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Case Report

Ann Liver Transplant 2021; 1(1): 105-111

Published online May 31, 2021 https://doi.org/10.52604/alt.21.0008

Copyright © The Korean Liver Transplantation Society.

Living donor liver transplantation in a pediatric patient with hepatic angiosarcoma: a case report

Jung-Man Namgoong1 , Shin Hwang2 , Gil-Chun Park2 , Suhyeon Ha1 , Seak Hee Oh3 , Kyung Mo Kim3

Divisions of 1Pediatric Surgery and 2Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine
3Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence to:Shin Hwang
Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
E-mail: shwang@amc.seoul.kr
https://orcid.org/0000-0002-9045-2531

Received: April 5, 2021; Revised: April 12, 2021; Accepted: April 18, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/bync/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Hepatic angiosarcoma (HAS) is a rare malignant disease in pediatric patients. We report the case of a 3‐year‐old boy with HAS, which was treated with neoadjuvant chemotherapy and living donor liver transplantation (LDLT). A previously healthy 3‐year‐old boy who presented with a firm mass in the upper quadrant of the abdomen was diagnosed with hepatoblastoma at a local general hospital and was referred to our institution. Percutaneous liver biopsy confirmed the diagnosis of HAS. The extent of the tumor was large, not allowing surgical resection; thus neoadjuvant chemotherapy was performed. The size of the tumor was markedly reduced after 2 cycles of chemotherapy for 2 months; thus LDLT was planned to remove the tumor completely. A left lateral section graft weighing 280 g was harvested from his 38-year-old father. The left lateral section graft was implanted according to the routine procedures of pediatric LDLT, including patch venoplasty of the recipient hepatic vein and portal vein. The explant liver showed a 9 cm-sized residual angiosarcoma with 60% regression. The patient recovered uneventfully and is doing well for 3 months with scheduled adjuvant chemotherapy. Although there are only a few pediatric liver transplantation cases showing prolonged survival, liver transplantation appears to be a viable treatment option for long‐term survival for pediatric patients with unresectable HAS.

Keywords: Hepatic malignancy, Chemotherapy, Venoplasty, Pediatric transplantation, Recurrence

Hepatic angiosarcoma (HAS) is a rare malignant disease in pediatric patients and it is found more often in adults. It is difficult to distinguish this disease from other vascular anomalies, such as infantile hemangioendothelioma. Malignant liver tumors account for approximately 1% of childhood malignancies and HAS comprises only 1‐2% of pediatric liver tumors [1,2]. Less than 50 pediatric cases have been reported in the literature worldwide [2,3]. The prognosis of HAS is very poor, regardless of the treatment or stage, with mean survival time ranging from only 10 months to 2 years [2,4]. Only six cases of disease‐free survival have been reported in the literature [3,5]. Complete surgical resection appears to provide the best chance for long‐term survival, but many patients have metastatic disease or their tumors are unresectable. Liver transplantation (LT) in such patients is controversial and often contraindicated because of the posttransplant tumor recurrence and mortality rates. Systemic chemotherapy yielded mixed results, but long‐term remission was largely not achievable.

We report the case of a 3‐year‐old boy with HAS, which was treated with neoadjuvant chemotherapy and living donor liver transplantation (LDLT).

A previously healthy 3‐year‐old boy who presented with a firm mass in the upper quadrant of the abdomen was diagnosed with hepatoblastoma at a local general hospital and was referred to our institution. The initial imaging diagnosis was hepatoblastoma with portal vein tumor thrombus, PRE-Treatment EXTent of tumor (PRETEXT) stage IV (Fig. 1). Percutaneous liver biopsy showed angiosarcoma, with immunohistochemical findings of CD31 positivity, non-contributory ERG, HEP PAR-1 negativity, and P53 negativity. The extent of the tumor was too large, not allowing surgical resection; thus neoadjuvant chemotherapy according to the Children's Oncology Group ARST0332 study using doxorubicin and ifosfamide was performed [6]. No significant side-effects occurred, with a normal-structured heart and an ejection fraction of 67%. The size of the tumor was markedly reduced after 2 cycles of chemotherapy for 2 months (Fig. 2); thus LDLT was planned to remove the tumor completely.

