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Abdelhamed W, El-Kassas M. Fibrolamellar hepatocellular carcinoma: A rare but unpleasant event. World J Gastrointest Oncol 2022; 14:1103-1114. [PMID: 35949219 PMCID: PMC9244987 DOI: 10.4251/wjgo.v14.i6.1103] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2022] [Revised: 03/19/2022] [Accepted: 05/08/2022] [Indexed: 02/06/2023] Open
Abstract
Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), comprising 1%–9% of all HCCs. FLC is a poorly understood malignancy, which seems to be more prevalent in young patients with no underlying liver diseases. The term “fibrolamellar” is derived from thick fibrous collagen bands surrounding the tumor cells. Unlike HCC, cirrhosis and viral hepatitis infection are not predisposing to FLC, and it is not associated with elevations in serum alpha-fetoprotein. FLC patients often present with vague abdominal pain, nausea, malaise, and weight loss. Most cases present are at an advanced stage at the time of initial diagnosis. However, curative treatment options can still be offered to up to 70% of patients. Surgery (resection/liver transplantation) is the mainstay of treatment and the only potentially curative option. FLCs have been less chemo-responsive than the conventional HCC, however, in advanced cases, multimodality treatments can be effective. Recent advances in molecular studies of FLC have found a unique DNAJB1–PRKACA fusion transcript in most of the cases studied. The review aims to describe clinical characteristics, diagnostic methods, and therapeutic modalities for this rare tumor to raise awareness among clinicians and surgeons.
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Affiliation(s)
- Walaa Abdelhamed
- Department of Endemic Medicine, Sohag University, Sohag 14322, Egypt
| | - Mohamed El-Kassas
- Department of Endemic Medicine, Faculty of Medicine, Helwan University, Cairo 11795, Egypt
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2
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Hoshina H, Takei H, Nakamura M, Nishimoto F, Hanamura S. Carcinomatous cirrhosis as radiographically occult liver metastases of breast cancer: A systematic literature review. Cancer Treat Res Commun 2021; 28:100388. [PMID: 34022480 DOI: 10.1016/j.ctarc.2021.100388] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2021] [Revised: 04/12/2021] [Accepted: 05/02/2021] [Indexed: 10/21/2022]
Abstract
In the present study, we aimed to clarify features of carcinomatous cirrhosis from breast cancer presenting as refractory transudate ascites and acute liver failure. In our systematic literature review, we identified 26 studies and 31 cases including our case of this rare condition. Our patient was a 49-year-old woman with a history of ascites and liver failure for the past 4 years and currently being treated for invasive ductal breast cancer. On radiography, she had occult liver metastases that were confirmed using laparoscopic liver biopsy. In the 31 cases, data on the reported year, age, type of primary breast cancer, time from breast cancer diagnosis, presence of ascites and/or varices, liver biopsy, diagnostic modalities, outcomes, and survival were documented and analyzed. All cases were reported during 1984-2020, with a mean patient age of 52.9 years. Eighteen patients (58.1%) were diagnosed with ductal breast cancer. Twenty-two patients (70.9%) had ascites. All patients had gradual progression to liver dysfunction. The following tests were performed: computed tomography (77.4%); ultrasound (58.0%); liver biopsy (100%); postmortem biopsy (35.5%), transjugular liver biopsy (32.3%), and laparoscopic liver biopsy (3.2%). Outcomes were reported for 29 patients, of whom 24 (82.3%) died after 1 day to 16 months. Invasive ductal carcinoma was the most common histological type; however, invasive lobular carcinoma was more frequent (32.3%) than its reported incidence in the breast. Carcinomatous cirrhosis has poor prognosis at relatively rash and is difficult to diagnose with usual modalities. It may be associated with E-cadherin loss or CD44 pronouncement.
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Affiliation(s)
- Hideko Hoshina
- Department of Breast Surgery, Kikuna Memorial Hospital, Yokohama, Kanagawa, Japan; Department of Breast Surgery and Oncology, Nippon Medical School, Tokyo, Japan.
