Journal of Pediatric Surgery Case Reports 21 (2017) 1e3

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Journal of Pediatric Surgery Case Reports

journal homepage: www.jpscasereports.com

Giant mesenchymal hamartoma in pediatric patients: A new

indication for liver transplantation
Ana M. Arrunategui, Luis Armando Caicedo, Laura S. Thomas, Vero
nica Botero,
Oscar Garca, Edwin Carrascal, Lorena I. Go
mez, Leidys Gutie

rrez, Alejandro Delgado,
Oscar Serrano, Jorge I. Villegas, Gabriel Jaime Echeverri*

n Valle del Lili, Center for Research in Advanced Surgery and Transplants (CICAT), ICESI University, Cali, Colombia
Clinical Research Center, Fundacio

a r t i c l e i n f o a b s t r a c t

Article history: Mesenchymal hamartoma is the second most frequent benign liver tumor in young children and usually
Received 15 February 2017 occurs in the rst two years of life. Currently, the gold standard for treatment is a complete surgical
Received in revised form resection. We report a 17-month old patient with a giant mesenchymal hamartoma who, due to the
22 March 2017
impossibility of resection and the risk of malignant transformation, received liver transplantation from a
Accepted 26 March 2017
living donor with a satisfactory outcome in the rst year post-transplant. In this report, we show that
Available online 28 March 2017
liver transplantation is a viable therapeutic alternative for this pathology, in patients who have no
possibility of receiving another type of treatment due to their clinical characteristics.
Keywords:
Mesenchymal hamartoma
2017 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
Pediatric license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Living donor liver transplantation

1. Introduction In this report, we present a pediatric patient with a diagnosis of

a unresectable giant mesenchymal hamartoma of the liver who
Mesenchymal hamartoma is the second most frequent benign received a living donor liver transplantation.
liver tumor in young children and constitutes approximately 8% of
all tumors in this population, with 80% of these tumors presenting
2. Presentation of the case
within the rst two years of life [1]. The clinical picture includes
gastrointestinal symptoms, weight loss, and anorexia. The physical
A 17-month old female patient, product of a normal birth, with a
examination of affected individuals includes a palpable homoge-
8-month history of noticeable weight loss, abdominal distention,
neous abdominal mass, malnutrition, and in advanced cases res-
and progressive deterioration was referred to the Fundacio
n Valle
piratory distress [2]. Elevated alpha-fetoprotein levels mandate a
del Lili. Upon physical examination the patient was found to be
differential diagnosis with hepatoblastoma, using radiological and
cachectic with a distended abdomen, collateral circulation and
pathology tests [3].
hepatomegaly reaching the right ank and crossing the middle line.
Some authors consider the mesenchymal hamartoma to be a
The initial blood tests were as follow: total bilirubin 0.32 mg/dL;
pre-malignant tumor, due to the possibility of its recurrence as an
direct bilirubin 0.27 mg/dL; alanine transaminase 24.5 U/L; aspar-
undifferentiated embryonic sarcoma and recommend a radical
tate transaminase 45.5 U/L, alkaline phosphatase 239 U/L; serum
resection of the tumor [4]. Currently, the gold standard for the
alpha-fetoprotein 4376 IU/ml. Initial differential diagnosis of hep-
treatment of this pathology is a complete surgical resection. Liver
atoblastoma and hydatidosis was made with the addition of an
transplantation is controversial for benign tumors in pediatric pa-
abdominal ultrasound and MRI, as shown in Fig. 1, that showed
tients and is not recommended in clinical practice guidelines [5].
hepatomegaly with multiple images of a complex cyst.
Yet in unresectable giant mesenchymal hamartoma, liver trans-
To clarify the diagnosis, a liver biopsy was performed. The
plantation should be considered as a therapeutic alternative [6].
microscopic examination revealed the presence of bro-connective
tissue with arterial vessels, venous capillaries, lymph nodes and the
* Corresponding author. Universidad ICESI, Calle 18 No. 122-135 Pance, Cali,
presence of stratied columnar epithelium arranged in a branched
Colombia. form. Nests of mature hepatocytes were observed and there was no
E-mail address: gjecheverri@icesi.edu.co (G.J. Echeverri). malignancy in the sample examined. Immunohistochemistry was

