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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
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 of diagnosis, suspecting malignant liver masses such as hepato-
Department diagnosed mesenchymal hamartoma. blastoma, as was the case for this patient [10].
Despite being a benign pathology, due to the condition of the In the past, non-radical resection of the tumor had been pro-
patient and the impossibility of surgical resection, it was decided posed as an acceptable management [11], yet it is now clear that
that a living donor liver transplantation from the patient's father mesenchymal hamartomas share a common genetic nding with
was the best surgical option for the patient. The liver explant undifferentiated embryonal sarcoma of the liver, breakpoint
weighed 2 kg, and the only intraoperative complication was a 19q13.4 [12e14]. More over, recent reports propose, if feasible, a
3000 cc hemoperitoneum. Twenty-four hours after the trans- radical surgical resection of the tumor since recurrence and ma-
plant, the patient was revised by the surgical team and discov- lignant transformation have been documented [4,13,14].
ered active bleeding from the arterial anastomosis. As a result, Currently, the gold standard for the treatment of mesenchymal
the patient received a transfusion of red blood cells and hamartoma is a complete surgical resection of the tumor to avoid
cryoprecipitate. local recurrences and long-term malignant transformation [15].
The pathology report of the liver explant reported the macro- However, cases of complex surgical management have been re-
scopical presence, between segments VIII and IV, of two cystic ported when dealing with multiple and giant tumors. In a series of
cavities of 7 6.8 3 cm and 5 4 2 cm; the remainder of the 17 cases, Karpelowsky et al. mention intraoperative problems
parenchyma presented a mottled hemorrhagic appearance. ranging from one intraoperative death, one bile duct injury and an
Microscopic examination revealed the presence of bro-connective accelerated recurrence after an incomplete tumor resection who
tissue with multiple arterial, venous and lymphatic vessels, nests of also died in surgery [16]. Liver transplantation is controversial in
mature hepatocytes and cystic bro-connective cavities lined with this pathology but in giant unresectable tumors, it is a viable
epithelium (Fig. 2). These observations conrmed the diagnosis of therapeutic option [3,17]. So far, three pediatric liver transplants
mesenchymal hamartoma. have been reported for unresectable giant mesenchymal hamar-
Ten months after the liver transplantation, the patient continues toma following an incomplete resection, and Tepetes et al. reported
with an adequate clinical and laboratory evolution, with an two cases in adult patients, with progressive liver failure and pre-
immunosuppressive management with tacrolimus 3 mg/day, vious partial hepatectomies, who experienced a satisfactory
mycophenolate mofetil 450 mg per day and prednisolone 5 mg per outcome [18,19].
day. 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
3. Discussion post-transplant, the patient has experienced a satisfactory evolu-
tion, with alpha-fetoprotein and liver enzymes levels within
Mesenchymal hamartoma of the liver was described in a normal ranges. This surgical approach was possible due to the
denitive manner in 1956 by Edmonson. This pathology usually characteristics of the tumor, the condition of the patient and the
presents in pediatric patients with a median time of appearance of availability of a living donor.
ten months [7,8]. Histologically, the condition shows a stroma of To our knowledge, this is the rst case of Liver Transplantation
bromyxoid tissue with wavy cells and cystic spaces in the middle as surgical management for a giant mesenchymal hamartoma in
of biliary ducts and normal hepatocytes [9]. Differential diagnosis Latin America. We believe that liver transplantation is a safe sur-
includes other liver masses and infectious cystic lesions. Laboratory gical option for pediatric patients with unresectable giant mesen-
tests are usually normal, yet in some cases elevated alpha- chymal hamartoma, who generally have no other therapeutic
fetoprotein levels are associated with poorly organized hepato- alternative.
cytes in liver biopsies. This can be a source of confusion at the time
A.M. Arrunategui et al. / Journal of Pediatric Surgery Case Reports 21 (2017) 1e3 3
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.