| | - Hiroyuki Takei
- Department of Breast Surgery and Oncology, Nippon Medical School, Tokyo, Japan
| | - Masanori Nakamura
- Department of Gastroenterology, Kikuna Memorial Hospital, Yokohama, Kanagawa, Japan
| | - Fumiya Nishimoto
- Department of Gastroenterology, Kikuna Memorial Hospital, Yokohama, Kanagawa, Japan
| | - Shotaro Hanamura
- Department of Gastroenterology, Kikuna Memorial Hospital, Yokohama, Kanagawa, Japan
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3
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Martelli O, Coppola L, De Quarto AL, Palma M, Sarmiento R, Foggi CM. Fulminant Hepatic Failure Caused by Diffuse Intrasinusoidal Metastatic Liver Disease: A Case Report. TUMORI JOURNAL 2018; 86:424-7. [PMID: 11130575 DOI: 10.1177/030089160008600512] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
A 53-year-old woman experienced rapidly progressing liver failure four years after a quadrantectomy for a breast carcinoma. She had received adjuvant chemotherapy and radiotherapy, and second-line chemotherapy for bone metastasis one year earlier. The hepatic failure manifested with ascites, jaundice, elevation of serum bilirubin and hepatic enzyme levels and hypoalbuminemia. Imaging studies showed an enlarged liver without metastatic lesions. The patient died of hepatic decompensation within two weeks. Liver examination at autopsy revealed massive neoplastic infiltration consistent with a primary breast carcinoma. It is important to realize that this unusual pattern of liver metastasis cannot be demonstrated even with the most advanced techniques of instrumental diagnosis (CT scan, ultrasonography and magnetic resonance imaging), and should be taken into account in the differential diagnosis of rapidly progressing liver failure.
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Affiliation(s)
- O Martelli
- Divisione Oncologia, Azienda Complesso Ospedaliero S. Filippo Neri, Rome, Italy
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Bernardo S, Carvalhana S, Antunes T, Ferreira P, Cortez-Pinto H, Velosa J. A rare cause of acute liver failure- a case report. BMC Gastroenterol 2017; 17:166. [PMID: 29262779 PMCID: PMC5738723 DOI: 10.1186/s12876-017-0730-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2017] [Accepted: 12/14/2017] [Indexed: 12/15/2022] Open
Abstract
Background Acute liver failure (ALF) induced by diffuse metastatic disease has rarely been reported. Case presentation We present a 51-years-old woman with relevant clinical history for breast cancer. The patient was admitted in the emergency department with jaundice, dark urine and pale stools. She was on the 10th day of hormonotherapy for recurrence of breast cancer, diagnosed 7 years previously. Usual causes of acute liver failure were excluded, all drugs were stopped and the imaging studies performed were positive only for steatosis. Nonetheless, ALF progressed and the patient died 4 days later. Autopsy demonstrated a massive intrasinusoidal infiltration of the liver by breast cancer cells. Conclusion We highlight a rare cause of ALF. Although uncommon, physicians should be alert for this situation as the diagnosis can be challenging and the imaging studies can remain normal.
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Affiliation(s)
- Sónia Bernardo
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal.
| | - Sofia Carvalhana
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal
| | - Teresa Antunes
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal
| | - Paula Ferreira
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal
| | - Helena Cortez-Pinto
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal
| | - José Velosa
- Departament of Gastroenterology and Hepatology, Hospital de Santa Maria, CHLN.Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal
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5
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Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. J Hepatocell Carcinoma 2015; 2:151-7. [PMID: 27508204 PMCID: PMC4918295 DOI: 10.2147/jhc.s75153] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma (HCC), which comprises ∼1%–9% of all HCCs. Although FLC is a variant of HCC, it is distinct from HCC in that it most often affects younger patients (10–35 years of age) with no underlying liver disease. FLC often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Surgery is the current mainstay of treatment for FLC and remains the only potentially curative option. While FLCs are considered less responsive to chemotherapy than their classic HCC counterparts, there have been suggestions that multimodality treatments may be effective, especially in advanced cases. Further research is necessary to determine effective systemic therapies as an adjunct to surgery for FLC.
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Affiliation(s)
- Kelly J Lafaro
- Center for Pancreatic Cancer Research, Memorial Sloan Kettering Cancer Center, New York, NY
| | - Timothy M Pawlik
- Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Podduturi V, Guileyardo JM, Soto LR, Krause JR. A case series of clinically undiagnosed hematopoietic neoplasms discovered at autopsy. Am J Clin Pathol 2015; 143:854-60. [PMID: 25972327 DOI: 10.1309/ajcpay9zis1xepro] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
Abstract
OBJECTIVES In the United States, autopsy rates have diminished to less than 5% during the last half of the 20th century and the beginning of the 21st century for a multitude of reasons. Many believe this results in unrecognized malignancies that could have explained a patient's death. METHODS We describe six deaths in which hematopoietic neoplasms were identified at autopsy but were not diagnosed clinically. RESULTS The six undiagnosed hematopoietic malignancy cases discovered at autopsy include four men and two women ranging from 50 to 78 years of age. One patient was African American and five patients were white, all with multiple comorbidities. The tumors included diffuse large B-cell lymphoma, activated B-cell type, intravascular large B-cell lymphoma, ALK-negative anaplastic large cell lymphoma arising in a setting of human immunodeficiency virus, and a myeloid sarcoma. CONCLUSIONS These cases illustrate the importance of the traditional postmortem examination in not only confirming clinical diagnoses but also identifying previously unknown diagnoses. Hematologic malignancies may present with nonspecific clinical manifestations, and this series of cases also emphasizes the necessity for widening the differential diagnosis in patients with unexplained lactic acidosis and hepatic failure to include hematopoietic malignancies since prompt treatment may be lifesaving.
