검색
검색 팝업 닫기

Ex) Article Title, Author, Keywords

Current Issue

Split Viewer

Review Article

Ann Liver Transplant 2023; 3(2): 69-72

Published online November 30, 2023 https://doi.org/10.52604/alt.23.0017

Copyright © The Korean Liver Transplantation Society.

Liver transplantation for giant hepatic hemangioma: A collective review

Cheon-Soo Park1 , Yong-Kyu Chung2

1Department of Surgery, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
2Department of Surgery, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea

Correspondence to:Cheon-Soo Park
Department of Surgery, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 1021 Tongil-ro, Eunpyeong-gu, Seoul 03312, Korea
E-mail: pskys74@hanmail.net
https://orcid.org/0000-0002-6150-702X

Received: October 9, 2023; Revised: November 5, 2023; Accepted: November 9, 2023

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.

Giant hepatic hemangiomas (HHs) have been often considered benign, but their huge size and potential complications can make them far from benign in their impact on the lives. Amid the myriad of treatment options available, liver transplantation (LT) has been performed for giant HHs that have serious clinical manifestations. This study reviewed the role of LT as an indication for giant HH. The literature was searched using search terms as follows in all possible combinations: “liver hemangioma”, “giant hemangioma”, “liver transplantation”, and “Kasabach-Merritt syndrome”. A total of 18 studies were finally included in the present analysis, which recruited 19 patients. The mean age of patients was 39.9±8.6 years. The most common reported symptoms were abdominal distention, respiratory distress, upper abdominal pain, excessive bleeding, and coagulopathy. Preoperative laboratory tests showed abnormal liver function in all patients. Nine patients were diagnosed with Kasabach-Merritt syndrome. Fourteen patients received deceased donor LT whereas the remaining 5 patients underwent living donor LT. No intraoperative or postoperative 90-day mortality following LT was documented. All abnormal blood parameters returned to normal within few days postoperatively in all patients. All patients were alive at the time of their documentation. In conclusion, LT is a safe and effective treatment option in the management of symptomatic or complicated giant HH in selected patients.

Keywords: Hemangioma, Liver transplantation, Abdominal distension, Kasabach-Merritt syndrome, Coagulopathy

In the intricate landscape of liver diseases, one condition that stands as an enigma is the giant hepatic hemangioma (HH). These massive vascular tumors, characterized by their formidable size, pose unique challenges to patients and healthcare providers alike [1]. Giant HHs are often considered benign, but their huger size and potential complications can make them far from benign in their impact on the lives of those affected [2]. Amid the myriad of treatment options available, liver transplantation (LT) has been performed for HHs that have serious clinical manifestations.

This study reviewed the role of LT as an indication for giant HH. Through an exploration of indications, diagnostic criteria, and the clinical rationale behind this decision, we aim to provide a comprehensive understanding of why LT stands as a pivotal strategy in the management of giant HH.

A meticulous search of the literature was performed for studies published up to September 2023 for cases of patients with giant HH who underwent LT. The literature was searched using search terms as follows in all possible combinations: “liver hemangioma,” “giant hemangioma,” “liver transplantation,” and “Kasabach-Merritt syndrome.” Literature in English was selected. All articles with statement of demographics (age, sex), symptoms, diagnostic evaluation, tumor characteristics (location, histology, size, weight), and post-transplantation outcomes (mortality, length of stay, complications) of patients with giant HH who undergo LT were evaluated.

A total of 18 studies were finally included in the present analysis, which recruited 19 patients, as shown in Table 1 [3-20]. The mean age of patients was 39.9±8.6 years. The most common reported symptoms were abdominal distention, respiratory distress, upper abdominal pain, excessive bleeding, and coagulopathy. Preoperative laboratory tests showed abnormal liver function in all patients (high aspartate transaminase and alanine transaminase levels, low platelet count, prolongation of prothrombin time, and of activated partial thromboplastin time, low fibrinogen, elevated D-dimers). Nine patients were diagnosed with Kasabach-Merritt syndrome.