http://dx.doi.org/10.1016/j.epsc.2017.03.012
2213-5766/ 2017 The Author(s). 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/
).
2 A.M. Arrunategui et al. / Journal of Pediatric Surgery Case Reports 21 (2017) 1e3
Fig. 1. MRI with axial T2-weighted cuts and post-contrast T1, where it can be seen large liver mass, solid 20 cm in length, which occupies most of the right hepatic lobe and extends
into the left hepatic lobe. It has hyperintense on T2 behavior and central areas with cystic artery enhancement and progressive peripheral phase in the later phases.
found to be positive, as expected for a mature tissue. The Pathology
Department diagnosed mesenchymal hamartoma.
Despite being a benign pathology, due to the condition of the
patient and the impossibility of surgical resection, it was decided
that a living donor liver transplantation from the patient's father
was the best surgical option for the patient. The liver explant
weighed 2 kg, and the only intraoperative complication was a
3000 cc hemoperitoneum. Twenty-four hours after the trans-
plant, the patient was revised by the surgical team and discov-
ered active bleeding from the arterial anastomosis. As a result,
the patient received a transfusion of red blood cells and
cryoprecipitate.
The pathology report of the liver explant reported the macro-
scopical presence, between segments VIII and IV, of two cystic
cavities of 7
6.8
3 cm and 5
4
2 cm; the remainder of the
parenchyma presented a mottled hemorrhagic appearance.
Microscopic examination revealed the presence of bro-connective
tissue with multiple arterial, venous and lymphatic vessels, nests of
mature hepatocytes and cystic bro-connective cavities lined with
epithelium (Fig. 2). These observations conrmed the diagnosis of
mesenchymal hamartoma.
Ten months after the liver transplantation, the patient continues
with an adequate clinical and laboratory evolution, with an
immunosuppressive management with tacrolimus 3 mg/day,
mycophenolate mofetil 450 mg per day and prednisolone 5 mg per
day.
3. Discussion
Mesenchymal hamartoma of the liver was described in a
denitive manner in 1956 by Edmonson. This pathology usually
presents in pediatric patients with a median time of appearance of
ten months [7,8]. Histologically, the condition shows a stroma of
bromyxoid tissue with wavy cells and cystic spaces in the middle
of biliary ducts and normal hepatocytes [9]. Differential diagnosis
includes other liver masses and infectious cystic lesions. Laboratory
tests are usually normal, yet in some cases elevated alpha-
fetoprotein levels are associated with poorly organized hepato-
cytes in liver biopsies. This can be a source of confusion at the time
of diagnosis, suspecting malignant liver masses such as hepato-
blastoma, as was the case for this patient [10].
In the past, non-radical resection of the tumor had been pro-
posed as an acceptable management [11], yet it is now clear that
mesenchymal hamartomas share a common genetic nding with
undifferentiated embryonal sarcoma of the liver, breakpoint
19q13.4 [12e14]. More over, recent reports propose, if feasible, a
radical surgical resection of the tumor since recurrence and ma-
lignant transformation have been documented [4,13,14].
Currently, the gold standard for the treatment of mesenchymal
hamartoma is a complete surgical resection of the tumor to avoid
local recurrences and long-term malignant transformation [15].
However, cases of complex surgical management have been re-
ported when dealing with multiple and giant tumors. In a series of
17 cases, Karpelowsky et al. mention intraoperative problems
ranging from one intraoperative death, one bile duct injury and an
accelerated recurrence after an incomplete tumor resection who
also died in surgery [16]. Liver transplantation is controversial in
this pathology but in giant unresectable tumors, it is a viable
therapeutic option [3,17]. So far, three pediatric liver transplants
have been reported for unresectable giant mesenchymal hamar-
toma following an incomplete resection, and Tepetes et al. reported
two cases in adult patients, with progressive liver failure and pre-
vious partial hepatectomies, who experienced a satisfactory
outcome [18,19].
Our article describes the case of a pediatric patient with a giant
unresectable mesenchymal hamartoma who received a living
donor liver transplantation as surgical management. At 10 months
post-transplant, the patient has experienced a satisfactory evolu-
tion, with alpha-fetoprotein and liver enzymes levels within
normal ranges. This surgical approach was possible due to the
characteristics of the tumor, the condition of the patient and the
availability of a living donor.
To our knowledge, this is the rst case of Liver Transplantation
as surgical management for a giant mesenchymal hamartoma in
Latin America. We believe that liver transplantation is a safe sur-
gical option for pediatric patients with unresectable giant mesen-
chymal hamartoma, who generally have no other therapeutic
alternative.
 A.M. Arrunategui et al. / Journal of Pediatric Surgery Case Reports 21 (2017) 1e3