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Affiliation(s)
- Varsha Podduturi
- Department of Pathology, Baylor University Medical Center, Dallas, TX
| | | | - Luis R. Soto
- Department of Pathology, Baylor University Medical Center, Dallas, TX
| | - John R. Krause
- Department of Pathology, Baylor University Medical Center, Dallas, TX
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Mogrovejo E, Manickam P, Amin M, Cappell MS. Characterization of the syndrome of acute liver failure caused by metastases from breast carcinoma. Dig Dis Sci 2014; 59:724-36. [PMID: 24370782 DOI: 10.1007/s10620-013-2943-z] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2013] [Accepted: 10/29/2013] [Indexed: 12/21/2022]
Abstract
AIM To characterize syndrome of acute liver failure (ALF) from metastatic breast cancer to promote premortem diagnosis. Up to now, only 25 % of the reported 32 cases of this syndrome were diagnosed premortem. METHODS Cases identified by computerized literature review and review of files maintained by senior investigator. RESULTS Among 32 cases, average age at presentation was 47.9 ± 9.9 years. Common signs include jaundice, hepatomegaly, shifting dullness, and bilateral leg edema. Mean serum level of AST was 296.4 ± 204.0 U/L, ALT, 183.2 ± 198.9 U/L; alkaline phosphatase, 641.5 ± 610.1; and total bilirubin, 8.6 ± 8.3 mg/dL. Twenty-seven patients (84 %) have known prior breast cancer (mean diagnosis = 4.1 + 4.8 years earlier). Abdominal ultrasound findings (N = 10) include hepatomegaly in three cases, heterogeneous/multifocal hepatic lesions in three, ascites in three, and other in two. Abdominal CT findings (N = 16) include heterogeneous/multifocal hepatic lesions in six cases, ascites in five, hepatomegaly in three, cirrhosis in three, fatty liver in two, other in two. Hepatic metastases may not be suspected when abdominal CT shows no hepatic lesions. The diagnosis is made postmortem in 24 cases and antemortem in eight, with a statistically significant trend of increasing premortem diagnosis since 2000 (0 % before 2000 vs. 50 % after 2000; p = .001, 95 %--ORCI ≥ 2.86, Fisher's exact test). A new case of ALF from breast cancer is reported with notable features: abdominal CT revealed no discrete hepatic lesions despite widespread hepatic metastases demonstrated by liver biopsy; hepatic metastases occurred 21 years after original breast primary; and original diagnosis of lobular breast cancer in primary lesion was corrected to mixed ductal and lobular carcinoma, based on immunohistochemistry, performed 21 years afterward. CONCLUSIONS This review characterizes the clinical presentation and natural history of this syndrome to promote liver biopsy for premortem diagnosis and appropriate therapy.
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Affiliation(s)
- Estela Mogrovejo
- Division of Gastroenterology and Hepatology, MOB 602, Department of Medicine, William Beaumont Hospital, 3535 West Thirteen Mile Road, Royal Oak, MI, 48073, USA
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8
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Lo AA, Lo EC, Li H, Zhang W, Liao J, Rao MS, Miller F, Yang GY. Unique morphologic and clinical features of liver predominant/primary small cell carcinoma--autopsy and biopsy case series. Ann Diagn Pathol 2014; 18:151-6. [PMID: 24667053 DOI: 10.1016/j.anndiagpath.2014.02.007] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2013] [Revised: 01/15/2014] [Accepted: 02/18/2014] [Indexed: 12/17/2022]
Abstract
Liver predominant small cell carcinoma is rare but often presents as hyperacute liver failure with unknown primary and is a medical emergency. We present 2 autopsy and 7 biopsy cases of liver predominant small cell carcinoma and demonstrate that these patients present with liver failure and identifiable hepatomegaly but lack discrete lesions on imaging as well as no mass lesions identified in other organs including lung. Compared with the multiple nodules of metastatic small cell carcinoma in the liver, unique morphologic feature of liver predominant/primary small cell carcinoma in autopsy and biopsy specimens was a diffuse infiltration of small blue neoplastic cells predominantly in the sinusoidal space in the liver parenchyma. Before diagnosing liver predominant/primary small cell carcinoma, other infiltrating small blue cell neoplasms including lymphoma and peripheral neuroectodermal tumor need to be ruled out through immunohistochemistry. We, therefore, demonstrate that liver biopsy together with a rapid panel of immunostains is necessary to firmly establish a diagnosis of liver predominant small cell carcinoma and allow clinicians to immediately implement potentially lifesaving chemotherapy.