Table 1 Collective review of literature on liver transplantation (LT) for giant hepatic hemangioma

Author (year)Age (yr)/sexPretransplant clinical symptomsKMSHemangioma size (cm)LT typeShort-term outcomeRe-LT
Zhao et al. (2022) [3]36/MAbdominal distentionNoNADDAliveNo
Zhao and Legan (2022) [4]39/FIntra-abdominal hemorrhagesYes14.3×14.6×14.9DDAliveNo
Eghlimi et al. (2020) [5]38/MAbdominal distention, leg edemaNo32.4×26×3.1DDAliveNo
Lee et al. (2018) [6]50/FUpper abdominal pain, abdominal distentionNo16LDAliveNo
Lange et al. (2015) [7]46/FAbdominal distentionNo21.7×23.7×25.5DDAliveNo
Yildiz et al. (2014) [8]44/FAbdominal distention, shortness of breathYes22×18×23DDAliveNo
Zhong et al. (2014) [9]27/FUpper abdominal pain, abdominal distentionNo50×40×25LDAliveNo
Unal et al. (2011) [10]56/FUpper abdominal painYesN/ADDAliveNo
Vagefi et al. (2011) [11]32/FAbdominal distentionYes18×23DDAliveNo
Aseni et al. (2010) [12]46/MPulmonary thromboembolismNoN/ADDAliveNo
Meguro et al. (2008) [13]45/FAbdominal distentionYes15LDAliveNo
Ferraz et al. (2004) [14]25/FProgressive abdominal distentionYes46×40×15DDAliveNo
Kumashiro et al. (2002) [15]48/FAbdominal distensionYesN/ALDAliveNo
Keegan et al. (2001) [16]34/MRespiratory distress, persistent abdominal distentionNo63×45×51DDAliveNo
Longeville et al. (1997) [17]47/MExcessive bleeding after teeth extractionYes25DDAliveNo
Russo et al. (1997) [18]43/FAbdominal distensionNo21DDAliveNo
Chui et al. (1996) [19]33/FUpper abdominal pain, exertional dyspneaNoN/ADDAliveYes
43/FGradually enlarged liver during second pregnancyNo40×35×15DDAliveNo
Klompmaker et al. (1989) [20]27/MHepatomegalyYesN/ALDAliveNo

KMS, Kasabach-Merritt syndrome; DD, deceased donor; LD, living donor; M, male; F, female; N/A, not available.



Concerning the type of liver graft, 14 patients received transplant from deceased donors whereas the remaining 5 patients underwent living donor LT. No intraoperative or postoperative 90-day mortality following LT was documented. All abnormal blood parameters returned to normal within few days postoperatively in all patients. Complications were encountered in 5 patients of the involved patients. Of these, 2 patients required re-operation; abdominal bleeding was detected in one patient and successfully controlled; and the other patient required re-transplantation due to acute liver rejection. All patients were alive at the time of their documentation.

The most effective approach to addressing giant HH remains a subject of ongoing investigation. In instances where giant HHs remain asymptomatic, a conservative management approach may be considered, with vigilant monitoring until symptoms or complications manifest [21]. Surgical intervention becomes necessary when symptoms or complications develop, and the two primary surgical techniques employed include resection or enucleation of the lesion. Alternatively, less invasive methods such as transarterial embolization of the feeding artery and radiofrequency ablation have been proposed as potential strategies to induce a reduction in the size of giant LHs [22].

LT for giant HHs is a rare and extreme measure and is usually not recommended as the first-line treatment. The decision to perform LT for giant HH is usually made on a case-by-case basis after thorough evaluation by multidisciplinary team of medical experts. The primary indications for LT are generally related to life-threatening complications or symptoms that cannot be managed by other means. When a giant HH causes life-threatening symptoms such as severe abdominal pain, hemorrhage, or rupture, and these symptoms cannot be controlled or managed through other means, LT may be considered. If giant HHs lead to complications like Kasabach-Merritt syndrome, uncontrolled bleeding, or compression of adjacent organs to the extent that it compromises their function, LT may be indicated. In instances where a giant HH exhibits rapid growth despite attempts at conservative management or other interventions, LT might be considered as a last resort to control the tumor’s progression. In addition, severe, unmanageable pain related to the giant HH that significantly impairs the patient’s quality of life and cannot be relieved by other therapeutic modalities may be an indication for LT [4-7]. In the present study, the main indications for LT included symptoms related to size of HH (respiratory distress, abdominal discomfort, and pain), risk of rupture, coagulopathy (Kasabach-Merritt syndrome, disseminated intravascular coagulation), and the failure of previous interventions (embolization, resection) to control the disease.

Kasabach-Merritt syndrome is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate [4,23]. Nine out of 19 patients in the present study had suffered from Kasabach-Merritt syndrome, and all of them survived after LT.