Figure 1.Initial radiologic study findings. At three months before the liver transplantation operation, computed tomography (A, B) and magnetic resonance imaging (C) studies show huge multiple tumors occupying the whole liver, and the abdomen is distended by the enlarged liver. Whole-body positron emission tomography (D) shows a heterogenous mild hypermetabolic huge mass in the liver. The diagnosis from these imaging studies is hepatoblastoma and differential diagnoses include solid variant of mesenchymal harmatoma, inflammatory pseudotumor and hemangioma.

Figure 2.Pretransplant computed tomography findings. The tumors show noticeable interval decrease in size (A), encasement of the middle and left hepatic veins (B), encasement of the right and left portal veins (C), and encasement of the second-order right hepatic artery branches (D).

LDLT operations were performed in both the donor and recipient according to the routine procedures. Pretransplant computed tomography findings suggested that the left hepatic vein trunk, the confluence of the hilar portal vein and the second-order branch of the right hepatic artery were suspected to show tumor involvement (Fig. 2); thus dissection of the recipient liver was limited and it did not include dissection of these involved areas (Fig. 3).

Figure 3.Intraoperative findings showing the extent of hepatic angiosarcoma at the dome (A) and hilar area (B).

A left lateral section graft weighing 280 g was harvested from his 38-year-old father. The graft left hepatic vein orifice was enlarged with patch venoplasty according to our institutional guidelines [7,8].

During recipient hepatectomy, the right and left hepatic arteries were transected below the involved tumor portion. The hilar portal vein was transected distal to the confluence portion. The recipient common bile duct was transected and closed. The major hepatic vein stumps were transected without leaving any hepatic parenchymal tissues. Because the tumor was located very close to the left hepatic vein trunk, the left hepatic vein stump was resected further and the resection margin was sent for frozen section biopsy, which revealed a tumor-negative finding. The left hepatic vein stump was very short and could not be reconstructed directly; thus a small patch of iliac vein homograft was attached to facilitate graft hepatic vein reconstruction (Fig. 4). Since the recipient portal vein appeared relatively small compared with the graft portal vein and a branch patch was not available, a small iliac vein homograft patch was attached to widen the portal vein orifice (Fig. 5).

Figure 4.Intraoperative findings showing patch venoplasty of the recipient hepatic vein stump. (A) The major hepatic vein stumps are transected without leaving any hepatic parenchymal tissues. (B) Because the tumor is located very close to the left hepatic vein trunk, the left hepatic vein stump is resected further. (C, D) The left hepatic vein stump appears to be very short and cannot be reconstructed directly; thus a small patch of iliac vein homograft (arrows) is attached.

Figure 5.Intraoperative findings showing patch venoplasty of the recipient portal vein. (A) The recipient portal vein appears relatively small due to removal of the hilar bifurcation portion. (B) A small longitudinal incision is made at the portal vein stump. (C, D) A small iliac vein homograft patch is attached to enlarge the portal vein orifice.

A left lateral graft was implanted according to the routine procedures of pediatric LDLT, including size-matched reconstruction of the graft hepatic vein and portal vein, single left hepatic artery reconstruction using surgical microscopy, and Roux-en-Y hepaticojejunostomy (Fig. 6).

Figure 6.Intraoperative findings showing implantation of the left lateral section graft. (A–C) Size-matched reconstruction of the graft hepatic vein. The intervening vein patch is visible (arrow). (D, E) Size-matched reconstruction of the graft portal vein. The intervening vein patch is visible (arrow). (F) Gross finding of the left lateral section graft after portal vein reperfusion.

The explant liver showed residual angiosarcoma measuring 9.2 cm × 7.8 cm × 5.2 cm in size in segments II, IV, V, VI, VII and VIII (Fig. 7). The tumor showed moderate grade of regression, in which the regressed area was approximately 60%. There was presence of Glisson capsule invasion with absence of lymphovascular invasion and perineural invasion.

Figure 7.Gross photographs of the explanted liver. There is a large viable tumor with extensive necrosis.

This patient recovered uneventfully from the LDLT operation (Fig. 8) and is doing well for 3 months to date. He is currently undergoing scheduled adjuvant chemotherapy.

Figure 8.Computed tomography findings at five days after transplantation. (A) The graft hepatic vein reconstruction shows only a slight stenosis at the anastomotic site (arrow). (B) The funnel-shaped recipient portal vein is well visualized (arrow).