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Affiliation(s)
- Amy A Lo
- Department of Pathology, Northwestern University, Chicago, IL 60611
| | - Edward C Lo
- Department of Radiology, University of Illinois at Chicago, Chicago, IL 60612
| | - Haonan Li
- Department of Pathology, Northwestern University, Chicago, IL 60611
| | - Wanying Zhang
- Department of Pathology, Northwestern University, Chicago, IL 60611
| | - Jie Liao
- Department of Pathology, Northwestern University, Chicago, IL 60611
| | - M Sambasivia Rao
- Department of Pathology, Northwestern University, Chicago, IL 60611
| | - Frank Miller
- Department of Radiology, Northwestern University, Chicago, IL 60601
| | - Guang-Yu Yang
- Department of Pathology, Northwestern University, Chicago, IL 60611.
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9
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de Campos FPF, Felipe-Silva A, Zerbini MCN. Anaplastic large cell lymphoma ALK-negative clinically mimicking alcoholic hepatitis - a review. Autops Case Rep 2013; 3:11-19. [PMID: 31528614 PMCID: PMC6671891 DOI: 10.4322/acr.2013.023] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2013] [Accepted: 08/22/2013] [Indexed: 11/24/2022]
Abstract
Anaplastic large cell lymphoma (ALCL), described less than 30 years ago by Karl Lennert and Herald Stein in Kiel, West Germany, is a T-cell or null non-Hodgkin lymphoma, with distinctive morphology (hallmark cells, prominent sinus and/or perivascular growth pattern), characteristic immunophenotype (CD30+, cytotoxic granules protein+, CD3-/+) and specific genetic features as translocations involving the receptor tyrosine kinase called anaplastic lymphoma kinase (ALK) on 2p23 and variable partners genes, which results in the expression of ALK fusion protein. The absence of ALK expression is also observed and is associated with poorer prognosis that seen with ALK expression. ALK-negative ALCL is more frequent in adults, with both nodal and extra nodal clinical presentation and includes several differential diagnoses with other CD30+ lymphomas. Liver involvement by ALCL is rare and is generally seen as mass formation; the diffuse pattern of infiltration is even more unusual. The authors present a case of a 72-year-old man who presented clinical symptoms of acute hepatic failure. The patient had a long history of alcohol abuse and the diagnosis of alcoholic hepatitis was highly considered, although the serum lactic dehydrogenase (LDH) value was highly elevated. The clinical course was fulminant leading to death on the fourth day of hospitalization. Autopsy demonstrated diffuse neoplastic hepatic infiltration as well as splenic, pulmonary, bone marrow, and minor abdominal lymph nodes involvement by the tumor. Based on morphological, immunophenotypical, and immunohistochemical features, a diagnosis of ALK- negative ALCL was concluded. When there is marked elevation of LDH the possibility of lymphoma, ALCL and other types, should be the principal diagnosis to be considered.
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Affiliation(s)
| | - Aloísio Felipe-Silva
- Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil
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10
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Palta R, McClune A, Esrason K. Hodgkin’s lymphoma coexisting with liver failure secondary to acute on chronic hepatitis B. World J Clin Cases 2013; 1:37-40. [PMID: 24303460 PMCID: PMC3845923 DOI: 10.12998/wjcc.v1.i1.37] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2013] [Accepted: 04/02/2013] [Indexed: 02/05/2023] Open
Abstract
Acute on chronic liver failure (ACLF) is rarely the initial manifestation of a malignant process or precipitated by the initiation of anti-viral treatment with a nucleoside or nucleotide agent. We report an unusual case of ACLF temporally associated with initiation of Entecavir for treatment of chronic hepatitis B. Early Hodgkin’s lymphoma (HL) was unmasked with initiation of the anti-viral treatment which may have exacerbated ACLF. To the best of our knowledge, this has not been described in the literature. In reviewing our patients clinical course and liver autopsy, he developed a severe acute exacerbation of his chronic hepatitis B virus coinciding with the institution of antiviral therapy and the underlying HL perhaps modulating the overall degree of hepatic injury.
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11
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Hope BC, Chau KY, Evans HM, Mouat S, Munn S, Yeong ML, Chin SE. Hodgkin disease relapse discovered at the time of liver transplant for acute liver failure. Pediatr Transplant 2012; 16:E10-4. [PMID: 21054714 DOI: 10.1111/j.1399-3046.2010.01380.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Lymphoma is a recognized cause of liver damage and in rare instances presents as ALF. In such cases, the underlying malignancy is often difficult to detect. Historically, the prognosis has been poor. Cure has occasionally been achieved with chemotherapy alone. LT in this setting is controversial, but has contributed to successful outcomes, as in the case of the five-yr-old girl reported here.
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Affiliation(s)
- B C Hope
- Department of Gastroenterology, Starship Children's Hospital, Auckland, New Zealand.