Considering that LT for giant HH is a rare and controversial procedure, it is difficult to clearly describe the post-transplant survival because it can vary widely depending on the individual patient’s condition, the extent of the tumor, the presence of complications, and the overall health of the patient as well as the skill and experience of the surgical and medical teams involved. LT is a procedure can be life-saving, and patients may experience long-term survival.

The majority (14 of 19) of patients undergoing LT for giant HH have received deceased donor LT in the present study. There are only five cases of living donor LT for giant HH worldwide [6,9,13,15,20]. Patients with giant HH maintain relatively stable liver function profiles, often resulting in low Model for End-stage Liver Disease scores. Given the exceptionally limited availability of deceased donor LT in the current setting of Korea, it becomes imperative to grant priority to patients with giant HH for living donor LT [24]. To the best of our knowledge, a few cases of living donor LT have been performed in patients with giant HH, but only one case was reported in literature to date [6].

In conclusion, LT is a safe and effective treatment option in the management of symptomatic or complicated giant HH in selected patients.

Conceptualization: CSP. Data curation: CSP. Formal analysis: All. Investigation: All. Methodology: CSP, YKC. Supervision: CSP. Validation: YKC. Visualization: YKC. Writing – original draft: All. Writing – review & editing: All.

  1. Prodromidou A, Machairas N, Garoufalia Z, Kostakis ID, Tsaparas P, Paspala A, et al. Liver transplantation for giant hepatic hemangioma: a systematic review. Transplant Proc 2019;51:440-442.
    Pubmed CrossRef
  2. Bajenaru N, Balaban V, Săvulescu F, Campeanu I, Patrascu T. Hepatic hemangioma -review-. J Med Life 2015;8(Spec Issue):4-11.
  3. Zhao Y, Li XP, Hu YY, Jiang JC, Zhao LJ. Liver transplantation for giant hemangioma of the liver: a case report and review of the literature. Front Med (Lausanne) 2022;9:985181.
    Pubmed KoreaMed CrossRef
  4. Zhao Y, Legan CE. Liver transplantation for giant hemangioma complicated by Kasabach-Merritt syndrome: a case report and literature review. Am J Case Rep 2022;23:e936042.
    CrossRef
  5. Eghlimi H, Arasteh P, Azade N. Orthotopic liver transplantation for Management of a Giant Liver Hemangioma: a case report and review of literature. BMC Surg 2020;20:142.
    Pubmed KoreaMed CrossRef
  6. Lee JH, Yoon CJ, Kim YH, Han HS, Cho JY, Kim H, et al. Living-donor liver transplantation for giant hepatic hemangioma with diffuse hemangiomatosis in an adult: a case report. Clin Mol Hepatol 2018;24:163-168.
    Pubmed KoreaMed CrossRef
  7. Lange UG, Bucher JN, Schoenberg MB, Benzing C, Schmelzle M, Gradistanac T, et al. Orthotopic liver transplantation for giant liver haemangioma: a case report. World J Transplant 2015;5:354-359.
    Pubmed KoreaMed CrossRef
  8. Yildiz S, Kantarci M, Kizrak Y. Cadaveric liver transplantation for a giant mass. Gastroenterology 2014;146:e10-e11.
    Pubmed CrossRef
  9. Zhong L, Men TY, Yang GD, Gu Y, Chen G, Xing TH, et al. Case report: living donor liver transplantation for giant hepatic hemangioma using a right lobe graft without the middle hepatic vein. World J Surg Oncol 2014;12:83.
    Pubmed KoreaMed CrossRef
  10. Unal E, Francis F, Aquino A, Xu R, Morgan G, Teperman L. Liver transplant for mixed capillary-cavernous hemangioma masquerading as hepatocellular carcinoma in a patient with hepatocellular carcinoma. Exp Clin Transplant 2011;9:344-348.
  11. Vagefi PA, Klein I, Gelb B, Hameed B, Moff SL, Simko JP, et al. Emergent orthotopic liver transplantation for hemorrhage from a giant cavernous hepatic hemangioma: case report and review. J Gastrointest Surg 2011;15:209-214.
    Pubmed KoreaMed CrossRef
  12. Aseni P, Lauterio A, Slim AO, Giacomoni A, Lamperti L, De Carlis L. Life-saving super-urgent liver transplantation with replacement of retrohepatic vena cava by Dacron graft. HPB Surg 2010;2010:828326.
    Pubmed KoreaMed CrossRef
  13. Meguro M, Soejima Y, Taketomi A, Ikegami T, Yamashita Y, Harada N, et al. Living donor liver transplantation in a patient with giant hepatic hemangioma complicated by Kasabach-Merritt syndrome: report of a case. Surg Today 2008;38:463-468.
    Pubmed CrossRef
  14. Ferraz AA, Sette MJ, Maia M, Lopes EP, Godoy MM, Petribú AT, et al. Liver transplant for the treatment of giant hepatic hemangioma. Liver Transpl 2004;10:1436-1437.
    Pubmed CrossRef
  15. Kumashiro Y, Kasahara M, Nomoto K, Kawai M, Sasaki K, Kiuchi T, et al. Living donor liver transplantation for giant hepatic hemangioma with Kasabach-Merritt syndrome with a posterior segment graft. Liver Transpl 2002;8:721-724.
    Pubmed CrossRef
  16. Keegan MT, Kamath GS, Vasdev GM, Findlay JY, Gores GJ, Steers JL, et al. Liver transplantation for massive hepatic haemangiomatosis causing restrictive lung disease. Br J Anaesth 2001;86:431-434.
    Pubmed CrossRef
  17. Longeville JH, de la Hall P, Dolan P, Holt AW, Lillie PE, Williams JA, et al. Treatment of a giant haemangioma of the liver with Kasabach-Merritt syndrome by orthotopic liver transplant a case report. HPB Surg 1997;10:159-162.
    Pubmed KoreaMed CrossRef
  18. Russo MW, Johnson MW, Fair JH, Brown RS Jr. Orthotopic liver transplantation for giant hepatic hemangioma. Am J Gastroenterol 1997;92:1940-1941.
  19. Chui AK, Vass J, McCaughan GW, Sheil AG. Giant cavernous haemangioma: a rare indication for liver transplantation. Aust N Z J Surg 1996;66:122-124.
    Pubmed CrossRef
  20. Klompmaker IJ, Sloof MJ, van der Meer J, de Jong GM, de Bruijn KM, Bams JL. Orthotopic liver transplantation in a patient with a giant cavernous hemangioma of the liver and Kasabach-Merritt syndrome. Transplantation 1989;48:149-151.
    Pubmed CrossRef
  21. Liu X, Yang Z, Tan H, Xu L, Liu L, Huang J, et al. Patient age affects the growth of liver haemangioma. HPB (Oxford) 2018;20:64-68.
    Pubmed CrossRef
  22. Moctezuma-Velázquez C, López-Arce G, Martínez-Rodríguez LA, Escalona-Huerta C, Chapa-Ibargüengoitia M, Torre A. Giant hepatic hemangioma versus conventional hepatic hemangioma: clinical findings, risk factors, and management. Rev Gastroenterol Mex 2014;79:229-237.
    Pubmed CrossRef
  23. Aziz H, Brown ZJ, Baghdadi A, Kamel IR, Pawlik TM. A comprehensive review of hepatic hemangioma management. J Gastrointest Surg 2022;26:1998-2007.
    Pubmed CrossRef
  24. Lee JM. Changes in the deceased-donor trend in Korea: establishment of regional trauma centers and KODA. J Clin Med 2022;11:1239.
    Pubmed KoreaMed CrossRef

