Presentation
It occasionally presents with abdominal pain. In our series, three patients presented with self-limiting abdominal pain (spontaneous resolution), and a palpable mass was found only in case 2. [analesdepediatria.org]
Abstract An autopsy case, 59-year old woman, of idiopathic portal hypertension with hepatic nodular hyperplasia and intra- and extrahepatic portal vein thrombosis was presented. [jstage.jst.go.jp]
May present with severe, acute abdominal pain with bleeding into the abdomen, leading to shock. Other rare presentations include obstructive jaundice. [patient.info]
Also, a literature review is presented. Resumo: Neste trabalho apresentamos um caso de hiperplasia nodular focal que foi diagnosticado aos seis anos de idade e que está sendo acompanhado até o momento presente. [rb.org.br]
Presentation A 35 -year-old woman with a history of oral contraception presents with right upper quadrant pain. Caption: B-mode gray scale imaging Description: Transabdominal ultrasound shows a mass in the right lobe of the liver. [sonoworld.com]
Entire Body System
- Pain
Seite 428 - Practice guidelines for acute pain management in the perioperative setting: A report by the American Society of Anesthesiologists Task Force on Pain Management, Acute Pain Section. [books.google.de]
May present with pain in the right upper quadrant or the epigastric region. A palpable mass may be noticed by the patient. May present with severe, acute abdominal pain with bleeding into the abdomen, leading to shock. [patient.info]
Patients with significant pain or fullness may require a partial liver resection. On CT and MRI the nodule appears to have central star-shaped scar. [gpnotebook.co.uk]
However, in some cases (e.g., large lesions), symptoms like upper abdominal pain and postprandial fullness may occur. Diagnosis is usually based on imaging, but may require biopsy in unclear cases. [amboss.com]
They often do not have abdominal pain. Amebic abscess has a more indolent presentation, with fever and right upper quadrant pain, in a person who has travelled to an endemic area. [clinicaladvisor.com]
- Asymptomatic
Hemangiomas are usually small and asymptomatic; they occur in 1 to 5% of adults. [merckmanuals.com]
Usually asymptomatic but may cause slight abdominal pain in the epigastrium and in the right hypochondrium, and also bloating. May cause elevations of gamma GT and alkaline phosphatase. [patient.info]
At 70, 37, 33 and 19 months of followup, respectively, patients remained asymptomatic and the lesions stable in imaging tests. References [1] R. Meyers. Tumors of the liver in children. Surg Oncol, 16 (2007), pp. 195-203 [2] J.Y. Choi, H.C. [analesdepediatria.org]
Benign liver tumors and cysts are mainly asymptomatic and are often incidental findings in patients undergoing abdominal imaging. However, in some cases (e.g., large lesions), symptoms like upper abdominal pain and postprandial fullness may occur. [amboss.com]
If the healthcare provider believes that the tumor is asymptomatic and does not pose a major health risk, they will often recommend that FNH be closely monitored, instead of a removal. [dovemed.com]
- Inflammation
Atypical hepatocytes Lack of portal tracts Portal tract chronic inflammation Presence of ductular reaction in fibrous bands Board review answer #2 D. [pathologyoutlines.com]
However, early or small FNH lesions also lack the typical characteristic features of more mature FNH lesions, such as fibrous septa containing chronic inflammation and cholangioles at the interface zone. [path.upmc.edu]
They also exhibit overexpression of Serum Amyloid Alpha (SAA) and C-Reactive Protein (CRP), two proteins of the acute phase of inflammation driven by inflammatory cytokines and chemokines, induced by STAT3. [e-cmh.org]