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12
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Esfahani K, Gold P, Wakil S, Michel RP, Solymoss S. Acute liver failure because of chronic lymphocytic leukemia: case report and review of the literature. ACTA ACUST UNITED AC 2011; 18:39-42. [PMID: 21331280 DOI: 10.3747/co.v18i1.756] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Infiltration of the liver by hematologic malignancies is an uncommon cause of liver failure. B-Cell chronic lymphocytic leukemia (cll) is a usually indolent disease that may infiltrate the liver, but based on a review of the literature, has never been reported to induce acute liver failure. Here, we describe the case of a 78-year-old woman with acute liver failure secondary to infiltration with cll being unresponsive to chemotherapy and causing death. This case is notable because of its atypical presentation and ultimate poor prognosis.
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Affiliation(s)
- K Esfahani
- Department of Medicine, McGill University, Montreal, QC
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13
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Abstract
Fibrolamellar hepatocellular carcinoma (FHLCC) generally occurs in young individuals lacking a background of chronic liver disease and other risk factors for hepatocellular carcinoma. The clinical presentations of FLHCC are generally nonspecific, and the alpha-fetoprotein level is typically within the normal range in most cases. Imaging studies have a major role in clinical diagnosis, but pathology is the gold standard in confirming diagnosis. Pathological characteristics of FLHCC include the presence of tumor cells with a deeply eosinophilic cytoplasm and macronucleoli surrounded by abundant fibrous bands. The most effective treatment for FLHCC is aggressive surgical resection. This comprehensive literature review gives a full account of the clinical, pathological, and molecular features of FLHCC.
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14
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Hwang YT, Shin JW, Lee JH, Hwang DS, Eum JB, Choi HJ, Park NH. [A case of fulminant hepatic failure secondary to hepatic metastasis of small cell lung carcinoma]. THE KOREAN JOURNAL OF HEPATOLOGY 2009; 13:565-70. [PMID: 18159155 DOI: 10.3350/kjhep.2007.13.4.565] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Although liver metastasis is commonly found in cancer patients, fulminant hepatic failure secondary to diffuse cancer infiltration into the liver is rare. Liver metastasis-induced fulminant hepatic failure has been reported in patients with primary cancer of the gastrointestinal tract, breast and uroepithelium, and in patients with melanoma and hematologic malignancy. Small cell lung cancer is so highly invasive that hepatic metastasis is common, but rapid progression to fulminant hepatic failure is extremely rare. We report here on a case of a patient who died because of rapid progression to fulminant hepatic failure as a result of hepatic metastasis of small cell lung carcinoma.
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Affiliation(s)
- Young Tae Hwang
- Department of Internal Medicine, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea
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15
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Valero B, Picó Sala MD, Palazón JM, Payá A. Acute liver failure in a patient with hairy cell leukemia. GASTROENTEROLOGIA Y HEPATOLOGIA 2007; 30:338-9. [PMID: 17662217 DOI: 10.1157/13107568] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
Acute liver failure as a manifestation of primary non-Hodkin's lymphoma is a rare phenomenon with a fatal prognosis. Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder, representing about 2 percent of all leukemies. We report a 78-year-old patient with a history of hairy cell leukemia since 10 years, presenting whith fulminant liver failure due to massive liver infiltration. He have reviewed several cases of infiltration of the liver by haematological malignancies, but we only have found after a review in MEDLINE between 1980 and 2006, one case of acute liver failure in a patient with hepatic invasion by hairy cell leukaemia.
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Affiliation(s)
- Beatriz Valero
- Servicio de Medicina Interna, Hospital General Universitario de Alicante, Servicio de Aparato Digestivo, Alicante, España
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16
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Dellon ES, Morris SR, Tang W, Dunphy CH, Russo MW. Acute liver failure due to natural killer-like T-cell leukemia/lymphoma: A case report and review of the Literature. World J Gastroenterol 2006; 12:4089-92. [PMID: 16810767 PMCID: PMC4087729 DOI: 10.3748/wjg.v12.i25.4089] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Acute liver failure (ALF) is a medical emergency requiring immediate evaluation for liver transplantation. We describe an unusual case of a patient who presented with ascites, jaundice, and encephalopathy and was found to have ALF due to natural killer (NK)-like T cell leukemia/lymphoma. The key immunophenotype was CD2+, CD3+, CD7+, CD56+. This diagnosis, which was based on findings in the peripheral blood and ascitic fluid, was confirmed with liver biopsy, and was a contraindication to liver transplantation. A review of the literature shows that hematologic malignancies are an uncommon cause of fulminant hepatic failure, and that NK-like T-cell leukemia/lymphoma is a relatively recently recognized entity which is characteristically CD3+ and CD56+. This case demonstrates that liver biopsy is essential in diagnosing unusual causes of acute liver failure, and that infiltration of the liver with NK-like T-cell lymphoma/leukemia can cause acute liver failure.