Article

Review Article

Ann Liver Transplant 2023; 3(2): 69-72

Published online November 30, 2023 https://doi.org/10.52604/alt.23.0017

Copyright © The Korean Liver Transplantation Society.

Liver transplantation for giant hepatic hemangioma: A collective review

Cheon-Soo Park1 , Yong-Kyu Chung2

1Department of Surgery, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
2Department of Surgery, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea

Correspondence to:Cheon-Soo Park
Department of Surgery, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 1021 Tongil-ro, Eunpyeong-gu, Seoul 03312, Korea
E-mail: pskys74@hanmail.net
https://orcid.org/0000-0002-6150-702X

Received: October 9, 2023; Revised: November 5, 2023; Accepted: November 9, 2023

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

Giant hepatic hemangiomas (HHs) have been often considered benign, but their huge size and potential complications can make them far from benign in their impact on the lives. Amid the myriad of treatment options available, liver transplantation (LT) has been performed for giant HHs that have serious clinical manifestations. This study reviewed the role of LT as an indication for giant HH. The literature was searched using search terms as follows in all possible combinations: “liver hemangioma”, “giant hemangioma”, “liver transplantation”, and “Kasabach-Merritt syndrome”. A total of 18 studies were finally included in the present analysis, which recruited 19 patients. The mean age of patients was 39.9±8.6 years. The most common reported symptoms were abdominal distention, respiratory distress, upper abdominal pain, excessive bleeding, and coagulopathy. Preoperative laboratory tests showed abnormal liver function in all patients. Nine patients were diagnosed with Kasabach-Merritt syndrome. Fourteen patients received deceased donor LT whereas the remaining 5 patients underwent living donor LT. No intraoperative or postoperative 90-day mortality following LT was documented. All abnormal blood parameters returned to normal within few days postoperatively in all patients. All patients were alive at the time of their documentation. In conclusion, LT is a safe and effective treatment option in the management of symptomatic or complicated giant HH in selected patients.

