Living‐donor liver transplantation for methylmalonic acidemia patient with hepatocellular carcinoma: A case report and literature review

Author:

Deguchi Harunori1ORCID,Sakamoto Seisuke1ORCID,Shimizu Seiichi1,Fukuda Akinari1ORCID,Uchida Hajime1ORCID,Yanagi Yusuke1,Nakao Toshimasa1ORCID,Kodama Tasuku1,Komine Ryuji1ORCID,Nishi Kentaro2,Kamei Koichi2,Haga Chizuko3,Yoshioka Takako3,Matsumoto Kimikazu4,Horikawa Reiko5,Kasahara Mureo1ORCID

Affiliation:

1. Organ Transplantation Center National Center for Child Health and Development Tokyo Japan

2. Division of Nephrology and Rheumatology National Center for Child Health and Development Tokyo Japan

3. Department of Pathology National Center for Child Health and Development Tokyo Japan

4. Children Cancer Center National Center for Child Health and Development Tokyo Japan

5. Department of Endocrinology and Metabolism National Center for Child Health and Development Tokyo Japan

Abstract

AbstractBackgroundMethylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl‐CoA (P‐CoA) catabolism; of note, liver neoplasms rarely occur as a long‐term complication of the disorder. Herein, we report the case of a patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated with a living‐donor liver transplant (LDLT) following prior kidney transplantation.Case ReportA 25‐year‐old male patient with MMA underwent LDLT with a left lobe graft because of metabolic instability and liver neoplasms. He had presented with chronic symptoms of MMA, which had been diagnosed by genetic testing. Additionally, he had undergone living‐donor kidney transplantation with his father as the donor due to end‐stage kidney disease 6 years before the LDLT. He had an episode of metabolic decompensation triggered by coronavirus disease in 2019. Imaging studies revealed an intrahepatic neoplasm in the right hepatic lobe. Due to concerns about metabolic decompensation after hepatectomy, LDLT was performed using a left lobe graft obtained from the patient's mother. Pathological findings were consistent with the characteristics of well‐to‐moderately differentiated HCC. The postoperative course was uneventful, and the patient was discharged 48 days after the LDLT without any complications. At the 9‐month follow‐up, the patient's condition was satisfactory, with sufficient liver graft function and without metabolic decompensation.ConclusionThis case indicates that although HCC is a rare complication in patients with MMA, clinicians should be aware of hepatic malignancies during long‐term follow‐up.

Publisher

Wiley

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