Paradis V, Champault A, Ronot M, Deschamps L, Valla DC, Vidaud D, Vilgrain V, et al: Telangiectatic adenoma: an entity associated with increased body mass index and inflammation. Hepatology 2007;46:140–146. [karger.com]
- Pallor
The background liver is usually normal, though there may be pallor, fibrosis, or brown pigmentation related respectively to fatty liver disease, glycogen storage disease, iron overload, or rare vascular disorders [ 8 ]. [e-cmh.org]
Gastrointestinal
- Abdominal Pain
It occasionally presents with abdominal pain. In our series, three patients presented with self-limiting abdominal pain (spontaneous resolution), and a palpable mass was found only in case 2. [analesdepediatria.org]
May present with pain in the right upper quadrant or the epigastric region. A palpable mass may be noticed by the patient. May present with severe, acute abdominal pain with bleeding into the abdomen, leading to shock. [patient.info]
They often do not have abdominal pain. Amebic abscess has a more indolent presentation, with fever and right upper quadrant pain, in a person who has travelled to an endemic area. [clinicaladvisor.com]
Larger lesions eventually present with numerous symptoms, the most frequent of which is abdominal pain. On examination, the child usually presents with a right upper abdominal mass [ 15 ]. [omicsonline.org]
- Dyspepsia
Abdominal pain can have many sources including but not limited to dyspepsia, peptic ulcer disease, inflammatory bowel disease, and irritable bowel disease. [clinicaladvisor.com]
In some patients (mainly those with large nodules), FNH causes vague, nonspecific abdominal pain (related to pressure exerted on Glisson's capsule or, if the nodule is superficial, on adjacent organs), dyspepsia, feelings of fullness or epigastric discomfort [ncbi.nlm.nih.gov]
- Early Satiety
Symptoms are more likely if they are > 4 cm; symptoms include discomfort, fullness, and, less often, anorexia, nausea, early satiety, and pain secondary to bleeding or thrombosis. These tumors often have a characteristic highly vascular appearance. [merckmanuals.com]
- Loss of Appetite
Preoperative symptoms included abdominal pain ( n = 36, 63 %), nausea/vomitus ( n = 14, 25 %), fatigue ( n = 21, 37 %), tenderness ( n = 29, 51 %), loss of weight ( n = 13, 23 %) and decreased/loss of appetite ( n = 13, 23 %). [eurjmedres.biomedcentral.com]
Liver, Gall & Pancreas
- Hepatomegaly
Congenital malformation of liver NOS nodular, focal K76.89 Hypertrophy, hypertrophic liver R16.0 ICD-10-CM Diagnosis Code R16.0 Hepatomegaly, not elsewhere classified 2016 2017 2018 2019 Billable/Specific Code Applicable To Hepatomegaly NOS acute K76.89 [icd10data.com]
Most are asymptomatic, but some cause hepatomegaly, right upper quadrant discomfort, or intraperitoneal hemorrhage. Most are detected incidentally on ultrasound or other scans (in Imaging Tests of the Liver and Gallbladder ). [merckmanuals.com]
[…] may be related to the vascular damage caused by this treatment, especially in patients that have undergone haematopoietic stem cell transplantation. 3 The disease is usually asymptomatic, and it most commonly presents as a palpable abdominal mass or hepatomegaly [analesdepediatria.org]
Examination is usually normal but there may be hepatomegaly and/or an arterial bruit over the right upper quadrant. [patient.info]
A symptom are usually rare and includes the following: Serum liver results that are abnormal Fever Abdominal enlargement (especially on the right side) Palpable mass on the right upper abdomen Hepatomegaly or enlargement of the liver Weight loss Causes [diseaseszoom.com]
Cardiovascular
- Hypertension
Although definite etiology of idiopathic portal hypertension in this case was not revealed, the possible causes were discussed. [jstage.jst.go.jp]
[…] portal or pulmonary hypertension 7 The origin of FNH is thought to be due to a hyperplastic growth of normal hepatocytes with a malformed biliary draining system, possibly in response to a pre-existent arteriovenous malformation 1,4. [radiopaedia.org]