Keywords: Hemangioma, Liver transplantation, Abdominal distension, Kasabach-Merritt syndrome, Coagulopathy

INTRODUCTION

In the intricate landscape of liver diseases, one condition that stands as an enigma is the giant hepatic hemangioma (HH). These massive vascular tumors, characterized by their formidable size, pose unique challenges to patients and healthcare providers alike [1]. Giant HHs are often considered benign, but their huger size and potential complications can make them far from benign in their impact on the lives of those affected [2]. Amid the myriad of treatment options available, liver transplantation (LT) has been performed for HHs that have serious clinical manifestations.

This study reviewed the role of LT as an indication for giant HH. Through an exploration of indications, diagnostic criteria, and the clinical rationale behind this decision, we aim to provide a comprehensive understanding of why LT stands as a pivotal strategy in the management of giant HH.

MATERIALS AND METHODS

A meticulous search of the literature was performed for studies published up to September 2023 for cases of patients with giant HH who underwent LT. The literature was searched using search terms as follows in all possible combinations: “liver hemangioma,” “giant hemangioma,” “liver transplantation,” and “Kasabach-Merritt syndrome.” Literature in English was selected. All articles with statement of demographics (age, sex), symptoms, diagnostic evaluation, tumor characteristics (location, histology, size, weight), and post-transplantation outcomes (mortality, length of stay, complications) of patients with giant HH who undergo LT were evaluated.

RESULTS

A total of 18 studies were finally included in the present analysis, which recruited 19 patients, as shown in Table 1 [3-20]. The mean age of patients was 39.9±8.6 years. The most common reported symptoms were abdominal distention, respiratory distress, upper abdominal pain, excessive bleeding, and coagulopathy. Preoperative laboratory tests showed abnormal liver function in all patients (high aspartate transaminase and alanine transaminase levels, low platelet count, prolongation of prothrombin time, and of activated partial thromboplastin time, low fibrinogen, elevated D-dimers). Nine patients were diagnosed with Kasabach-Merritt syndrome.

Table 1 . Collective review of literature on liver transplantation (LT) for giant hepatic hemangioma.

Author (year)Age (yr)/sexPretransplant clinical symptomsKMSHemangioma size (cm)LT typeShort-term outcomeRe-LT
Zhao et al. (2022) [3]36/MAbdominal distentionNoNADDAliveNo
Zhao and Legan (2022) [4]39/FIntra-abdominal hemorrhagesYes14.3×14.6×14.9DDAliveNo
Eghlimi et al. (2020) [5]38/MAbdominal distention, leg edemaNo32.4×26×3.1DDAliveNo
Lee et al. (2018) [6]50/FUpper abdominal pain, abdominal distentionNo16LDAliveNo
Lange et al. (2015) [7]46/FAbdominal distentionNo21.7×23.7×25.5DDAliveNo
Yildiz et al. (2014) [8]44/FAbdominal distention, shortness of breathYes22×18×23DDAliveNo
Zhong et al. (2014) [9]27/FUpper abdominal pain, abdominal distentionNo50×40×25LDAliveNo
Unal et al. (2011) [10]56/FUpper abdominal painYesN/ADDAliveNo
Vagefi et al. (2011) [11]32/FAbdominal distentionYes18×23DDAliveNo
Aseni et al. (2010) [12]46/MPulmonary thromboembolismNoN/ADDAliveNo
Meguro et al. (2008) [13]45/FAbdominal distentionYes15LDAliveNo
Ferraz et al. (2004) [14]25/FProgressive abdominal distentionYes46×40×15DDAliveNo
Kumashiro et al. (2002) [15]48/FAbdominal distensionYesN/ALDAliveNo
Keegan et al. (2001) [16]34/MRespiratory distress, persistent abdominal distentionNo63×45×51DDAliveNo
Longeville et al. (1997) [17]47/MExcessive bleeding after teeth extractionYes25DDAliveNo
Russo et al. (1997) [18]43/FAbdominal distensionNo21DDAliveNo
Chui et al. (1996) [19]33/FUpper abdominal pain, exertional dyspneaNoN/ADDAliveYes
43/FGradually enlarged liver during second pregnancyNo40×35×15DDAliveNo
Klompmaker et al. (1989) [20]27/MHepatomegalyYesN/ALDAliveNo

KMS, Kasabach-Merritt syndrome; DD, deceased donor; LD, living donor; M, male; F, female; N/A, not available..



