• Case report
• Extrahepatic
• Lymph node
• Metastasis
• Portocaval

• adrenal infiltration
• case report
• ectopic hepatocellular carcinoma
• endocrine function
• retroperitoneal tumor

• Ectopic liver
• Gastrointestinal stromal tumor
• Hepatocellular carcinoma
• Subepithelial tumor

Findings of ectopic hepatocellular carcinoma (EHCC) have been rarely documented. Complicated clinical features and unpredictable medical prognosis make diagnosis and treatment difficult.

We reported a 59-year-old male patient who came to the hospital with epigastric discomfort and regurgitation of gastric acid. An enhanced CT scan revealed a 1.8 cm × 1.4 cm mass in the tail of pancreas without any positive finding in the liver. Postoperative MRI scan was performed but did not reveal any evidence of hepatic tumor. The tumor was resected in toto. Meanwhile, a 1 cm × 1 cm mass in the body of the stomach was found that was removed simultaneously. Histopathology showed that the pancreatic tumor was ectopic hepatocellular carcinoma (EHCC), and that the gastric nodule was gastrointestinal stromal tumor (GIST). The patient had an uneventful postoperative recovery. He has been living without recurrence for over 7 years since surgery. Owing to our knowledge, this is the second-longest disease-free survival time for EHCC in the literature.

Here, we present a rare case of EHCC in the pancreas, and review the current literature on EHCC. Operation was an effective treatment for patients with curable EHCC. EHCC with metastasis still needs more practice to improve the poor prognosis.

• hepatocellular carcinoma
• pancreatectomy
• robotic

An ectopic hepatocellular carcinoma (EHCC) arises from the ectopic liver which is defined as a hepatic organ or tissue not connected to surrounding tissues. EHCC is a rare disease and it is difficult to diagnose preoperatively. Furthermore, the clinical features are not fully elucidated.

A retroperitoneal tumor (6 cm) was located at the dorsal side of the pancreas head on abdominal ultrasonography in an 81-year old woman positive for hepatitis C virus antibody. Contrast enhanced-computed tomography and gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging showed viable HCC patterns with early enhancement and delayed washout. The tumor markers — serum alpha-fetoprotein and alpha-fetoprotein-L3% — were increased to 30.1 ng/mL and 83.1%, respectively. Protein induced by vitamin K absence or antagonist-II was within normal levels (17 mAU/mL). Positron emission tomography-computed tomography showed strong accumulation into the tumor (Standardized Uptake Value max: 13.8), and the tumor cytology following endoscopic ultrasound-guided fine needle aspiration showed poorly differentiated carcinoma. Tumor extirpation was performed, and operative findings showed that the retroperitoneal tumor was disconnected from the pancreas and the liver. Swollen lymph nodes near the tumor were histologically normal. On histological examination, the tumor was finally diagnosed as EHCC with Arginase-1 positive expression.

We report our experience of a rare EHCC which was difficult to diagnose, and we present a review of the literature.

• Ectopic hepatocellular carcinoma
• Differential diagnosis
• Retroperitoneal tumor
• Case report

• Aged, 80 and over
• Biomarkers, Tumor/analysis
• Carcinoma, Hepatocellular/diagnosis
• Carcinoma, Hepatocellular/pathology
• Carcinoma, Hepatocellular/surgery
• Choristoma/diagnosis
• Choristoma/pathology
• Choristoma/surgery
• Diagnosis, Differential
• Endoscopic Ultrasound-Guided Fine Needle Aspiration
• Female
• Humans
• Liver
• Positron Emission Tomography Computed Tomography
• Retroperitoneal Neoplasms/diagnosis
• Retroperitoneal Neoplasms/pathology
• Retroperitoneal Neoplasms/surgery

The differential diagnosis of perigastric masses is broad, ranging from benign to malignant entities. Among the benign entities, accessory liver lobes and ectopic liver are unusual and often incidentally discovered. Here, we report a patient with malignant melanoma who was clinically suspected to have a perigastric metastasis or a gastrointestinal stromal tumor but was ultimately diagnosed by fine needle aspiration (FNA) to have benign ectopic liver. A 47-year-old male was diagnosed with malignant melanoma of the scalp in May 2015 at a tertiary care hospital. He was found to have a 2.6 cm enhancing mass adjacent to the fundus of the stomach and below the diaphragm by computed tomography imaging. To exclude metastasis, the patient was referred to endoscopy, and an endoscopic ultrasound-guided FNA was performed with rapid on-site evaluation (ROSE) by a cytopathologist. A relatively new FNA needle (Shark Core) was used, which produced useful core biopsy material. Cytopathology demonstrated flat sheets, single cells, and small clusters of polygonal cells. There was abundant granular cytoplasm, often containing pigment. Cells lacked pleomorphism. The smear findings appeared consistent with hepatocytes. The cell block demonstrated small core fragments of hepatic parenchyma with portal tracts. Immunohistochemistry for arginase-1 confirmed that this was hepatic tissue. ROSE was useful for communicating with the endoscopist that the mass was both far from, and not connected to, the liver. This is the first documented account of perigastric ectopic liver diagnosed by FNA. This entity should be considered in the differential of perigastric masses.

• Ectopic liver
• Shark Core needle
• fine needle aspiration

We present a case of hepatocellular carcinoma located within the pancreas. These tumors occur in the body and tail of the pancreas, with a male predominance, and at a younger age. Tumors with pure hepatocellular histopathology have better survival and recurrence rates and should be offered surgical therapy if possible.