Pediatric HAS is a rare and aggressive vascular malignant tumor of endothelial cell origin that often presents with a rapidly enlarging abdominal mass and occasional pain [4]. The overall prognosis of HAS is very poor regardless of the treatment modalities. Because of the rarity of this tumor, there are no treatment consensus guidelines as yet. Treatment approaches vary widely, but they often include surgical treatment combined with systemic therapy. The results of treatment are mixed, but the overall outcomes and prognosis remain very poor.

Platinum‐based chemotherapy regimens have often been attempted, as well as targeted therapy with sirolimus and vascular endothelial growth factor inhibitors including bevacizumab [2,5]. A sizable tumor response to bevacizumab, gemcitabine, and docetaxel was reported in a case of metastatic HAS, but the pediatric patient died after 16 cycles of chemotherapy [9]. Adriamycin combined with cisplatin was reported to significantly reduce the size of an unresectable HAS [10]. After complete resection and over one year of alternating courses of varying cytotoxic agents, the patient remained disease‐free for 44 months [11]. Sclerotherapy, directed embolization, and radiation therapy have also been attempted, with little success [2]. Although many different agents have been tried with varying benefit as briefly commented above, the majority of patients never achieve remission or long‐term survival.

Complete surgical resection appears to provide the best survival advantage for children with HAS; however, LT in such patients remains controversial because of the poor long‐term survival outcomes. A retrospective review of the European Liver Transplant Registry concluded that HAS should be considered an absolute contraindication to LT due to poor outcomes [11]. The majority of patients with HAS will succumb to the diagnosis or related complications, and there are only a few reports of long‐term survival. Among the six reported surviving pediatric patients with HAS, all patients underwent successful tumor excision with four patients undergoing LT and were still disease‐free at the time of publication [1-3]. Interestingly, five out of these six patients were not diagnosed with HAS until after surgical excision or LT.

Our case report presents a patient with localized HAS that was not amenable to surgical resection and who was treated with neoadjuvant chemotherapy and LDLT. Although there are only a few pediatric LT cases showing prolonged survival, LT appears to be a viable treatment option for long‐term survival of pediatric patients with unresectable HAS.


All authors have no conflicts of interest to declare.


Conceptualization: SH. Data curation: GCP, SH. Methodology: SHO, KMK. Visualization: SH. Writing - original draft: SH, JMN. Writing - review & editing: SH.

  1. Xue M, Masand P, Thompson P, Finegold M, Leung DH. Angiosarcoma successfully treated with liver transplantation and sirolimus. Pediatr Transplant 2014;18:E114-E119.
    Pubmed CrossRef
  2. Grassia KL, Peterman CM, Iacobas I, Margolin JF, Bien E, Padhye B, et al. Clinical case series of pediatric hepatic angiosarcoma. Pediatr Blood Cancer 2017;64:e26627.
    Pubmed CrossRef
  3. Pilbeam K, Eidenschink B, Sulciner M, Luquette M, Neglia J, Chinnakotla S. Success of chemotherapy and a liver transplant in a pediatric patient with hepatic angiosarcoma: a case report. Pediatr Transplant 2019;23:e13410.
    Pubmed CrossRef
  4. Geramizadeh B, Safari A, Bahador A, Nikeghbalian S, Salahi H, Kazemi K, et al. Hepatic angiosarcoma of childhood: a case report and review of literature. J Pediatr Surg 2011;46:e9-e11.
    Pubmed CrossRef
  5. Potanos KM, Hodgkinson N, Fullington NM, Narla A, Albritton K, Kozakewich H, et al. Long term survival in pediatric hepatic angiosarcoma (PHAS): a case report and review of the literature. J Pediatr Surg Case Rep 2015;3:410‐413.
    CrossRef
  6. Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, et al. A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study. Lancet Oncol 2020;21:145-161.
    CrossRef
  7. Namgoong JM, Hwang S, Park GC, Ahn CS, Kim KH, Kim KM, et al. Outflow vein venoplasty of left lateral section graft for living donor liver transplantation in infant recipients. Pediatr Transplant 2021. [Epub ahead of print]
    Pubmed CrossRef
  8. Hwang S, Kim KH, Kim DY, Kim KM, Ahn CS, Moon DB, et al. Anomalous hepatic vein anatomy of left lateral section grafts and customized unification venoplasty for pediatric living donor liver transplantation. Liver Transpl 2013;19:184-190.
    Pubmed CrossRef
  9. Jeng MR, Fuh B, Blatt J, Gupta A, Merrow AC, Hammill A, et al. Malignant transformation of infantile hemangioma to angiosarcoma: response to chemotherapy with bevacizumab. Pediatr Blood Cancer 2014;61:2115-2117.
    Pubmed CrossRef
  10. Gunawardena SW, Trautwein LM, Finegold MJ, Ogden AK. Hepatic angiosarcoma in a child: successful therapy with surgery and adjuvant chemotherapy. Med Pediatr Oncol 1997;28:139-143.
    CrossRef
  11. Orlando G, Adam R, Mirza D, Soderdahl G, Porte RJ, Paul A, et al. Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation--the European Liver Transplant Registry experience. Transplantation 2013;95:872-877.
    Pubmed CrossRef
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Article