Concerning the type of liver graft, 14 patients received transplant from deceased donors whereas the remaining 5 patients underwent living donor LT. No intraoperative or postoperative 90-day mortality following LT was documented. All abnormal blood parameters returned to normal within few days postoperatively in all patients. Complications were encountered in 5 patients of the involved patients. Of these, 2 patients required re-operation; abdominal bleeding was detected in one patient and successfully controlled; and the other patient required re-transplantation due to acute liver rejection. All patients were alive at the time of their documentation.

DISCUSSION

The most effective approach to addressing giant HH remains a subject of ongoing investigation. In instances where giant HHs remain asymptomatic, a conservative management approach may be considered, with vigilant monitoring until symptoms or complications manifest [21]. Surgical intervention becomes necessary when symptoms or complications develop, and the two primary surgical techniques employed include resection or enucleation of the lesion. Alternatively, less invasive methods such as transarterial embolization of the feeding artery and radiofrequency ablation have been proposed as potential strategies to induce a reduction in the size of giant LHs [22].

LT for giant HHs is a rare and extreme measure and is usually not recommended as the first-line treatment. The decision to perform LT for giant HH is usually made on a case-by-case basis after thorough evaluation by multidisciplinary team of medical experts. The primary indications for LT are generally related to life-threatening complications or symptoms that cannot be managed by other means. When a giant HH causes life-threatening symptoms such as severe abdominal pain, hemorrhage, or rupture, and these symptoms cannot be controlled or managed through other means, LT may be considered. If giant HHs lead to complications like Kasabach-Merritt syndrome, uncontrolled bleeding, or compression of adjacent organs to the extent that it compromises their function, LT may be indicated. In instances where a giant HH exhibits rapid growth despite attempts at conservative management or other interventions, LT might be considered as a last resort to control the tumor’s progression. In addition, severe, unmanageable pain related to the giant HH that significantly impairs the patient’s quality of life and cannot be relieved by other therapeutic modalities may be an indication for LT [4-7]. In the present study, the main indications for LT included symptoms related to size of HH (respiratory distress, abdominal discomfort, and pain), risk of rupture, coagulopathy (Kasabach-Merritt syndrome, disseminated intravascular coagulation), and the failure of previous interventions (embolization, resection) to control the disease.

Kasabach-Merritt syndrome is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate [4,23]. Nine out of 19 patients in the present study had suffered from Kasabach-Merritt syndrome, and all of them survived after LT.

Considering that LT for giant HH is a rare and controversial procedure, it is difficult to clearly describe the post-transplant survival because it can vary widely depending on the individual patient’s condition, the extent of the tumor, the presence of complications, and the overall health of the patient as well as the skill and experience of the surgical and medical teams involved. LT is a procedure can be life-saving, and patients may experience long-term survival.

The majority (14 of 19) of patients undergoing LT for giant HH have received deceased donor LT in the present study. There are only five cases of living donor LT for giant HH worldwide [6,9,13,15,20]. Patients with giant HH maintain relatively stable liver function profiles, often resulting in low Model for End-stage Liver Disease scores. Given the exceptionally limited availability of deceased donor LT in the current setting of Korea, it becomes imperative to grant priority to patients with giant HH for living donor LT [24]. To the best of our knowledge, a few cases of living donor LT have been performed in patients with giant HH, but only one case was reported in literature to date [6].

In conclusion, LT is a safe and effective treatment option in the management of symptomatic or complicated giant HH in selected patients.

FUNDING

There was no funding related to this study.

CONFLICT OF INTEREST

All authors have no conflicts of interest to declare.

AUTHORS’ CONTRIBUTIONS

Conceptualization: CSP. Data curation: CSP. Formal analysis: All. Investigation: All. Methodology: CSP, YKC. Supervision: CSP. Validation: YKC. Visualization: YKC. Writing – original draft: All. Writing – review & editing: All.

