Abstract
The case involved a 71-year-old man who presented with an episodic abnormal behavior at the age of 68 and was diagnosed with adult-onset citrullinemia resulting from close examinations including a gene study. A periodic abdominal ultrasonography while he had been followed in the clinic revealed a solid tumor at the segment 7 of the liver. Contrast-enhanced CT scan and primovist enhanced MRI scan showed early enhancement pattern. The patient was thus referred to our hospital with a suspicion of hepatocellular carcinoma. Partial hepatectomy (S7) was performed and highly to moderately differentiated hepatocellular carcinoma was histopathologically diagnosed. In the sixth month after the operation, recurrence occurred in the vicinity of the resected area, and partial hepatectomy (S7) was performed again. We tried to minimize the resecting area and permitted him to resume oral ingestion of a diet which was made for each patient as well as oral arginine and pyrubic acid preparation in an early time after the second operation. Accordingly he could follow a satisfactory postoperative course.
We sometimes encounter reports on cases of hepatocellular carcinoma associated with adult-onset citrullinemia, however, few reports on such cases performed surgical resection have been seen. As this case is considered to be rare, we present this case, together with a review of the literature.