Case Report

Ann Liver Transplant 2021; 1(1): 105-111

Published online May 31, 2021 https://doi.org/10.52604/alt.21.0008

Copyright © The Korean Liver Transplantation Society.

Living donor liver transplantation in a pediatric patient with hepatic angiosarcoma: a case report

Jung-Man Namgoong1 , Shin Hwang2 , Gil-Chun Park2 , Suhyeon Ha1 , Seak Hee Oh3 , Kyung Mo Kim3

Divisions of 1Pediatric Surgery and 2Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine
3Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence to:Shin Hwang
Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
E-mail: shwang@amc.seoul.kr
https://orcid.org/0000-0002-9045-2531

Received: April 5, 2021; Revised: April 12, 2021; Accepted: April 18, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/bync/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hepatic angiosarcoma (HAS) is a rare malignant disease in pediatric patients. We report the case of a 3‐year‐old boy with HAS, which was treated with neoadjuvant chemotherapy and living donor liver transplantation (LDLT). A previously healthy 3‐year‐old boy who presented with a firm mass in the upper quadrant of the abdomen was diagnosed with hepatoblastoma at a local general hospital and was referred to our institution. Percutaneous liver biopsy confirmed the diagnosis of HAS. The extent of the tumor was large, not allowing surgical resection; thus neoadjuvant chemotherapy was performed. The size of the tumor was markedly reduced after 2 cycles of chemotherapy for 2 months; thus LDLT was planned to remove the tumor completely. A left lateral section graft weighing 280 g was harvested from his 38-year-old father. The left lateral section graft was implanted according to the routine procedures of pediatric LDLT, including patch venoplasty of the recipient hepatic vein and portal vein. The explant liver showed a 9 cm-sized residual angiosarcoma with 60% regression. The patient recovered uneventfully and is doing well for 3 months with scheduled adjuvant chemotherapy. Although there are only a few pediatric liver transplantation cases showing prolonged survival, liver transplantation appears to be a viable treatment option for long‐term survival for pediatric patients with unresectable HAS.

Keywords: Hepatic malignancy, Chemotherapy, Venoplasty, Pediatric transplantation, Recurrence

INTRODUCTION

Hepatic angiosarcoma (HAS) is a rare malignant disease in pediatric patients and it is found more often in adults. It is difficult to distinguish this disease from other vascular anomalies, such as infantile hemangioendothelioma. Malignant liver tumors account for approximately 1% of childhood malignancies and HAS comprises only 1‐2% of pediatric liver tumors [1,2]. Less than 50 pediatric cases have been reported in the literature worldwide [2,3]. The prognosis of HAS is very poor, regardless of the treatment or stage, with mean survival time ranging from only 10 months to 2 years [2,4]. Only six cases of disease‐free survival have been reported in the literature [3,5]. Complete surgical resection appears to provide the best chance for long‐term survival, but many patients have metastatic disease or their tumors are unresectable. Liver transplantation (LT) in such patients is controversial and often contraindicated because of the posttransplant tumor recurrence and mortality rates. Systemic chemotherapy yielded mixed results, but long‐term remission was largely not achievable.

We report the case of a 3‐year‐old boy with HAS, which was treated with neoadjuvant chemotherapy and living donor liver transplantation (LDLT).