Table 1 Collective review of literature on liver transplantation (LT) for giant hepatic hemangioma

Author (year)Age (yr)/sexPretransplant clinical symptomsKMSHemangioma size (cm)LT typeShort-term outcomeRe-LT
Zhao et al. (2022) [3]36/MAbdominal distentionNoNADDAliveNo
Zhao and Legan (2022) [4]39/FIntra-abdominal hemorrhagesYes14.3×14.6×14.9DDAliveNo
Eghlimi et al. (2020) [5]38/MAbdominal distention, leg edemaNo32.4×26×3.1DDAliveNo
Lee et al. (2018) [6]50/FUpper abdominal pain, abdominal distentionNo16LDAliveNo
Lange et al. (2015) [7]46/FAbdominal distentionNo21.7×23.7×25.5DDAliveNo
Yildiz et al. (2014) [8]44/FAbdominal distention, shortness of breathYes22×18×23DDAliveNo
Zhong et al. (2014) [9]27/FUpper abdominal pain, abdominal distentionNo50×40×25LDAliveNo
Unal et al. (2011) [10]56/FUpper abdominal painYesN/ADDAliveNo
Vagefi et al. (2011) [11]32/FAbdominal distentionYes18×23DDAliveNo
Aseni et al. (2010) [12]46/MPulmonary thromboembolismNoN/ADDAliveNo
Meguro et al. (2008) [13]45/FAbdominal distentionYes15LDAliveNo
Ferraz et al. (2004) [14]25/FProgressive abdominal distentionYes46×40×15DDAliveNo
Kumashiro et al. (2002) [15]48/FAbdominal distensionYesN/ALDAliveNo
Keegan et al. (2001) [16]34/MRespiratory distress, persistent abdominal distentionNo63×45×51DDAliveNo
Longeville et al. (1997) [17]47/MExcessive bleeding after teeth extractionYes25DDAliveNo
Russo et al. (1997) [18]43/FAbdominal distensionNo21DDAliveNo
Chui et al. (1996) [19]33/FUpper abdominal pain, exertional dyspneaNoN/ADDAliveYes
43/FGradually enlarged liver during second pregnancyNo40×35×15DDAliveNo
Klompmaker et al. (1989) [20]27/MHepatomegalyYesN/ALDAliveNo

KMS, Kasabach-Merritt syndrome; DD, deceased donor; LD, living donor; M, male; F, female; N/A, not available.