Fig. 2. Is observed broconnective presence of loose tissue and in the middle there are arterial, venous and lymphatic capillary vessels, branch conducts surrounded by columnar
epithelium and cystic cavities. On the periphery of the mass are seen mature hepatocytes. Hematoxylin and eosin staining 200
; 400
.
Authors contributions
Luis Armando Caicedo, Oscar Serrano, Jorge I. Villegas, Gabriel
Jaime Echeverri performed the surgery on the liver donor and
recipient. Veronica Botero pre-operative and post-operative man-
agement, Ana M. Arrunategui, Lorena I. Gomez and Edwin Carrascal
biopsies caption, and images. Oscar Garcia MRI images lecture.
Ana M. Arrunategui, Laura S. Thomas, Luis A. Caicedo concept
and design, Laura S. Thomas, Leidys Gutie
rrez, Alejandro Delgado
drafting of the article. All authors did the critical revision and
approved the submitted nal version of the article.
References
[1] Abdulkader I, Fraga M, Pe
rez-Becerra E, Reyes-Santas RM, Bautista A,
Chaves E, et al. Mesenchymal hamartoma of the liver: clinicopathological,
immunohistochemical and ow cytometric study of two cases. Hepatol Res
2004;28(4):216e9.
[2] Mulrooney DA, Carpenter B, Georgieff M, Angel C, Hunter D, Foker J, et al.
Hepatic mesenchymal hamartoma in a neonate: a case report and review of
the literature. J Pediatr Hematol Oncol 2001;23(5):316e7.
[3] Stringer MD, Alizai NK. Mesenchymal hamartoma of the liver: a systematic
review. J Pediatr Surg 2005;40(11):1681e90.
[4] Wildhaber BE, Montaruli E, Gue
rin F, Branchereau S, Martelli H, Gauthier F.
Mesenchymal hamartoma or embryonal sarcoma of the liver in childhood: a
difcult diagnosis before complete surgical excision. J Pediatr Surg [Internet]
2014;49(9):1372e7. http://dx.doi.org/10.1016/j.jpedsurg.2014.04.005. Elsev-
ier Inc.
[5] Tannuri ACA, Tannuri U, Gibelli NEM, Roma ~o RLP. Surgical treatment of he-
patic tumors in children: lessons learned from liver transplantation. J Pediatr
Surg [Internet] 2009;44(11):2083e7. http://dx.doi.org/10.1016/j.jped-
surg.2009.06.003. Elsevier Inc.
[6] Buist LJ, Kelly DA, Raafat F, Mcmaster P, Mayer AD, Buckels JAC. Unresectable
hepatic tumors in childhood and the role. J Pediatr Surg 1996;31(11):1563e7.
[7] Hernandez JC, Alfonso C, Gonzalez L, Samada M, Ramos L, Cepero-Valdez M,
et al. Solid mesenchymal hamartoma in an adult: a case report. J Clin Pathol
[Internet] 2006;59(5):542e5. Available from: http://www.ncbi.nlm.nih.gov/
3
entrez/query.fcgi?cmdRetrieve&dbPubMed&doptCitation&list_
uids16644886.
[8] Yandza T, Valayer J. Benign tumors of the liver in children: analysis of a series
of 20 cases. J Pediatr Surg 1986;21(5):419e23.
[9] Odze R, Goldblum J. Benign and malignant tumors of the liver. In: Pathology of
the GI tract, liver, biliary tract and pancreas. Third. Elsevier; 2015. p. 1438e43.
[10] Boman F, Bossard C, Fabre M, Diab N, Bonnevalle M, Boccon-Gibod L.
Mesenchymal hamartomas of the liver may Be associated with increased
serum alpha foetoprotein concentrations and mimic hepatoblastomas. Eur J
Pediatr Surg 2004;14(1):63e6.
[11] Stocker J, Ishak K. Mesenchymal hamartoma of the liver: report of 30 cases
and review of the literature. Pediatr Pathol 1983;1(3):245e67.
[12] Sharif K, Ramani P, Lochbhler H, Grundy R, de Ville de Goyet J. Recurrent
mesenchymal hamartoma associated with 19q translocation. A call for more
radical surgical resection. Eur J Pediatr Surg [Internet] 2006;16(1):64e7.
Available from: http://www.thieme-connect.de/DOI/DOI?10.1055/s-2005-
873072.
[13] Begueret H, Trouette H, Vielh P, Laurent C, MacGrogan G, Delsol M, et al.
Hepatic undifferentiated embryonal sarcoma: malignant evolution of
mesenchymal hamartoma? Study of one case with immunohistochemical and
ow cytometric emphasis [4]. J Hepatol 2001;34(1):178e9.
[14] Shehata BM, Gupta NA, Katzenstein HM, Steelman CK, Wulkan ML, Gow KW,
et al. Undifferentiated embryonal sarcoma of the liver is associated with
mesenchymal hamartoma and multiple chromosomal abnormalities: a review
of eleven cases. Pediatr Dev Pathol [Internet] 2011;14(2):111e6. Available
from: http://www.pedpath.org/doi/abs/10.2350/09-07-0681-OA.1.
[15] Fernandez-Pineda I, Cabello-Laureano R. Differential diagnosis and manage-
ment of liver tumors in infants. World J Hepatol 2014;6(7):486e95.
[16] Karpelowsky JS, Pansini A, Lazarus C, Rode H, Millar AJW. Difculties in the
management of mesenchymal hamartomas. Pediatr Surg Int 2008;24(10):
1171e5.
[17] Bejarano PA, Serrano MF, Casillas J, Dehner LP, Kato T, Mittal N, et al. Con-
current infantile hemangioendothelioma and mesenchymal hamartoma in a
developmentally arrested liver of an infant requiring hepatic transplantation.
Pediatr Dev Pathol 2003;6(6):552e7.
[18] Tapetes K, Selby R, Webb M. Orthotopic liver transplantation for benign he-
patic neoplasms. Arch Surg 1995;130(2):153e6.
[19] Li J, Cai J-Z, Guo Q-J, Li J-J, Sun X-Y, Hu Z-D, et al. Liver transplantation for a
giant mesenchymal hamartoma of the liver in an adult: case report and re-
view of the literature. World J Gastroenterol 2015;21(20):6409e16.