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Affiliation(s)
- Evan S Dellon
- University of North Carolina School of Medicine, Division of Gastroenterology and Hepatology, CB#7080, Bioinformatics Bldg, Rm 1140, 130 Mason Farm Rd, Chapel Hill, NC 27599-7080, United States.
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17
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Longerich T, Schirmacher P, Dienes HP, Stein H, Loddenkemper C. Maligne Lymphome in der Leber. DER PATHOLOGE 2006; 27:263-72. [PMID: 16758166 DOI: 10.1007/s00292-006-0838-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
Primary hepatic lymphomas represent rare neoplasms, which are partly observed in association with chronic viral hepatitis, immunosuppression and autoimmune diseases. In contrast, secondary hepatic lymphomas are much more frequent and represent disseminated disease. Lymphomas involving the liver include, with decreasing frequency, diffuse large B-cell lymphoma, small lymphocytic lymphoma, Hodgkin's lymphoma, peripheral T-cell lymphoma, follicular lymphoma and extranodal marginal zone B-cell lymphoma. Many B-cell lymphomas in the liver reveal a characteristic infiltration pattern allowing a rapid and cost-effective diagnosis based on focused immunohistochemical analyses. In contrast, most T-cell lymphomas show a more diverse morphology, which is sometimes difficult to differentiate from a reactive condition. Therefore, additional molecular analyses are frequently necessary. The differential diagnosis includes hepatitis and inflammatory bile duct diseases, undifferentiated carcinoma, inflammatory myofibroblastic tumor as well as histiocytic and dendritic cell neoplasms.
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Affiliation(s)
- T Longerich
- Pathologisches Institut, Universitätsklinikum, Im Neuenheimer Feld 220/221, 69120, Heidelberg.
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18
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McElreath DP, Angtuaco TL, Staggs B, Malik AH. T cell prolymphocytic leukemia: a rare cause of acute liver failure. Dig Dis Sci 2006; 51:819-21. [PMID: 16615010 DOI: 10.1007/s10620-006-3213-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2004] [Accepted: 12/02/2004] [Indexed: 12/09/2022]
Affiliation(s)
- David P McElreath
- Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205-7199, USA
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19
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Bhat YM, Krasinskas A, Craig FE, Shaw-Stiffel TA. Acute liver failure as an initial manifestation of an infiltrative hematolymphoid malignancy. Dig Dis Sci 2006; 51:63-7. [PMID: 16416213 DOI: 10.1007/s10620-006-3085-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/02/2004] [Accepted: 05/11/2005] [Indexed: 12/09/2022]
Affiliation(s)
- Yasser M Bhat
- Department of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213, USA.
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20
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Rajvanshi P, Kowdley KV, Hirota WK, Meyers JB, Keeffe EB. Fulminant hepatic failure secondary to neoplastic infiltration of the liver. J Clin Gastroenterol 2005; 39:339-43. [PMID: 15758630 DOI: 10.1097/01.mcg.0000155123.97418.06] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
Abstract
Two patients with previously normal liver function, who presented with fulminant hepatic failure (FHF) of unknown etiology despite an extensive evaluation, are described. No etiology for FHF was apparent with initial evaluation. One patient was found to have nearly complete replacement of hepatic parenchyma by metastasis from an occult small cell lung carcinoma identified postmortem. The other patient had lymphomatous infiltration of the liver detected by a liver biopsy. Imaging studies were performed in the patients and did not reveal any evidence of neoplastic infiltration of the liver. Neoplastic involvement of liver should be considered in the differential diagnosis of FHF of unknown etiology. The imaging studies in this setting can be misleading.
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Affiliation(s)
- Pankaj Rajvanshi
- Division of Gastroenterology and Hepatology, University of Washington School of Medicine, Seattle, WA 98195-6424, USA
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21
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Boyiadzis M, Nam M, Dahut W. Fulminant Hepatic Failure Secondary to Metastatic Prostate Cancer. Urol Int 2005; 74:185-7. [PMID: 15756074 DOI: 10.1159/000083293] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2003] [Accepted: 01/12/2004] [Indexed: 01/30/2023]
Abstract
The most common sites of metastatic disease from prostate cancer include the bones, lymph nodes and less commonly the lungs, adrenal glands, brain and kidneys. Acute fulminant hepatic failure secondary to cancer metastasis is rare and has unique clinical presentation. We describe a case of fatal liver failure in a 71-year-old male due to metastases to the liver from a prostatic adenocarcinoma. Thus in patients with metastatic cancer and elevated liver function tests, the possibility of hepatic involvement by the cancer should be considered as a possible cause of hepatic failure.
