|
1
|
Nakamura S, Kakegawa T, Takeuchi H, Takahashi H, Tomita Y, Abe M, Yoshimasu Y, Kasai Y, Sugimoto K, Furuichi Y, Itoi T. A case of hepatic adrenal rest tumor diagnosed by various clinical examination findings. KANZO 2022; 63:69-76. [DOI: 10.2957/kanzo.63.69] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 04/14/2025]
Affiliation(s)
- Shunsuke Nakamura
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Tatsuya Kakegawa
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Hirohito Takeuchi
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Hiroshi Takahashi
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Yusuke Tomita
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Masakazu Abe
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Yu Yoshimasu
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | - Yoshitaka Kasai
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| | | | | | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University
| |
Collapse
|
|
2
|
Chen J, Wan X, Lu Y, Wang W, Zhao D, Lu Z, Mao Y, Chen J. An ectopic adrenocortical oncocytic adenoma in the liver highly mimicking hepatocellular carcinoma: case report and literature review. Diagn Pathol 2021; 16:58. [PMID: 34218806 PMCID: PMC8255004 DOI: 10.1186/s13000-021-01097-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2020] [Accepted: 04/13/2021] [Indexed: 11/22/2022] Open
Abstract
Background Ectopic adrenocortical tissue is a lesion usually found incidentally during autopsy or inguinal surgery. Here, we demonstrate an extremely unusual case of intrahepatic adrenocortical adenoma which highly mimicks hepatocellular carcinoma (HCC) and brings challenges for clinicians and pathologists. The diagnostic pitfalls have been discussed in detail to provide clues for guiding differential diagnosis and future treatment. Case presentation A 44-year-old man was admitted into our hospital for evaluation of a hepatic mass identified during routine examination. Enhanced CT revealed its margin displayed apparent enhancement in arterial phase, but hypointensity in portal and delayed phase. HCC was suspected and partial hepatectomy was performed. Microscopically, cells were arranged in solid sheets. Most of the tumor cells were large, polygonal, had prominent nucleoli and were rich in eosinophilic cytoplasm. Pleomorphic nucleus was frequently found. Focally, smaller cells were found with small nuclei and granular cytoplasm. Immunohistochemically, tumor cells were negative for Arg-1, glypican-3 (GPC3), hepatocyte specific antigen (HSA), and positive for synaptophysin (Syn), α-inhibin, and Melan A. The Ki-67 index was 1 %. The final diagnosis was ectopic adrenocortical oncocytic adenoma and the patient was uneventful after the surgery. Conclusion Intrahepatic adrenocortical adenoma in the liver can hardly be diagnosed through radiology and little experience in pathology has been reported. In the present case, massive oncocytic changes and huge pleomorphism add greatly to the difficulties of making correct diagnosis. This lesion should be carefully kept in mind and a combination of markers is suggested for differentiating from HCC.
Collapse
Affiliation(s)
- Jingci Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Xueshuai Wan
- Department of Liver Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Yao Lu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Wenze Wang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Dachun Zhao
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Zhaohui Lu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Yilei Mao
- Department of Liver Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China
| | - Jie Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100730, Beijing, China.
| |
Collapse
|
|
3
|
Bannasch P, Ribback S, Su Q, Mayer D. Clear cell hepatocellular carcinoma: origin, metabolic traits and fate of glycogenotic clear and ground glass cells. Hepatobiliary Pancreat Dis Int 2017; 16:570-594. [PMID: 29291777 DOI: 10.1016/s1499-3872(17)60071-7] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2017] [Accepted: 08/14/2017] [Indexed: 02/05/2023]
Abstract
Clear cell hepatocellular carcinoma (CCHCC) has hitherto been considered an uncommon, highly differentiated variant of hepatocellular carcinoma (HCC) with a relatively favorable prognosis. CCHCC is composed of mixtures of clear and/or acidophilic ground glass hepatocytes with excessive glycogen and/or fat and shares histology, clinical features and etiology with common HCCs. Studies in animal models of chemical, hormonal and viral hepatocarcinogenesis and observations in patients with chronic liver diseases prone to develop HCC have shown that the majority of HCCs are preceded by, or associated with, focal or diffuse excessive storage of glycogen (glycogenosis) which later may be replaced by fat (lipidosis/steatosis). In ground glass cells, the glycogenosis is accompanied by proliferation of the smooth endoplasmic reticulum, which is closely related to glycogen particles and frequently harbors the hepatitis B surface antigen (HBsAg). From the findings in animal models a sequence of changes has been established, commencing with preneoplastic glycogenotic liver lesions, often containing ground glass cells, and progressing to glycogen-poor neoplasms via various intermediate stages, including glycogenotic/lipidotic clear cell foci, clear cell hepatocellular adenomas (CCHCA) rich in glycogen and/or fat, and CCHCC. A similar process seems to take place in humans, with clear cells frequently persisting in CCHCC and steatohepatitic HCC, which presumably represent intermediate stages in the development rather than particular variants of HCC. During the progression of the preneoplastic lesions, the clear and ground glass cells transform into cells characteristic of common HCC. The sequential cellular changes are associated with metabolic aberrations, which start with an activation of the insulin signaling cascade resulting in pre-neoplastic hepatic glycogenosis. The molecular and metabolic changes underlying the glycogenosis/lipidosis are apparently responsible for the dramatic metabolic shift from gluconeogenesis to the pentose phosphate pathway and Warburg-type glycolysis, which provide precursors and energy for an ever increasing cell proliferation during progression.