CASE PRESENTATION

A previously healthy 3‐year‐old boy who presented with a firm mass in the upper quadrant of the abdomen was diagnosed with hepatoblastoma at a local general hospital and was referred to our institution. The initial imaging diagnosis was hepatoblastoma with portal vein tumor thrombus, PRE-Treatment EXTent of tumor (PRETEXT) stage IV (Fig. 1). Percutaneous liver biopsy showed angiosarcoma, with immunohistochemical findings of CD31 positivity, non-contributory ERG, HEP PAR-1 negativity, and P53 negativity. The extent of the tumor was too large, not allowing surgical resection; thus neoadjuvant chemotherapy according to the Children's Oncology Group ARST0332 study using doxorubicin and ifosfamide was performed [6]. No significant side-effects occurred, with a normal-structured heart and an ejection fraction of 67%. The size of the tumor was markedly reduced after 2 cycles of chemotherapy for 2 months (Fig. 2); thus LDLT was planned to remove the tumor completely.

Figure 1. Initial radiologic study findings. At three months before the liver transplantation operation, computed tomography (A, B) and magnetic resonance imaging (C) studies show huge multiple tumors occupying the whole liver, and the abdomen is distended by the enlarged liver. Whole-body positron emission tomography (D) shows a heterogenous mild hypermetabolic huge mass in the liver. The diagnosis from these imaging studies is hepatoblastoma and differential diagnoses include solid variant of mesenchymal harmatoma, inflammatory pseudotumor and hemangioma.

Figure 2. Pretransplant computed tomography findings. The tumors show noticeable interval decrease in size (A), encasement of the middle and left hepatic veins (B), encasement of the right and left portal veins (C), and encasement of the second-order right hepatic artery branches (D).

LDLT operations were performed in both the donor and recipient according to the routine procedures. Pretransplant computed tomography findings suggested that the left hepatic vein trunk, the confluence of the hilar portal vein and the second-order branch of the right hepatic artery were suspected to show tumor involvement (Fig. 2); thus dissection of the recipient liver was limited and it did not include dissection of these involved areas (Fig. 3).

Figure 3. Intraoperative findings showing the extent of hepatic angiosarcoma at the dome (A) and hilar area (B).

A left lateral section graft weighing 280 g was harvested from his 38-year-old father. The graft left hepatic vein orifice was enlarged with patch venoplasty according to our institutional guidelines [7,8].

During recipient hepatectomy, the right and left hepatic arteries were transected below the involved tumor portion. The hilar portal vein was transected distal to the confluence portion. The recipient common bile duct was transected and closed. The major hepatic vein stumps were transected without leaving any hepatic parenchymal tissues. Because the tumor was located very close to the left hepatic vein trunk, the left hepatic vein stump was resected further and the resection margin was sent for frozen section biopsy, which revealed a tumor-negative finding. The left hepatic vein stump was very short and could not be reconstructed directly; thus a small patch of iliac vein homograft was attached to facilitate graft hepatic vein reconstruction (Fig. 4). Since the recipient portal vein appeared relatively small compared with the graft portal vein and a branch patch was not available, a small iliac vein homograft patch was attached to widen the portal vein orifice (Fig. 5).

Figure 4. Intraoperative findings showing patch venoplasty of the recipient hepatic vein stump. (A) The major hepatic vein stumps are transected without leaving any hepatic parenchymal tissues. (B) Because the tumor is located very close to the left hepatic vein trunk, the left hepatic vein stump is resected further. (C, D) The left hepatic vein stump appears to be very short and cannot be reconstructed directly; thus a small patch of iliac vein homograft (arrows) is attached.

Figure 5. Intraoperative findings showing patch venoplasty of the recipient portal vein. (A) The recipient portal vein appears relatively small due to removal of the hilar bifurcation portion. (B) A small longitudinal incision is made at the portal vein stump. (C, D) A small iliac vein homograft patch is attached to enlarge the portal vein orifice.

A left lateral graft was implanted according to the routine procedures of pediatric LDLT, including size-matched reconstruction of the graft hepatic vein and portal vein, single left hepatic artery reconstruction using surgical microscopy, and Roux-en-Y hepaticojejunostomy (Fig. 6).

Figure 6. Intraoperative findings showing implantation of the left lateral section graft. (A–C) Size-matched reconstruction of the graft hepatic vein. The intervening vein patch is visible (arrow). (D, E) Size-matched reconstruction of the graft portal vein. The intervening vein patch is visible (arrow). (F) Gross finding of the left lateral section graft after portal vein reperfusion.

