Our patient is a 59-year-old woman with a history of hypertension and hyperglycemia for more than 15 years without previous history of alcohol consumption or smoking nor history of liver disease. She presented with intermittent fever without obvious inducement for 3 weeks (specific time points in Figure 1) with the highest temperature of 39 , accompanied by jaundice, occasional nausea, vomiting, and slight discomfort in the right upper abdomen. Biochemical examination revealed glutamic-pyruvate transaminase (ALT) levels of 198 U/L, glutamic-oxaloacetic transaminase (AST) 131 U/L, total bilirubin (TBil) 68 mumol/L, and direct bilirubin (DBil) 35.1 mumol/L. Blood routine examination revealed no obvious abnormalities. CT of the chest and abdomen showed no obvious active lesions in both lungs but gallbladder enlargement and dilatation of intrahepatic and extrahepatic bile ducts, common bile duct, and main pancreatic duct. MRI of the abdomen showed low biliary obstruction, and the obstruction level was located on the ampulla. Considering the possibility of inflammatory lesions, local hospitals gave anti-infection, fluid rehydration, liver protection, and other symptomatic treatment, but no obvious relief of symptoms.
Abdominal color ultrasound was performed at the general surgery department of our hospital on 19 October 2020. It showed that there is a mass in the ampullary region with dilation of intrahepatic and extrahepatic bile ducts and main pancreatic duct. Moreover, enlargement of the gallbladder was also shown. No obvious abnormality was observed in the liver. CT of pancreatic vascular reconstruction showed a mass in the ampulla, surrounded by enlarged lymph nodes, and was considered malignant. The intrahepatic and extrahepatic bile ducts and pancreatic ducts were dilated, the gallbladder was enlarged, and no obvious abnormalities of the liver and retroperitoneal lymph nodes were observed (Figures 2A, D). Tumor marker AFP was 5.00 ng/ml (normal range 0-10 ng/ml) (Table 1). The pathological result of EUS-FNA was moderately to poorly differentiated ampullary adenocarcinoma (Figure 3A); the immunohistochemistry (IHC) of EUS-FNA was AFP (+) (Figure 3B).
After the patient was admitted to the hospital, the patient was evaluated with an Eastern Cooperative Oncology Group (ECOG) score result of 1. Physical examination revealed yellow skin and yellow sclera. The patient and family had no previous medical history of chronic viral hepatitis, bowel polyps, and inflammatory bowel disease (IBD). The patient underwent pancreaticoduodenectomy on 30 October 2020. Intraoperative exploration showed that there were no free ascites or metastatic nodules in the abdominal cavity, the liver had a normal size with cholestasis-like changes, and the gallbladder was swollen. A mass with a size of about 3 cm * 3 cm could be reached in the ampulla, and the texture was hard. Postoperative pathology (pancreaticoduodenectomy specimen) (Figure 3C) showed that the duodenal papilla had a poorly differentiated adenocarcinoma, the size of the mass was 4 cm * 2.5 cm * 1.2 cm, and the cancer tissue penetrated the duodenal muscle wall and invaded the pancreatic tissue. There is no definite cancer tissue invasion in the common bile duct. Vascular cancer embolus and perineural invasion can be seen. No cancer remains at the gastric margin, small intestinal margin, common bile duct margin, broken end margin of the pancreas, peripheral margin of the pancreas, and hook process margin of the pancreas. Cancer metastasis was found in 1/6 lymph nodes around the pancreas, and no cancer metastasis was found in 3 lymph nodes around the stomach, 1 lymph node around the intestine, and 2 lymph nodes around the common bile duct. Another 3 lymph nodes were sent, and no cancer metastasis was found. Pathological stage was IIIA (T4, N1, cM0); IHC: cancer cell expression CK(+), S100(-), Her2(Sto)(++), COX2(+), VEGFR2(-), EGFR(++), Ki67(about 60%+), P16(focal +), CD56(-), PD-1(-), PD-L1(SP142)(the tumor cells -, interstitial immune cells about 5%+), MLH1(+), MSH2(+), MSH6(+), PMS2(+), c-Met(+), CgA(-); AFP (+) (Figure 3D).
One month after surgery, CT and MRI scan showed multiple hepatic masses (Figures 2B, C) and retroperitoneal enlarged lymph nodes (Figures 2E, F), which were considered metastases. The patient refused to accept gene testing and targeted therapy, so one cycle of XELOX 3-week regimen chemotherapy was performed according to National Comprehensive Cancer Network (NCCN) guidelines for CRC.
After one cycle of chemotherapy, tumor marker AFP rose from 300 to 1,931.90 ng/ml (Table 1). In order to clarify the nature of the liver lesions, liver biopsy was performed. Microscopic observation showed that the tumor cells were distributed in plate shape, and abundant blood sinus structures were seen, adenocarcinoma area and hepatoid differentiation area coexist, polygonal tumor cells with larger volume and eosinophilic cytoplasm can be seen in the hepatoid differentiation area (Figure 4A). Liver biopsy pathology showed adenocarcinoma, poorly differentiated; IHC showed cancer cell expression Hept1 (-), CK19 (+++), CK7 (-), CDX-2 (focal +), Ki67 (70%+), CK20 (-), Villin (+++), Syn (focal +), CgA (focal +), CD56 (focal +); AFP (+), GPC-3 (+) (Figures 4B, C).
Combining the abnormal increase of serum AFP, microscopic pathological morphology and IHC features of tissue samples from presurgical EUS-FNS, surgery, and liver biopsy, this is a rare case of HAC of the duodenum with liver metastasis.
However, the disease progressed rapidly, and the patient died within 1 month after biopsy. The survival time was only 161 days. Patients and their families hope that the information they provide can help clinicians have a deeper understanding of this kind of diseases, thus helping more patients.