References

  1. Prodromidou A, Machairas N, Garoufalia Z, Kostakis ID, Tsaparas P, Paspala A, et al. Liver transplantation for giant hepatic hemangioma: a systematic review. Transplant Proc 2019;51:440-442.
    Pubmed CrossRef
  2. Bajenaru N, Balaban V, Săvulescu F, Campeanu I, Patrascu T. Hepatic hemangioma -review-. J Med Life 2015;8(Spec Issue):4-11.
  3. Zhao Y, Li XP, Hu YY, Jiang JC, Zhao LJ. Liver transplantation for giant hemangioma of the liver: a case report and review of the literature. Front Med (Lausanne) 2022;9:985181.
    Pubmed KoreaMed CrossRef
  4. Zhao Y, Legan CE. Liver transplantation for giant hemangioma complicated by Kasabach-Merritt syndrome: a case report and literature review. Am J Case Rep 2022;23:e936042.
    CrossRef
  5. Eghlimi H, Arasteh P, Azade N. Orthotopic liver transplantation for Management of a Giant Liver Hemangioma: a case report and review of literature. BMC Surg 2020;20:142.
    Pubmed KoreaMed CrossRef
  6. Lee JH, Yoon CJ, Kim YH, Han HS, Cho JY, Kim H, et al. Living-donor liver transplantation for giant hepatic hemangioma with diffuse hemangiomatosis in an adult: a case report. Clin Mol Hepatol 2018;24:163-168.
    Pubmed KoreaMed CrossRef
  7. Lange UG, Bucher JN, Schoenberg MB, Benzing C, Schmelzle M, Gradistanac T, et al. Orthotopic liver transplantation for giant liver haemangioma: a case report. World J Transplant 2015;5:354-359.
    Pubmed KoreaMed CrossRef
  8. Yildiz S, Kantarci M, Kizrak Y. Cadaveric liver transplantation for a giant mass. Gastroenterology 2014;146:e10-e11.
    Pubmed CrossRef
  9. Zhong L, Men TY, Yang GD, Gu Y, Chen G, Xing TH, et al. Case report: living donor liver transplantation for giant hepatic hemangioma using a right lobe graft without the middle hepatic vein. World J Surg Oncol 2014;12:83.
    Pubmed KoreaMed CrossRef
  10. Unal E, Francis F, Aquino A, Xu R, Morgan G, Teperman L. Liver transplant for mixed capillary-cavernous hemangioma masquerading as hepatocellular carcinoma in a patient with hepatocellular carcinoma. Exp Clin Transplant 2011;9:344-348.
  11. Vagefi PA, Klein I, Gelb B, Hameed B, Moff SL, Simko JP, et al. Emergent orthotopic liver transplantation for hemorrhage from a giant cavernous hepatic hemangioma: case report and review. J Gastrointest Surg 2011;15:209-214.
    Pubmed KoreaMed CrossRef
  12. Aseni P, Lauterio A, Slim AO, Giacomoni A, Lamperti L, De Carlis L. Life-saving super-urgent liver transplantation with replacement of retrohepatic vena cava by Dacron graft. HPB Surg 2010;2010:828326.
    Pubmed KoreaMed CrossRef
  13. Meguro M, Soejima Y, Taketomi A, Ikegami T, Yamashita Y, Harada N, et al. Living donor liver transplantation in a patient with giant hepatic hemangioma complicated by Kasabach-Merritt syndrome: report of a case. Surg Today 2008;38:463-468.
    Pubmed CrossRef
  14. Ferraz AA, Sette MJ, Maia M, Lopes EP, Godoy MM, Petribú AT, et al. Liver transplant for the treatment of giant hepatic hemangioma. Liver Transpl 2004;10:1436-1437.
    Pubmed CrossRef
  15. Kumashiro Y, Kasahara M, Nomoto K, Kawai M, Sasaki K, Kiuchi T, et al. Living donor liver transplantation for giant hepatic hemangioma with Kasabach-Merritt syndrome with a posterior segment graft. Liver Transpl 2002;8:721-724.
    Pubmed CrossRef
  16. Keegan MT, Kamath GS, Vasdev GM, Findlay JY, Gores GJ, Steers JL, et al. Liver transplantation for massive hepatic haemangiomatosis causing restrictive lung disease. Br J Anaesth 2001;86:431-434.
    Pubmed CrossRef
  17. Longeville JH, de la Hall P, Dolan P, Holt AW, Lillie PE, Williams JA, et al. Treatment of a giant haemangioma of the liver with Kasabach-Merritt syndrome by orthotopic liver transplant a case report. HPB Surg 1997;10:159-162.
    Pubmed KoreaMed CrossRef
  18. Russo MW, Johnson MW, Fair JH, Brown RS Jr. Orthotopic liver transplantation for giant hepatic hemangioma. Am J Gastroenterol 1997;92:1940-1941.
  19. Chui AK, Vass J, McCaughan GW, Sheil AG. Giant cavernous haemangioma: a rare indication for liver transplantation. Aust N Z J Surg 1996;66:122-124.
    Pubmed CrossRef
  20. Klompmaker IJ, Sloof MJ, van der Meer J, de Jong GM, de Bruijn KM, Bams JL. Orthotopic liver transplantation in a patient with a giant cavernous hemangioma of the liver and Kasabach-Merritt syndrome. Transplantation 1989;48:149-151.
    Pubmed CrossRef
  21. Liu X, Yang Z, Tan H, Xu L, Liu L, Huang J, et al. Patient age affects the growth of liver haemangioma. HPB (Oxford) 2018;20:64-68.
    Pubmed CrossRef
  22. Moctezuma-Velázquez C, López-Arce G, Martínez-Rodríguez LA, Escalona-Huerta C, Chapa-Ibargüengoitia M, Torre A. Giant hepatic hemangioma versus conventional hepatic hemangioma: clinical findings, risk factors, and management. Rev Gastroenterol Mex 2014;79:229-237.
    Pubmed CrossRef
  23. Aziz H, Brown ZJ, Baghdadi A, Kamel IR, Pawlik TM. A comprehensive review of hepatic hemangioma management. J Gastrointest Surg 2022;26:1998-2007.
    Pubmed CrossRef
  24. Lee JM. Changes in the deceased-donor trend in Korea: establishment of regional trauma centers and KODA. J Clin Med 2022;11:1239.
    Pubmed KoreaMed CrossRef
The Korean Liver Transplantation Society

Vol.3 No.2
November 2023

pISSN 2765-5121
eISSN 2765-6098

Stats or Metrics

Share this article on :

  • line