Because of the limitations of organ supply, liver transplantation is generally reserved for patients with unresectable tumors either due to location or presence of portal hypertension. [clinicaladvisor.com]
To K76.89 K76.0 Fatty (change of) liver, not elsewhere classified K76.1 Chronic passive congestion of liver K76.2 Central hemorrhagic necrosis of liver K76.3 Infarction of liver K76.4 Peliosis hepatis K76.5 Hepatic veno-occlusive disease K76.6 Portal hypertension [icd10data.com]
The pathophysiological mechanisms underlying neurological decline include venous hypertension, ischemia and a mass effect due to the typically hugely dilated venous structures. The current clinical case has several major points of interest. [em-consulte.com]
- Thrombosis
Abstract An autopsy case, 59-year old woman, of idiopathic portal hypertension with hepatic nodular hyperplasia and intra- and extrahepatic portal vein thrombosis was presented. [jstage.jst.go.jp]
) cystic disease of liver (congenital) ( Q44.6 ) hepatic vein thrombosis ( I82.0 ) hepatomegaly NOS ( R16.0 ) pigmentary cirrhosis (of liver) ( E83.110 ) portal vein thrombosis ( I81 ) toxic liver disease ( K71.- ) Other diseases of liver Approximate [icd10data.com]
Symptoms are more likely if they are > 4 cm; symptoms include discomfort, fullness, and, less often, anorexia, nausea, early satiety, and pain secondary to bleeding or thrombosis. These tumors often have a characteristic highly vascular appearance. [merckmanuals.com]
It is widely believed that the pathogenic mechanism may be related to a hyperplastic response of hepatocytes to haemodynamic disturbances caused by local (vascular abnormalities or local venous thrombosis) or systemic factors (oral contraceptives and [analesdepediatria.org]
HCC and Portal Vein thrombosis Many patients with cirrhosis have portal venous thrombosis and many patients with HCC have thrombosis. These are two common findings and they can be coincidental. [radiologyassistant.nl]
- Heart Failure
However, large hemangiomas occasionally cause arteriovenous shunting sufficient to cause heart failure and sometimes consumption coagulopathy. [merckmanuals.com]
Neurologic
- Confusion
The definition of the type of lesion is essential, and, amongthe lesions more easily confused with FNH, hepatic adenoma shouldbe highlighted because of its unequivocal relationship with theuse of oral contraceptives, especially in high doses and duringlong [rb.org.br]
The diagnosis can be confusing in patients with certain types of vascular tumors that exhibit iso- or hyperdensity in the portal venous and delayed phases. Even in our study, two patients were misdiagnosed with hemangioma. [dovepress.com]
They have connections to the biliary tree and are not true liver masses but can be confused with a cystic liver mass. [clinicaladvisor.com]
Pseudo-portal tracts should not be confused with true portal tracts (containing normal branch of hepatic artery, portal vein and interlobular bile ducts, without a ductular reaction) which may be trapped within the lesion at the borders. [e-cmh.org]
Workup
However, during the abdominal workup, B-mode ultrasound examination showed several focal liver lesions, ranging from 1–5.4 cm in size. Endoscopy and colonoscopy showed no primary gastrointestinal tumor. [em-consulte.com]
Characteristics of common solid liver lesions and recommendations for diagnostic workup. World J Gastroenterol. 2009;15(26):3217–3227. 16. Ronot M, Vilgrain V. Imaging of benign hepatocellular lesions: current concepts and recent updates. [dovepress.com]
Assy N, Nasser G, Djibre A, Beniashvili Z, Elias S, Zidan J: Characteristics of common solid liver lesions and recommendations for diagnostic workup. World J Gastroenterol 2009;15:3217–3227. [karger.com]
