Liver transplantation for a giant mesenchymal hamartoma of the liver in an adult: Case report and review of the literature

Abstract

Mesenchymal hamartomas of the liver (MHLs) in adults are rare and potentially premalignant lesions, which present as solid/cystic neoplasms. We report a rare case of orthotopic liver transplantation in a patient with a giant MHL. In 2013, a 34-year-old female sought medical advice after a 2-year history of progressive abdominal distention and respiratory distress. Physical examination revealed an extensive mass in the abdomen. Computed tomography (CT) of her abdomen revealed multiple liver cysts, with the diameter of largest cyst being 16 cm × 14 cm. The liver hilar structures were not clearly displayed. The adjacent organs were compressed and displaced. Initial laboratory tests, including biochemical investigations and coagulation profile, were unremarkable. Tumor markers, including levels of AFP, CEA and CA19-9, were within the normal ranges. The patient underwent orthotopic liver transplantation in November 2013, the liver being procured from a 40-year-old man after cardiac death following traumatic brain injury. Warm ischemic time was 7.5 min and cold ischemic time was 3 h. The recipient underwent classical orthotopic liver transplantation. The recipient operative procedure took 8.5 h, the anhepatic phase lasting for 1 h without the use of venovenous bypass. The immunosuppressive regimen included intraoperative induction with basiliximab and high-dose methylprednisolone, and postoperative maintenance with tacrolimus, mycophenolate mofetil, and prednisone. The recipient's diseased liver weighed 21 kg (dry weight) and measured 41 cm × 32 cm × 31 cm. Histopathological examination confirmed the diagnosis of an MHL. The patient did not experience any acute rejection episode or other complication. All the laboratory tests returned to normal within one month after surgery. Three months after transplantation, the immunosuppressive therapy was reduced to tacrolimus monotherapy, and the T-tube was removed after cholangiography showed no abnormalities. Twelve months after transplantation, the patient remains well and is fulfilling all normal activities. Adult giant MHL is extremely rare. Symptoms, physical signs, laboratory results, and radiographic imaging are nonspecific and inconclusive. Surgical excision of the lesion is imperative to make a definite diagnosis and as a cure. Liver transplantation should be considered as an option in the treatment of a non-resectable MHL.

Mesenchymal Hamartoma in Children: A Diagnostic Challenge

“…Advanced imaging is usually helpful in the early diagnosis although nonspecific findings may also be noted. A delay in diagnosis can lead to complications from local compression [10]. Rarely, malignant transformation has also been reported [11].…”

“…The role of liver transplant is not clearly defined in this benign condition. However, if the tumor is unresectable or recurs after partial hepatectomy, liver transplantation remains a viable therapeutic option in both children and adults [9, 10].…”

Liver transplantation as definitive treatment of an unresectable mesenchymal hamartoma in a child with Beckwith–Wiedemann Syndrome

“…Sequential resection can be considered in certain cases of massive tumors [ 10 ], but unresectable tumors may necessitate liver transplantation. Five cases of MLH treated with liver transplantation have been previously reported in the literature—three infants and two adults, with four successful outcomes and one death due to intraoperative hemorrhagic shock [ 3 – 6 ]. To the best of our knowledge, this is the first report in the literature of liver transplantation in a patient with BWS for the treatment of unresectable MLH.…”

“…Despite being classified as a benign tumor, if left untreated, MLH can grow dramatically, causing fatal compression of adjacent structures or destruction of functional hepatic parenchyma. Definitive treatment requires resection, and while transplant has been cited as a treatment option, only a few reported cases have required full organ resection and transplantation [ 3 – 6 ]. We report a rare case of unresectable MLH requiring liver transplantation in a patient with BWS.…”

A 4 and a half years old boy with mesenchymal hamartomas in the left lateral lobe of the liver

“…Only 46 adult cases have been reported up to now. [ 5 ] About 75% of MHL was found in the right lobe of the liver. [ 4 ] The clinical manifestations and radiological examinations of MHL are typically nonspecific.…”