Collapse
Affiliation(s)
| | - Silvia Ribback
- Institut für Pathologie, Universitätsmedizin Greifswald, Greifswald, Germany
| | - Qin Su
- Cell Marque, Millipore-Sigma Rocklin, USA
| | - Doris Mayer
- German Cancer Research Center, Heidelberg, Germany
| |
Collapse
|
|
4
|
Bannasch P. Glycogenotic hepatocellular carcinoma with glycogen-ground-glass hepatocytes: A heuristically highly relevant phenotype. World J Gastroenterol 2012; 18:6701-6708. [PMID: 23239906 PMCID: PMC3520157 DOI: 10.3748/wjg.v18.i46.6701] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/09/2012] [Revised: 09/21/2012] [Accepted: 11/15/2012] [Indexed: 02/06/2023] Open
Abstract
Glycogenotic hepatocellular carcinoma (HCC) with glycogen-ground-glass hepatocytes has recently been described as an allegedly “novel variant” of HCC, but neither the historical background nor the heuristic relevance of this observation were put in perspective. In the present contribution, the most important findings in animal models and human beings related to the emergence and further evolution of excessively glycogen storing (glycogenotic) hepatocytes with and without ground glass features during neoplastic development have been summarized. Glycogenotic HCCs with glycogen-ground-glass hepatocytes represent highly differentiated neoplasms which contain subpopulations of cells phenotypically resembling those of certain types of preneoplastic hepatic foci and benign hepatocellular neoplasms. It is questionable whether the occurrence of glycogen-ground-glass hepatocytes in a glycogenotic HCC justifies its classification as a specific entity. The typical appearance of ground-glass hepatocytes is due to a hypertrophy of the smooth endoplasmic reticulum, which is usually associated with an excessive storage of glycogen and frequently also with an expression of the hepatitis B surface antigen. Sequential studies in animal models and observations in humans indicate that glycogen-ground-glass hepatocytes are a facultative, integral part of a characteristic cellular sequence commencing with focal hepatic glycogenosis potentially progressing to benign and malignant neoplasms. During this process highly differentiated glycogenotic cells including ground-glass hepatocytes are gradually transformed via various intermediate stages into poorly differentiated glycogen-poor, basophilic (ribosome-rich) cancer cells. Histochemical, microbiochemical, and molecular biochemical studies on focal hepatic glycogenosis and advanced preneoplastic and neoplastic lesions in tissue sections and laser-dissected specimens in rat and mouse models have provided compelling evidence for an early insulinomimetic effect of oncogenic agents, which is followed by a fundamental metabolic switch from gluconeogenesis towards the pentose-phosphate pathway and the Warburg type of glycolysis during progression from preneoplastic hepatic glycogenosis to the highly proliferative malignant phenotype.