Characteristics of common solid liver lesions and recommendations for diagnostic workup. World J Gastroenterol. 2009;15:3217–27. [eurjmedres.biomedcentral.com]
Ultrasound
- Enlargement of the Liver
A symptom are usually rare and includes the following: Serum liver results that are abnormal Fever Abdominal enlargement (especially on the right side) Palpable mass on the right upper abdomen Hepatomegaly or enlargement of the liver Weight loss Causes [diseaseszoom.com]
Biopsy
- Hepatocellular Carcinoma
Although hepatocellular carcinoma is known to accompany Alagille syndrome, HNH should also be considered when large vessels running through the lesion are demonstrated. [ncbi.nlm.nih.gov]
硬化性肝細胞癌 scirrhous hepatocellular carcinoma 肉腫様肝細胞癌 sarcomatoid hepatocellular carcinoma リンパ上皮腫様癌 lymphoepithelioma-like carcinoma 間質浸潤 stromal invasion 混合型肝癌 combined hepatocellular-cholangiocellular carcinoma, combined hepatocellular-cholangiocarcinoma [jsge.or.jp]
carcinoma and other well-differentiated forms of hepatocellular carcinoma. [emedicine.medscape.com]
Gyorffy, E.J., Bredfeldt, J.E., Black, W.C.: Transformation of hepatic cell adenoma to hepatocellular carcinoma due to oral contraceptive use. Ann. Intern. Med. 110 :489, 1989 Google Scholar 25. [link.springer.com]
- Liver Biopsy
Liver function tests are nondiagnostic. A liver scan and arteriogram are often helpful in determining a diagnosis. Percutaneous liver biopsy is contraindicated. [annals.org]
biopsy [ 2 ]. [e-cmh.org]
An ultrasound-guided liver biopsy was then performed, which found no signs of metastatic disease. Figure 1. Enhanced CT scan showing an arteriovenous malformation (AVM) in the lower right lobe of the lung. Zoom Figure 2. [em-consulte.com]
If diagnosis remains unclear, a liver biopsy may be helpful. [nature.com]
Makhlouf HR, Abdul-Al HM, Goodman ZD (2005) Diagnosis of focal nodular hyperplasia of the liver by needle biopsy. Hum Pathol 36: 1210-1216. [omicsonline.org]
Treatment
General Practice Notebook General Practice Notebook The information provided herein should not be used for diagnosis or treatment of any medical condition. [gpnotebook.co.uk]
Coverage of the entire breadth and depth of gastroenterology ranges from biology to pathobiology, as well as diagnosis and treatment of diseases of the gastrointestinal, pancreatic, and hepatobiliary systems. [books.google.com]
Treatment is rarely needed. Hemangiomas are usually small and asymptomatic; they occur in 1 to 5% of adults. [merckmanuals.com]
Treatment options for HCC can be either curative or palliative. There is now substantial research data confirming the effectiveness of treatments for HCC. [clinicaladvisor.com]
Conservative treatment is often sufficient Surgical treatment if symptomatic and/or complications arise For hepatic adenoma Discontinue oral contraceptives If the tumor is > 5 cm → surgical resection due to increased risk of rupture, bleeding, or malignant [amboss.com]
Prognosis
Most often these subtypes do not affect treatment or prognosis (outlook). But one of these subtypes, fibrolamellar, is important to recognize. It is rare, making up less than 1% of HCCs and is most often seen in women younger than age 35. [cancer.org]
Treatment and prognosis FNH is benign, with no malignant potential and a minuscule risk of complication (rupture, hemorrhage) and thus are usually treated conservatively 1. [radiopaedia.org]
Its clinical manifestations are diverse and usually seen distal to the SCAVS site, and its prompt diagnosis and management will make for a better neurological prognosis. [em-consulte.com]
Prognosis and treatment Two main complications can affect HCA: hemorrhage and malignant transformation in HCC [ 53 - 55 ]. Even if rare, these events are difficult to predict and potentially life-threatening. [e-cmh.org]
Etiology
Although definite etiology of idiopathic portal hypertension in this case was not revealed, the possible causes were discussed. [jstage.jst.go.jp]
