION hepatic solid and macro-cystic lesions with no hepatic calcifica- tions (Fig. 1). Benign liver tumors count for one-third of all hepatic lesions [3] Measurement of human chorionic gonadotropin was within the while malignant lesions, primarily metastasis, form the other normal range while alpha-fetoprotein was elevated for her age two-thirds. Many studies showed that among these benign (3562.2 ng/ml). Random needle transcutaneous biopsies were done lesions, IHH represent the most common benign tumor of the but unfortunately, histopathological examination was inconclusive. liver in children mostly in the first 6 months of life and Three days later, the patient developed a sudden abdominal accounts for 12% of all pediatric lesions. MH of the liver form compartment syndrome (hemodynamic instability, respiratory 8% of benign lesions and presents frequently as an enlarging and renal failure). Urgent laparotomy and decompressing non- mass in the young children. Nearly 85% of hamartoma lesions anatomical resection through a bilateral subcostal incision was occur in children under 2 years of age and rarely above the age performed. Four hepatic cysts were excised, two from the right of 5 [1]. However, the association between these two lesions is and two from the left liver lobe while many other small non- exceptional and extremely rare in pediatric age group [2]. The compressive cysts were not excised. The removed cysts mea- lesion in these three patients presented differently than our sured between 4 and 9 cm in diameter and between 46 and case. The first child had synchronous lesions of IHH and MH 300 g in weight (Fig. 2). but was separately located in the liver while in the second case; Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Co-existing infantile hepatic hemangioma and mesenchymal hamartoma 3 IHH appeared 6 months after of the surgical treatment of MH. even in clinically unstable patients and programmed secondary In the third case, they were located in the same extensive resection for persistent non-responsive lesions in optimal clin- tumor, but IHH was GLUT-1 stain was negative. ical situation could be a considerable option permitting good Bejarano et al.[4] reported a case of a newborn girl who preservation and regeneration of liver parenchyma in case of underwent surgical resection or large lesion of MH while IHH benign extended tumors. developed lately after 4 months of the excision. Kuang-Hung et al.[5] also conducted a case of a female new- born that had a hepatic mass detected prenatally. Segmental CONFLICT OF INTEREST STATEMENT resection was performed initially and complete resection of None declared. these tumors and left lobectomy were performed 21 days later. Pathological examination showed synchronous lesions of MH and IHH of the same lobe. REFERENCES This case report shows a lot of similarity to our case but managed differently. We performed repeated non-anatomical 1. Stocker JT. Hepatic tumors in children. Clin Liver Dis. 1 févr resections in association with medical treatment for IHH (ster- 2001;5:259–81. oids and beta-blockers in large doses) while Kuang-Hung et al. 2. Behr GG, Fishman SJ, Caty MG, Kulungowski AM, Paltiel HJ, performed only anatomical lobectomy without medical treat- Alomari AI. Hepatic mesenchymal hamartoma and infantile ment reported. Furthermore, no information was given about hemangioma: a rare association. J Pediatr Surg 2012;47: histochemical staining for GLUT-1 in their study. 448–52. Our management composed of both surgical and medical 3. Chung EM, Cube R, Lewis RB, Conran RM. From the archives treatment. First surgery permitted us to obtain decompression of the AFIP: pediatric liver masses: radiologic-pathologic cor- and histopathological diagnosis. Discovery of IHH lesions in relation part 1. Benign tumors. Radiographics 2010;30:801–26. histopathological analysis indicated medical treatment with 4. Bejarano PA, Serrano MF, Casillas J, Dehner LP, Kato T, Mitral beta-blockers. The second and third tumor resections were N, et al. Concurrent infantile hemangioendothelioma and mainly performed for the recurrence of lesions and non- mesenchymal hamartoma in a developmentally arrested responsive to medical treatment. This management helped in liver of an infant requiring hepatic transplantation. Pediatr minimizing the advanced liver resection and regeneration of Dev Pathol 2003;6:552–7. hepatic parenchyma. 5. Hsiao K-H, Lin L-H, Chen D-F, Huang S-H. Hepatic mesenchy- Synchronous IHH and MH are exceptional situations. Its mal hamartoma combined with infantile hepatic heman- treatment could include both medical (beta-blockers) and surgi- gioendothelioma in an infant. J Formos Med Assoc 2007;106: cal excision. Non-anatomical tumor resection in large lesions, S1–4. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/1/rjx260/4822188 by Ed 'DeepDyve' Gillespie user on 16 March 2018

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