The explant liver showed residual angiosarcoma measuring 9.2 cm × 7.8 cm × 5.2 cm in size in segments II, IV, V, VI, VII and VIII (Fig. 7). The tumor showed moderate grade of regression, in which the regressed area was approximately 60%. There was presence of Glisson capsule invasion with absence of lymphovascular invasion and perineural invasion.

Figure 7. Gross photographs of the explanted liver. There is a large viable tumor with extensive necrosis.

This patient recovered uneventfully from the LDLT operation (Fig. 8) and is doing well for 3 months to date. He is currently undergoing scheduled adjuvant chemotherapy.

Figure 8. Computed tomography findings at five days after transplantation. (A) The graft hepatic vein reconstruction shows only a slight stenosis at the anastomotic site (arrow). (B) The funnel-shaped recipient portal vein is well visualized (arrow).

DISCUSSION

Pediatric HAS is a rare and aggressive vascular malignant tumor of endothelial cell origin that often presents with a rapidly enlarging abdominal mass and occasional pain [4]. The overall prognosis of HAS is very poor regardless of the treatment modalities. Because of the rarity of this tumor, there are no treatment consensus guidelines as yet. Treatment approaches vary widely, but they often include surgical treatment combined with systemic therapy. The results of treatment are mixed, but the overall outcomes and prognosis remain very poor.

Platinum‐based chemotherapy regimens have often been attempted, as well as targeted therapy with sirolimus and vascular endothelial growth factor inhibitors including bevacizumab [2,5]. A sizable tumor response to bevacizumab, gemcitabine, and docetaxel was reported in a case of metastatic HAS, but the pediatric patient died after 16 cycles of chemotherapy [9]. Adriamycin combined with cisplatin was reported to significantly reduce the size of an unresectable HAS [10]. After complete resection and over one year of alternating courses of varying cytotoxic agents, the patient remained disease‐free for 44 months [11]. Sclerotherapy, directed embolization, and radiation therapy have also been attempted, with little success [2]. Although many different agents have been tried with varying benefit as briefly commented above, the majority of patients never achieve remission or long‐term survival.

Complete surgical resection appears to provide the best survival advantage for children with HAS; however, LT in such patients remains controversial because of the poor long‐term survival outcomes. A retrospective review of the European Liver Transplant Registry concluded that HAS should be considered an absolute contraindication to LT due to poor outcomes [11]. The majority of patients with HAS will succumb to the diagnosis or related complications, and there are only a few reports of long‐term survival. Among the six reported surviving pediatric patients with HAS, all patients underwent successful tumor excision with four patients undergoing LT and were still disease‐free at the time of publication [1-3]. Interestingly, five out of these six patients were not diagnosed with HAS until after surgical excision or LT.

Our case report presents a patient with localized HAS that was not amenable to surgical resection and who was treated with neoadjuvant chemotherapy and LDLT. Although there are only a few pediatric LT cases showing prolonged survival, LT appears to be a viable treatment option for long‐term survival of pediatric patients with unresectable HAS.

FUNDING


None of the authors were supported financially for this study.

CONFLICT OF INTEREST


All authors have no conflicts of interest to declare.

AUTHORS’ CONTRIBUTIONS


Conceptualization: SH. Data curation: GCP, SH. Methodology: SHO, KMK. Visualization: SH. Writing - original draft: SH, JMN. Writing - review & editing: SH.

Fig 1.

Figure 1.Initial radiologic study findings. At three months before the liver transplantation operation, computed tomography (A, B) and magnetic resonance imaging (C) studies show huge multiple tumors occupying the whole liver, and the abdomen is distended by the enlarged liver. Whole-body positron emission tomography (D) shows a heterogenous mild hypermetabolic huge mass in the liver. The diagnosis from these imaging studies is hepatoblastoma and differential diagnoses include solid variant of mesenchymal harmatoma, inflammatory pseudotumor and hemangioma.
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 2.

Figure 2.Pretransplant computed tomography findings. The tumors show noticeable interval decrease in size (A), encasement of the middle and left hepatic veins (B), encasement of the right and left portal veins (C), and encasement of the second-order right hepatic artery branches (D).
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 3.

Figure 3.Intraoperative findings showing the extent of hepatic angiosarcoma at the dome (A) and hilar area (B).
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 4.