Etiology of Increased Uptake The cause of the relatively increased Tc-99m sulfur colloid uptake in focal nodular hyperplasia, as opposed to hepatic adenoma, remains controversial. [med.harvard.edu]
The etiology of hepatic adenomatosis is largely unknown, but there are good arguments to suppose a shared disease mechanism with HA [ 42 ]. [karger.com]
Epidemiology Frequency: hepatic hemangioma (most common) > focal nodular hyperplasia ( FNH ) > hepatocellular adenoma (rare) Sex: ♀ > ♂ (∼ 6:1) Age: can appear at any age, but mostly affects young women Most often an incidental finding on imaging tests Etiology [amboss.com]
(Etiology) Focal Nodular Hyperplasia of the Liver is believed to be caused by congenital abnormalities in the liver blood vessels. [dovemed.com]
Epidemiology
Epidemiology HA is usually diagnosed in young females using oral contraceptives. Incidence and prevalence of HA are not exactly known. [karger.com]
Benign liver tumors General Epidemiology Frequency: hepatic hemangioma (most common) > focal nodular hyperplasia ( FNH ) > hepatocellular adenoma (rare) Sex: ♀ > ♂ (∼ 6:1) Age: can appear at any age, but mostly affects young women Most often an incidental [amboss.com]
[…] noncirrhotic liver Immunoreactivity for glutamine synthetase with a map-like pattern of staining Terminology Also known as focal cirrhosis, pedunculated adenoma, solitary hyperplastic nodule, mixed adenoma, hamartoma and hamartomatous cholangiohepatoma Epidemiology [pathologyoutlines.com]
Changing epidemiology of hepatocellular adenoma in the United States: review of the literature. Int J Hepatol 2013;2013:604860. 24. Rooks JB, Ory HW, Ishak KG, Strauss LT, Greenspan JR, Hill AP, et al. Epidemiology of hepatocellular adenoma. [e-cmh.org]
Definition and epidemiology The term focal nodular hyperplasia (FNH), which was coined by Edmondson in 1958 [1], refers to a relatively common benign hepatic tumor. [ncbi.nlm.nih.gov]
Pathophysiology
[…] these lesions may be multiple or can occur as part of a syndrome with hemangiomas, epithelioid hemangioendothelioma, hepatic adenomas, fibrolamellar hepatocellular carcinoma, vascular malformations of the brain, meningiomas, and/or astrocytomas. [1] Pathophysiology [en.wikipedia.org]
This concludes this unit on pathophysiology of the liver. Now you can go on to Specific Liver Diseases. Brief Quiz on what you have just covered >> < Liver Menu [vet.uga.edu]
Pathophysiology FNH most probably arises as a reaction to local hemodynamic instability in the liver. [karger.com]
We describe here in detail this rare case of multiple FNH syndrome associated with lung and spinal arteriovenous malformations (AVM), and briefly discuss the possible pathophysiology underlying the association of these apparently unrelated lesions. [em-consulte.com]
[…] occurrence in childhood More common in females than males (F:M = 8:1) Accounts for ~8% of all primary liver tumors in adults ( Dig Surg 2010;27:24 ) Prevalence of 4 - 30 per 1,000 in adults and 0.2 per 1,000 in persons < 18 years ( Dig Surg 2010;27:24 ) Pathophysiology [pathologyoutlines.com]
Prevention
When doctors discover a large adenoma, they may recommend that it be surgically removed to prevent that possibility. [liverfoundation.org]
Moderation in alcohol, medications and fat intake must be observed to prevent straining the liver. Of the right part of the liver is affected, the gallbladder may also be removed. [diseaseszoom.com]
[…] atypical FNH into several variants which include 8 : telangiectatic variant: most common mixed hyperplastic and adenomatous variant lesions with large cell hepatocellular atypia As FNH is usually treated conservatively, accurate imaging is essential in preventing [radiopaedia.org]
To prevent miscalculation from technical differences, a size change of ≤10% was not considered as growth or regression. [ijri.org]