A 51-year-old man presented to the emergency department with complaints of an unrelenting cough, nausea, and constant, nonspecific pain in the right-upper quadrant of his abdomen. The patient's cough worsened at night, causing insomnia. The patient also complained of night sweats and a 60-pound weight loss from anorexia in the past year. His abdominal pain and anorexia began at the same time. His past medical history was notable for hypertension, hypercholesterolemia, anemia, renal lithiasis, and impaired coagulation. The patient had no history of alcohol or tobacco abuse and no family history of liver disease. The patient also had no history of skin rashes, hypoglycemia, gastrointestinal bleeding, or vitiligo. While he had no personal history of cancer, his family history was remarkable for his maternal grandmother having cancer of the jaw, ear, and parotid gland. The patient was taking colesevelam hydrochloride for his hyperlipidemia. Despite the use of acid suppression, the patient's pain did not improve. In addition, he could not elicit reflux during positional therapy.
The patient's physical examination revealed an enlarged liver and marked tenderness in the right-upper quadrant and epigastrium regions of his abdomen. The examination did not show evidence of scleral icterus, splenomegaly, ascites, spider angiomata, or palmar erythema. Results of the patient's ear, nose, and throat and lung examinations were normal. Laboratory findings (reference range) were as follows: hemoglobin level, 9.2 g/dL (13.5-16.7 g/dL); blood platelet count, 472 × 10
3/μL (150-400 × 10
3/μL); prothrombin time, 16.4 seconds (11.5-14.3 seconds); albumin, 3 g/dL (3.9-4.8 g/dL); and alkaline phosphatase, 214 U/L (39-117 U/L) with normal transaminase levels. Viral serologies, autoimmune markers, iron levels, and alpha fetoprotein levels were unremarkable. Genetic testing was negative for von Hippel-Lindau disease. A computed tomography (CT) scan of the chest, abdomen, and pelvis was completed with intravenous and oral contrast. The results of the CT scan revealed several small masses in the right lobe of the liver and complete replacement of the left lobe of the liver by a mass that was approximately 16 × 11 × 12 cm
3 (
Figure 1 and
Figure 2). Results of a tagged red blood cell scan confirmed uptake in three homogenous areas of the right lobe of the liver, peripheral uptake in a large region of the left lobe of the liver, and a decreased central uptake consistent with CH. The presence of CH was confirmed with a microscopic evaluation.
After an exploratory laparotomy, the CH in the patient's liver was initially deemed unresectable and the patient was treated with one session of left-sided hepatic artery chemoembolization. While the patient's symptoms initially improved, they gradually returned over the next 6 months. At a follow-up examination 6 months after the hepatic artery embolization, a CT scan revealed no change in the size of the GCH. Results of the follow-up examination also showed new findings including a small cervical CH at the C7 vertebrae, an 8 × 6 × 8 mm3 intracanalicular acoustic neuroma, a bony tumor of the left-sided zygomatic bone, and a 5 × 5 × 9 mm3 prolactinoma.
Nine months after embolization, the patient underwent excision and reconstruction of the vascular lesion on his left-sided zygomatic bone because of progressive pain in the area. Three months after the zygomatic bone resection, serial imaging showed little change in the acoustic neuroma, C7 hemangioma, and prolactinoma. The patient's symptoms of cough with insomnia and abdominal pain continued to persist.
Based on these findings, the liver transplant team reconsidered the patient for surgery. A left-sided hepatic lobectomy and resection of a 20 × 10 × 12 cm3 GCH was performed. The smaller CHs in the remainder of the patient's liver were not resected. The patient's postoperative course was uncomplicated, and he was discharged after a 3-day hospital stay. After the surgery, the patient's cough resolved within 1 week and the rest of his symptoms resolved within 2 weeks. He has remained asymptomatic for more than 1 year.