Ectopic hepatocellular carcinoma, an unexpected diagnosis of a retroperitoneal mass: A case report and literature review.

Ectopic hepatocellular carcinoma (HCC) is defined as HCC arising from hepatic parenchyma located in an extrahepatic organ or tissue without any communication with the mother liver. It is very rare and difficult to diagnose by imaging alone. We report a case of a rare ectopic HCC mimicking a right para-aortic retroperitoneal mass and present a review of the literature. It is about a 79-year-old female patient, who presented with a progressive enlarged right paraaortic retroperitoneal mass, thought first to be leiomyosarcoma of vena cava on imaging. Subsequently, high alpha-fetoprotein (AFP) level and biopsy allowed the diagnosis of primary extrahepatic hepatocellular carcinoma.

A rare and challenging case of extrahepatic costal metastases from an unknown primary hepatocellular carcinoma.

Hepatocellular carcinoma (HCC) typically presents with a primary hepatic mass. Nevertheless, on rare occasions, the initial presentation can be exclusively related to extrahepatic metastases and the most common sites of metastases are the lungs, lymph nodes, bones, and adrenal glands. While, bone metastases are generally accompanied by multiple metastatic spreads elsewhere in the body or previously diagnosed HCC, cases of solitary bone metastases with no liver lesion at imaging have been reported. Indeed, two rare entities of HCC have been reported in the literature which are the ectopic hepatocellular carcinoma and the infiltrative type of hepatocellular carcinoma with a very challenging radiologic diagnosis and poor prognosis. In this article, we present a case of extrahepatic costal metastases of hepatocellular carcinoma, which was diagnosed through a bone biopsy, with no focal lesion on liver imaging including ultrasound, multiphase MRI, and CT scan except for the presence of a portal vein thrombosis. It is important to consider the possibility of HCC metastases when evaluating rapidly growing extrahepatic lesions in patients with chronic liver disease and to consider the tumor characteristics and imaging findings as well as limitations to make accurate and timely diagnosis leading to improved patient management. Our patient had probably an infiltrating HCC because of two prominent factors: the presence of portal vein thrombosis and a markedly elevated alpha-fetoprotein (AFP). A liver biopsy was crucial in order to confirm the diagnosis but unfortunately it could not be performed because of the unexpected death of the patient due to hemorrhagic shock. It is also worth noting in this case, that the elevated level of AFP raised the suspicion on an underlying HCC and contributed to more elaborate diagnostic tests.

Ectopic Hepatocellular Carcinoma in the Gallbladder Wall With Choledocholithiasis: A Case Report.

An ectopic hepatocellular carcinoma (HCC) that originates from the ectopic liver is defined as a hepatic organ or tissue not conventionally linked to surrounding tissues. Ectopic HCC has a rare clinical incidence, and diagnosing it before surgery is challenging. Its characteristics and biological behavior have not been fully elucidated. This report presents a unique case of ectopic HCC in the gallbladder, discontinuous with the liver. A 74-year-old man was referred to our hospital after primarily complaining of fever and right hypochondrium pain. Plain computed tomography revealed a significantly thickened gallbladder wall containing fluid collection and incarceration of gallstone in the common bile duct. He was diagnosed with acute cholecystitis and obstructive cholangitis due to choledocholithiasis. After percutaneous transhepatic gallbladder drainage and endoscopic lithotomy for choledocholithiasis, a cholecystectomy was performed. Macroscopically, the resected gallbladder had a yellowish tumor (1 cm) within the significantly thickened gallbladder wall. Histopathological examination identified moderately differentiated HCC on the ectopic liver, discontinuous with the liver. Immunohistologically, the tumor was finally diagnosed as ectopic HCC with alpha-fetoprotein positive expression.

Left epigastric isolated tumor fed by the inferior phrenic artery diagnosed as ectopic hepatocellular carcinoma: A case report.

BACKGROUND: Hepatocellular carcinoma (HCC) is one of the most frequent cancers and the main cause of cancer-related death worldwide. Ectopic HCC, an extremely rare type of HCC, exhibits a wide range of clinical signs and radiographic features, making preoperative identification challenging. CASE SUMMARY: A 47-year-old man underwent routine abdominal color ultrasonography, which identified an asymptomatic tumor in the left upper abdomen. The patient had no history of hepatitis, did not drink alcohol, and had no family history of cancer. Abdominal contrast-enhanced computed tomography (CT) revealed a heterogeneously enhanced lesion between the spleen and stomach that had invaded the diaphragm, with blood supplied by the left inferior phrenic artery. The patient underwent laparoscopic surgery, and HCC was identified by postoperative pathology. Additionally, specific immunohistochemical staining was performed to assess the molecular biological characteristics of the HCC. The patient underwent two rounds of hepatic arterial interventional chemotherapy after surgery. Abdominal plain and enhanced magnetic resonance imaging and lung CT 3 mo postoperatively revealed no signs of local recurrence or distant metastasis. CONCLUSION: This asymptomatic ectopic HCC case described achieved an excellent result due to early detection, radical resection, and systematic surveillance.

A large ectopic hepatocellular carcinoma with adrenal infiltration: a rare case report.