Collapse
|
|
5
|
Tajima T, Funakoshi A, Ikeda Y, Hachitanda Y, Yamaguchi M, Yokota M, Yabuuchi H, Satoh T, Koga M. Nonfunctioning adrenal rest tumor of the liver: radiologic appearance. J Comput Assist Tomogr 2001; 25:98-101. [PMID: 11176302 DOI: 10.1097/00004728-200101000-00018] [Citation(s) in RCA: 37] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
We describe the radiologic features of an adrenal rest tumor of the liver. The adrenal rest tumor appeared on ultrasound as a round, well defined, heterogeneous, solid mass in the posterior aspect of the liver, on angiography as a homogeneous hypervascular mass, and on dynamic CT as a mass containing components of both fat density and soft tissue density and showing early fill-in and early fill-out. Adrenal rest tumors should be included in the lists of hypervascular or fat-containing masses in the liver.
Collapse
Affiliation(s)
- T Tajima
- Department of Radiology, National Kyushu Cancer Center, Fukuoka, Japan.
| | | | | | | | | | | | | | | | | |
Collapse
|
|
6
|
Arai K, Muro H, Suzuki M, Oba N, Ito K, Sasano H. Adrenal rest tumor of the liver: a case report with immunohistochemical investigation of steroidogenesis. Pathol Int 2000; 50:244-8. [PMID: 10792789 DOI: 10.1046/j.1440-1827.2000.01029.x] [Citation(s) in RCA: 20] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
A case of adrenal rest tumor arising in the liver of a 62-year-old male with chronic hepatitis type C is reported. The tumor was clinically non-functioning and required distinction from hepatocellular carcinoma. The yellowish-brown tumor measured 25 x 18 x 15 mm and was located in the subcapsular portion of the right hepatic lobe. Histologically, the tumor presented features similar to those of the adrenal cortex and was predominantly composed of pale cells. Electron micrograph revealed lipid droplets and mitochondria with tubulo-vesicular cristae, consistent with the characteristics of steroid-producing cells. Immunohistochemically, the tumor expressed the adrenal 4 binding protein and a number of enzymes involved in the synthesis of adrenocortical steroids. At surgery, the right adrenal gland was present independently from the liver. This hepatic tumor was considered to be an adrenal rest tumor with steroidogenic capability.
Collapse
Affiliation(s)
- K Arai
- Departments of Pathology, Shizuoka General Hospital, Shizuoka, Japan
| | | | | | | | | | | |
Collapse
|
|
7
|
Adamek HE, Spiethoff A, Kaufmann V, Jakobs R, Riemann JF. Primary clear cell carcinoma of noncirrhotic liver: immunohistochemical discrimination of hepatocellular and cholangiocellular origin. Dig Dis Sci 1998; 43:33-8. [PMID: 9508531 DOI: 10.1023/a:1018859617522] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Affiliation(s)
- H E Adamek
- Department of Medicine C, Klinikum Ludwigshafen, Academic Hospital of the University of Mainz, Germany
| | | | | | | | | |
Collapse
|
|
8
|
Jaeck D, Pâris F, Welsch M, Stephan D, Chenard-Neu MP, Steib A, Bachellier P, Bellocq JP, Imbs JL. Primary hepatic pheochromocytoma: a second case. Surgery 1995; 117:586-90. [PMID: 7740432 DOI: 10.1016/s0039-6060(05)80260-0] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Affiliation(s)
- D Jaeck
- Centre de Chirurgie Viscerale et de Transplantation, Hôpital de Hautepierre, Strasbourg, France
| | | | | | | | | | | | | | | | | |
Collapse
|
|
9
|
Wallace EZ, Leonidas JR, Stanek AE, Avramides A. Endocrine studies in a patient with functioning adrenal rest tumor of the liver. Am J Med 1981; 70:1122-5. [PMID: 7234878 DOI: 10.1016/0002-9343(81)90886-x] [Citation(s) in RCA: 33] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
We report a case of a calcified liver tumor in a 23 year old female patient who presented with virilization and a mild degree of Cushing's syndrome. Androgen levels were elevated; there was loss of cortisol circadian rhythm and marked increase in urinary 17-ketogenic and 17-ketosteroids which failed to suppress with administration of dexamethasone. Venous sampling by inferior vena cava catheterization showed that the highest steroid hormone levels were in blood from the right hepatic vein. After death, in vitro studies revealed that the tumor contained testosterone and cortisol as determined by immunofluorescence techniques. The adrenals and ovaries were atrophic. Results of metyrapone testing indicated dyshormonogenesis. To our knowledge, this is the first case of an adrenal rest tumor of the liver proved to be functionally active.
Collapse
|
|
10
|
|
|
11
|
|
|
12
|
|
|
13
|
|