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Affiliation(s)
- Michael Boyiadzis
- Medical Oncology Clinical Research Unit, National Cancer Institute, Bethesda, MD 20892, USA
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22
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Aoki CA, Bowlus CL, Rossaro L. An adult case of acute lymphoblastic leukaemia presenting as hepatic dysfunction. Dig Liver Dis 2005; 37:206-10. [PMID: 15888287 DOI: 10.1016/j.dld.2004.05.016] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2003] [Accepted: 05/10/2004] [Indexed: 12/11/2022]
Abstract
Acute hepatic dysfunction is a rare and often fatal presentation of haematological malignancies. We describe an adult case of acute lymphoblastic leukaemia presenting as an acute hepatitis. Due to the elevation in the patient's transaminases and bilirubin, standard acute lymphoblastic leukaemia induction therapy could not be used. Instead the combination of prednisone and asaparaginase were used to successfully induce remission.
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Affiliation(s)
- C A Aoki
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of California Davis Medical Centre, 4150 V Street, Suite 3500, Sacramento, CA 95817, USA
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23
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Athanasakis E, Mouloudi E, Prinianakis G, Kostaki M, Tzardi M, Georgopoulos D. Metastatic liver disease and fulminant hepatic failure: presentation of a case and review of the literature. Eur J Gastroenterol Hepatol 2003; 15:1235-40. [PMID: 14560159 DOI: 10.1097/00042737-200311000-00014] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Although liver metastases are commonly found in cancer patients, fulminant hepatic failure (FHF) secondary to diffuse liver infiltration is rare. Furthermore, clinical presentation and laboratory findings are obscure and far from being pathognomonic for the disease. We report a case of a patient who died in the intensive care unit of our hospital from multiple organ failure syndrome secondary to FHF, as a result of liver infiltration from poorly differentiated small cell lung carcinoma. We also present the current knowledge about the clinical picture, laboratory findings and physical history of neoplastic liver-metastasis-induced FHF.
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Affiliation(s)
- Elias Athanasakis
- Intensive Care Unit and Laboratory of Pathology, University Hospital of Heraklion, Heraklion, Crete, Greece
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24
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Lettieri CJ, Berg BW. Clinical features of non-Hodgkins lymphoma presenting with acute liver failure: a report of five cases and review of published experience. Am J Gastroenterol 2003; 98:1641-6. [PMID: 12873593 DOI: 10.1111/j.1572-0241.2003.07536.x] [Citation(s) in RCA: 61] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Hematological malignancies frequently affect the liver, but typically do not result in hepatic dysfunction and rarely present with advanced hepatic involvement. We report five patients who presented with advanced hepatic involvement and were found to have a high-grade lymphoma infiltrating the liver. Despite early diagnosis and initiation of therapy, all five patients deteriorated rapidly and died shortly after the onset of liver failure. Several similar cases have been reported. They share common clinical features, including hepatomegaly, lactic acidosis, and death shortly after the onset of symptoms. Non-Hodgkins lymphoma is a common malignancy, which is increasing in incidence. Non-Hodgkins lymphoma is not commonly entertained as an etiology of advanced liver failure and is likely underrecognized. In cases of acute advanced liver failure without an apparent etiology, especially if associated with hepatomegaly and lactic acidosis, lymphoma should be considered because of its poor prognosis and potential for treatment if recognized early. Liver biopsy is an invaluable tool that provides an early diagnosis while excluding other potential etiologies for acute advanced liver failure.
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Affiliation(s)
- Christopher J Lettieri
- Pulmonary/Critical Care Service, Department of Medicine, Walter Reed Army Medical Center, Washington, D.C., USA
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25
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Agarwal K, Jones DEJ, Burt AD, Hudson M, James OFW. Metastatic breast carcinoma presenting as acute liver failure and portal hypertension. Am J Gastroenterol 2002; 97:750-1. [PMID: 11926211 DOI: 10.1111/j.1572-0241.2002.05559.x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Affiliation(s)
- Kosh Agarwal
- Liver Unit, Freeman Hospital, Newcastle-upon-Tyne, United Kingdom
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26
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Bégin LR, Boucher D, Lamoureux E. Diffuse hepatic intravascular carcinomatous embolization resulting in fatal liver failure: a clinicopathologic study of 4 cases. Pathol Res Pract 2002; 197:433-40. [PMID: 11432671 DOI: 10.1078/0344-0338-00057] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
The authors report four cases in which patients died of acute or fulminant hepatic failure resulting from massive intravascular metastatic carcinomatous embolization, a rarely reported manifestation of metastatic disease. Neoplasms were high grade carcinomas. Tumor emboli were present within portal branches ranging 0.12-2.9 mm in diameter and were free floating or attached to the vascular wall, with or without varying degrees of superimposed organization. In one case, intravascular tumor necrosis was prominent and appeared as granular casts with superimposed dystrophic calcification and/or entrapped foamy histiocytes. There were associated geographical areas of parenchymal (4 cases) and tumor (1 case) ischemic necrosis with a multifocal and regional topographic distribution. An associated predominant pattern of intrasinusoidal tumor infiltration (with or without fibrosis) was present in 3 cases, whereas the fourth case had underlying micronodular cirrhosis, providing ancillary evidence for preexisting altered intrahepatic microcirculation. The literature on fatal hepatic failure resulting from neoplasia is reviewed with a reassessment of its pathobiological significance.