Ectopic hepatocellular carcinoma (EHCC) originates from the ectopic liver, which refers to a liver organ or tissue unrelated to surrounding tissues. EHCC is a rare disease that lacks specific clinical signs, and preoperative diagnosis is often difficult. In a 61-year-old male patient with positive hepatitis B virus antibody, abdominal contrast-enhanced computed tomography scan showed a large heterogenously enhancing mass both on arterial and portal venous phase imaging arising from the right adrenal gland. Similar enhancement features were seen on magnetic resonance imaging. Serum potassium, aldosterone, cortisol, and plasma metanephrines were normal. The tumor markers of serum alpha-fetoprotein and alpha-fetoprotein-L3% were increased to 23.69 ng/mL and 82.1%, respectively. Exploratory laparotomy was performed and operative findings showed that the retroperitoneal tumor was disconnected from the right kidney and the liver, but invaded the right adrenal gland. Immunohistochemical examination showed that Arginase-1 was positive expression, and the retroperitoneal tumor was finally diagnosed as EHCC. We report a rare EHCC with adrenal infiltration that is difficult to diagnose preoperatively and mimics a retroperitoneal tumor or adrenal tumor, and we present a review of the literature on EHCC case reports.

Challenge in Diagnosis and Treatment of Ectopic Hepatocellular Carcinoma: A Case Report and Literature Review.

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

Ectopic hepatocellular carcinoma mimicking a retroperitoneal tumor: A case report.

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

Ectopic hepatocellular carcinoma in the adrenal gland with inferior vena cava thrombosis and right atrial extension.

Intravascular tumor extension in the inferior vena cava (IVC) is known to occur with abdominal tumors, such as renal cell, hepatocellular, adrenal cell carcinoma, and Wilm's tumor. We encountered a 53-year-old male patient presenting with pulmonary embolism and a right atrial mass with imaging evidence of an adrenal tumor extending into the IVC, up to the right atrium. The patient underwent surgery for the resection of the tumor using cardiopulmonary bypass by a team of cardiothoracic surgeons and urologists. Histology identified the tumor as hepatocellular carcinoma, which developed as ectopic hepatic tissue in the right adrenal gland.

A case report: delayed high fever and maculopapules during Sorafenib treatment of ectopic hepatocellular carcinoma.

BACKGROUND: Sorafenib is the standard first-line therapy for hepatocellular carcinoma (HCC) and probably ectopic hepatocellular carcinoma (EHCC) as well. No report involves a side effect of delayed high fever of sorafenib. This manuscript describes a case of EHCC in the thoracic and abdominal cavities, who showed a delayed high fever and maculopapules during sorafenib treatment. CASE PRESENTATION: The patient is a 63-year-old Chinese male with advanced EHCC, taking sorafenib 400 mg twice daily. On the tenth day, red maculopapules appeared all over the body. On the same day, the patient began to suffer from continuous high fever. Due to these effects, the patient was asked to cease sorafenib treatment, and the high fever and maculopapules were alleviated quickly. However, the symptoms were present again upon re-challenge of sorafenib. Prednisone was then administered to control the symptoms, with the dosage gradually reduced from 30 to 5 mg/day in 1.5 months. No recurrence of fever or maculopapules has been found. Tumor response reached partial response (PR) and progression free survival (PFS) reached 392 days + by the date of Apr. 14th, 2016. CONCLUSION: EHCC could be treated like orthotopic HCC by oral administration of sorafenib, which shows good tumor response and survival benefit. Delayed high fever and maculopapules are potential, rare and severe side effects of sorafenib, and could be effectively controlled by glucocorticoid.

Ectopic Hepatocellular Carcinoma Arising from the Peritoneum in a Patient with a History of Oropharyngeal Cancer: A Case Report.

A subphrenic mass was noted on a surveillance computed tomography (CT) scan of a 65-year-old man who had achieved complete remission of oropharyngeal cancer after concurrent chemoradiotherapy. The mass was 3.2 cm in size and showed a multilobular enhancing pattern along the peritoneal lining. The patient was negative for hepatitis B surface antigen and anti-hepatitis C virus antibody. His carcinoembryonic antigen level was within the normal range. Contrast-enhanced CT revealed no mass in the liver. He underwent surgery, and a pale yellowish soft tumor measuring 3.8 × 3.2 × 1.2 cm was resected. Histologically, the tumor was confirmed to be a hepatocellular carcinoma. Currently, he is doing well, and has been followed up without any signs of recurrence.

Multiple ectopic hepatocellular carcinomas arising in the abdominal cavity.

Ectopic hepatocellular carcinoma (HCC) is a very rare clinical entity that is defined as HCC arising from extrahepatic liver tissue. This report presents a case of ectopic multiple HCC arising in the abdominal cavity. A 42-year-old otherwise healthy male presented with liver dysfunction at a general health checkup. Both HCV antibody and hepatitis B surface antigen were negative. Laboratory examination showed elevations in serum alpha-fetoprotein and PIVKA-II. Ultrasonography and computed tomography revealed multiple nodular lesions in the abdominal cavity with ascites without a possible primary tumor. Exploratory laparoscopy was performed, which revealed bloody ascites and multiple brown nodular tumors measuring approximately 10 mm in size that were disseminated on the perineum and mesentery. A postoperative PET-CT scan was performed but it did not reveal any evidence of a tumor in the liver. The tumors resected from the peritoneum were diagnosed as HCC. The present case of HCC was thought to have possibly developed from ectopic liver on the peritoneum or mesentery.