Figure 4.Intraoperative findings showing patch venoplasty of the recipient hepatic vein stump. (A) The major hepatic vein stumps are transected without leaving any hepatic parenchymal tissues. (B) Because the tumor is located very close to the left hepatic vein trunk, the left hepatic vein stump is resected further. (C, D) The left hepatic vein stump appears to be very short and cannot be reconstructed directly; thus a small patch of iliac vein homograft (arrows) is attached.
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 5.

Figure 5.Intraoperative findings showing patch venoplasty of the recipient portal vein. (A) The recipient portal vein appears relatively small due to removal of the hilar bifurcation portion. (B) A small longitudinal incision is made at the portal vein stump. (C, D) A small iliac vein homograft patch is attached to enlarge the portal vein orifice.
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 6.

Figure 6.Intraoperative findings showing implantation of the left lateral section graft. (A–C) Size-matched reconstruction of the graft hepatic vein. The intervening vein patch is visible (arrow). (D, E) Size-matched reconstruction of the graft portal vein. The intervening vein patch is visible (arrow). (F) Gross finding of the left lateral section graft after portal vein reperfusion.
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 7.

Figure 7.Gross photographs of the explanted liver. There is a large viable tumor with extensive necrosis.
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

Fig 8.

Figure 8.Computed tomography findings at five days after transplantation. (A) The graft hepatic vein reconstruction shows only a slight stenosis at the anastomotic site (arrow). (B) The funnel-shaped recipient portal vein is well visualized (arrow).
Annals of Liver Transplantation 2021; 1: 105-111https://doi.org/10.52604/alt.21.0008

References

  1. Xue M, Masand P, Thompson P, Finegold M, Leung DH. Angiosarcoma successfully treated with liver transplantation and sirolimus. Pediatr Transplant 2014;18:E114-E119.
    Pubmed CrossRef
  2. Grassia KL, Peterman CM, Iacobas I, Margolin JF, Bien E, Padhye B, et al. Clinical case series of pediatric hepatic angiosarcoma. Pediatr Blood Cancer 2017;64:e26627.
    Pubmed CrossRef
  3. Pilbeam K, Eidenschink B, Sulciner M, Luquette M, Neglia J, Chinnakotla S. Success of chemotherapy and a liver transplant in a pediatric patient with hepatic angiosarcoma: a case report. Pediatr Transplant 2019;23:e13410.
    Pubmed CrossRef
  4. Geramizadeh B, Safari A, Bahador A, Nikeghbalian S, Salahi H, Kazemi K, et al. Hepatic angiosarcoma of childhood: a case report and review of literature. J Pediatr Surg 2011;46:e9-e11.
    Pubmed CrossRef
  5. Potanos KM, Hodgkinson N, Fullington NM, Narla A, Albritton K, Kozakewich H, et al. Long term survival in pediatric hepatic angiosarcoma (PHAS): a case report and review of the literature. J Pediatr Surg Case Rep 2015;3:410‐413.
    CrossRef
  6. Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, et al. A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study. Lancet Oncol 2020;21:145-161.
    CrossRef
  7. Namgoong JM, Hwang S, Park GC, Ahn CS, Kim KH, Kim KM, et al. Outflow vein venoplasty of left lateral section graft for living donor liver transplantation in infant recipients. Pediatr Transplant 2021. [Epub ahead of print]
    Pubmed CrossRef
  8. Hwang S, Kim KH, Kim DY, Kim KM, Ahn CS, Moon DB, et al. Anomalous hepatic vein anatomy of left lateral section grafts and customized unification venoplasty for pediatric living donor liver transplantation. Liver Transpl 2013;19:184-190.
    Pubmed CrossRef
  9. Jeng MR, Fuh B, Blatt J, Gupta A, Merrow AC, Hammill A, et al. Malignant transformation of infantile hemangioma to angiosarcoma: response to chemotherapy with bevacizumab. Pediatr Blood Cancer 2014;61:2115-2117.
    Pubmed CrossRef
  10. Gunawardena SW, Trautwein LM, Finegold MJ, Ogden AK. Hepatic angiosarcoma in a child: successful therapy with surgery and adjuvant chemotherapy. Med Pediatr Oncol 1997;28:139-143.
    CrossRef
  11. Orlando G, Adam R, Mirza D, Soderdahl G, Porte RJ, Paul A, et al. Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation--the European Liver Transplant Registry experience. Transplantation 2013;95:872-877.
    Pubmed CrossRef