• Ectopic
• hepatoid
• pancreas

Developmental abnormalities are rare in the liver. This study presents two case reports of ectopic liver. The first case was a 31-year-old male with clinical indication for laparoscopic appendectomy. Laparoscopy identified a perforated appendix and an unknown tumorous lesion in the ligamentum hepato umbilicalis. The patient underwent a laparoscopic appendectomy, intraoperative lavage of the peritoneal cavity, and extirpation of the lesion in the ligamentum hepato umbilicalis. Histpathological examination of the excised tumor revealed that it comprised liver tissue with fibrinous changes. The tumor was completely separate from the liver with no connection. It was classified as an ectopic liver. No further therapy was required. The second case was a 59-year-old male with a tumor on the upper pole of the spleen, incidentally diagnosed in an ultrasound examination. The biopsy raised suspicion of hepatocellular carcinoma. A positron emission tomography-computed tomography examination revealed accumulation of F-18 fluorodeoxyglucose only in the tumor. The patient underwent a splenectomy with a resection and reconstruction of diaphragm. After the hepatocellular carcinoma was confirmed, adjuvant therapy (sorafenib) was initialized. The operations and postoperative recoveries were uncomplicated in both cases. Despite the low incidence of ectopic liver and rare complications, it is necessary to maintain awareness of this possibility. The potential malignancy risk for ectopic liver tissue is the basis for radical surgical removal. Therapy for hepatocellular carcinoma in an ectopic liver follows the same guidelines as those followed for treating the “mother” liver.

• Ectopic
• Liver
• Hepatocellular carcinoma
• Diagnostic
• Treatment

• Adult
• Biopsy
• Carcinoma, Hepatocellular/diagnosis
• Carcinoma, Hepatocellular/surgery
• Choristoma/diagnosis
• Choristoma/surgery
• Diverticulum/diagnosis
• Diverticulum/surgery
• Humans
• Incidental Findings
• Liver
• Liver Neoplasms/diagnosis
• Liver Neoplasms/surgery
• Male
• Middle Aged
• Splenectomy
• Splenic Neoplasms/diagnosis
• Splenic Neoplasms/surgery
• Tomography, X-Ray Computed
• Treatment Outcome

Ectopic livers are infrequently reported in the literature. The reported size for ectopic livers range from a few millimeters up to several centimeters. They are often clinically silent and incidentally discovered during imaging of the hepatobiliary tract, regional surgical procedures or autopsy. They are predestined for benign liver diseases otherwise observed in normal livers like fatty change or develop malignancies such as hepatocellular carcinoma, in a manner analogous to the parent orthotopic liver. The presence of abnormal alpha 1-antitrypsin retention in an ectopic liver has, to our knowledge, not been reported in the literature. Hereby, we present the first reported case featuring alpha 1-antitrypsin retention in an ectopic liver attached to the fundus of the gallbladder and present the clinical, radiological and pathological findings in a caucasian woman undergoing cholecystectomy for acute cholecystitis. Special liver stains showed an alpha 1-antitrypsin retention which was confirmed immunohistochemically. Although ectopic livers are rare and usually an incidental finding, the radiologist and the surgeon should take this into the differential diagnosis of a mass attached to the gall bladder. A secondary disease should be considered by the pathologist in such a specimen and alpha 1-antitrypsin retention should be ruled out by special liver stains. Finally, such a finding should prompt the managing clinician to exclude systemic alpha-1 antitrypsin deficiency in the patient through further appropriate tests.

A 56-year-old man was found to have a pancreatic tail tumor. His blood chemistry showed no infection with hepatitis B or C virus and no elevations of tumor markers or pancreatic hormones. Abdominal ultrasound showed an encapsulated, rather heterogeneous, hypoechoic tumor, 6.5 cm in maximum diameter, with a beak sign. Helical dynamic CT revealed an irregularly enhanced tumor with pooling of contrast medium in the delayed phase. Abdominal angiography showed a hypervascular tumor. With a tentative diagnosis of non-functional islet-cell tumor, the patient underwent resection of the pancreatic body and tail with splenectomy. The contour of the liver and its surface were normal. In microscopic examination, tumor cells arranged in a trabecular pattern with focal bile pigment resembling hepatocellular carcinoma (HCC). Immunohistochemically, these tumor cells were positivefor HEPPAR-1, CAM5.2, cytokeratin 18 and COX-2, but negative for MUC-1, and cytokeratins 7, 20 and 8. These results supported a diagnosis of HCC without any adenocarcinoma component. The patient is currently doing well without any signs of recurrence in either the remaining pancreas or liver three years after surgery. We report the rare case with ectopic HCC in the pancreas with a review of the literature.

• Hepatocellular carcinoma
• Ectopic liver
• Ectopic hepatocellular carcinoma
• Pancreas
• Pancreatic tumor
• Islet-cell tumor

• Angiography
• Biomarkers/metabolism
• Carcinoma, Hepatocellular
• Choristoma/diagnosis
• Choristoma/metabolism
• Choristoma/pathology
• Humans
• Keratin-18/metabolism
• Keratins/metabolism
• Liver Neoplasms
• Male
• Middle Aged
• Pancreatic Neoplasms/diagnosis
• Pancreatic Neoplasms/metabolism
• Pancreatic Neoplasms/pathology
• Tomography, X-Ray Computed