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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.

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. [ABSTRACT FROM AUTHOR]

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 alphafetoprotein 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. [ABSTRACT FROM AUTHOR]

BackgroundFindings of ectopic hepatocellular carcinoma (EHCC) have been rarely documented. Complicated clinical features and unpredictable medical prognosis make diagnosis and treatment difficult.Case PresentationWe 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.ConclusionHere, 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 (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., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

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. [ABSTRACT FROM AUTHOR]

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.

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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Keywords: adrenal tumor; ectopic hepatocellular carcinoma EN adrenal tumor ectopic hepatocellular carcinoma 1405 1406 2 07/14/20 20200701 NES 200701 Filippos-Paschalis R et al1 described a very interesting and unique case of ectopic hepatocellular carcinoma (HCC) in the adrenal gland with inferior vena cava (IVC) thrombosis and right atrial extension. The adrenal gland, the right atrium tumor, and IVC tumor thrombus were removed successfully. There are select cases of RCC and adrenal HCC with tumor thrombus extension which can be removed safely from the right atrium and IVC without the use of CPB.2,7,8 It is important to remember that these tumors do not cause thrombosis of the IVC, as the tumor thrombus (different from thrombosis) extends into the IVC. [Extracted from the article]

We describe the case of a 72 year-old man with a huge tumor in his lower abdomen and extremely high serum α-fetoprotein levels (99 100 ng/ml). The patient had no risk factors for hepatocellular carcinoma (HCC) or liver disease. Computed tomography, magnetic resonance imaging, and hepatic angiography detected no tumors in the liver before surgery. The arteries feeding the tumor arose from the superior mesenteric artery, but were not recognized on celiac angiography. Histologically, the tumor cells had features of HCC. Immunohistochemical staining revealed that the tumor cells were positive for AFP and the hepatocyte paraffin 1 monoclonal antibody. Furthermore, the tumor cells were strongly positive for cytokeratin 8 and cytokeratin 18, which are usually expressed on hepatocytes in HCC, and negative for both cytokeratin 7 and cytokeratin 20, which are not usually expressed in HCC. Hence, the tumor was diagnosed as an ectopic HCC that possibly developed from ectopic liver tissue in the jejunum. Approximately 2 months after the operation, transarterial chemoembolization was performed for liver metastasis from this tumor. One year after the transarterial chemoembolization procedure, the patient remains well with no evidence of a recurrent tumor or serum AFP elevation. [ABSTRACT FROM AUTHOR]

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., Competing Interests: Conflict-of-interest statement: The authors declare that they have no conflicts of interest., (©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.)

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.