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Affiliation(s)
- L R Bégin
- Department of Pathology, McGill University and Sir Mortimer B. Davis Jewish General Hospital, Montreal, Canada.
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27
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Kovalev Y, Lurie M, Naschitz JE, Yeshurun D, Zuckerman E. Metastatic small cell carcinoma presenting as acute hepatic failure. Am J Gastroenterol 2001; 96:3471-3. [PMID: 11774994 DOI: 10.1111/j.1572-0241.2001.05366.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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28
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Morali GA, Rozenmann E, Ashkenazi J, Munter G, Braverman DZ. Acute liver failure as the sole manifestation of relapsing non-Hodgkin's lymphoma. Eur J Gastroenterol Hepatol 2001; 13:1241-3. [PMID: 11711783 DOI: 10.1097/00042737-200110000-00019] [Citation(s) in RCA: 22] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Acute liver failure due to hepatic involvement by haematological malignancies is very rare, and usually has a rapidly fatal prognosis. We describe the case of a man who was treated for diffuse large B-cell lymphoma, and achieved a complete remission after eight courses of chemotherapy. He then presented with acute liver failure. Transjugular liver biopsy revealed massive infiltration by lymphomatous cells, with extensive necrosis. A CT scan did not show any evidence of extrahepatic lymphoma. The patient recovered from the liver failure following combined immuno-chemotherapy. Recurrence of the disease should be included in the differential diagnosis of patients with haematological malignancies and acute liver failure, even without evidence of extrahepatic involvement.
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Affiliation(s)
- G A Morali
- Department of Gastroenterology, Shaare Zedek Medical Centre, Jerusalem, Israel
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29
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Oswiecimski P, Khan A, Salwan R, Marshall R, Berkelhammer C. Profound hypocalcemia in fulminant hepatic failure. Am J Gastroenterol 2000; 95:824-5. [PMID: 10710091 DOI: 10.1111/j.1572-0241.2000.01891.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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30
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Ribera J, Vaquero M, Altet J, Ortega R, Vilardell F. Mujer de 87 años con mal estado general, fiebre, hepatomegalia y hemofagocitosis. Med Clin (Barc) 2000. [DOI: 10.1016/s0025-7753(00)71643-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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31
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Hwang YT, Chen PJ, Kao JH, Wang TD, Wang HH, Chu JS, Su IJ, Chen DS. Rapid hepatic failure associated with a contracted liver mimicking cirrhosis in a case of nasopharyngeal carcinoma with liver metastasis. LIVER 1996; 16:283-7. [PMID: 8878002 DOI: 10.1111/j.1600-0676.1996.tb00744.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
A 46-year-old women had an undifferentiated nonkeratinizing nasopharyngeal carcinoma, locally controlled by radiotherapy. Initially, she had normal liver function tests and normal hepatic ultrasonography. Seven months later, she experienced a rapidly progressive hepatic failure manifested by the development of ascites, elevation of serum bilirubin level, and prolongation of prothrombin time. Imaging studies showed a contracted liver and serum biochemical tests were compatible with chronic liver disease, except for an increase of alkaline phosphatase and gamma-glutamyl transpeptidase levels. An endoscopic retrograde cholangiogram ws compatible with changes of sclerosing cholangitis. The patient died of hepatic decompensation within two months. A liver necropsy disclosed diffuse infiltration of carcinoma cells into the hepatic sinusoids and obliterative angio-invasion of the tumor cells with massive fibrotic stroma replacing almost all hepatocytes. In situ hybridization demonstrated expression of Epstein-Barr virus transcripts EBER1 in the tumor cells and proved a metastatic nasopharyngeal carcinoma. The contracted liver is likely to be explained by the tumor-associated desmoplastic change and the obliterative angio-invasion of the tumor. It is important to be aware that, although rare, such an unusual pattern of liver metastasis may mimick cirrhosis clinically and cause rapid hepatic failure in patients with nasopharyngeal carcinoma.
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Affiliation(s)
- Y T Hwang
- Department of Internal Medicine, College of Medicine, National Taiwan University Hospital, National Taiwan University, Tapei, Taiwan
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32
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33
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Affiliation(s)
- E S Zafrani
- Département de Pathologie, Hôpital Henri Mondor, Créteil, France
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