Liver hemangioma or cavernous hemangioma is a presumably congenital growth that is benign, consisting of a large cavity with a generous supply of arterial blood. In most people, the condition remains unnoticed causing no particular signs or symptoms.
Presentation
Hepatic angiomas are frequently located in the right lobe of the liver but which are mostly small and clinically insignificant. Oftentimes these are incidentally seen only in routine laparotomy for other medical purposes, or at necropsy. Goodman documented lesions measuring 4 cm, causing symptoms in 40% of patients and 90% among those with 10 cm hemangiomas [7]. Symptoms are usually associated with large and multiple angiomas. Chief complaints are feeling of fullness or right upper quadrant pain, due to pressure on adjacent tissues or organs, hemorrhage, infarcts and thrombosis at the site of lesions.
Physical examination reveals hepatomegaly or arterial bruit on auscultation at the level of the right lobe. Presence of a large abdominal mass is rare. Complications may develop such as:
- Jaundice from biliary duct obstruction
- Arteriovenous shunting leading to heart failure
- Bleeding in the biliary passages (hemobilia) [8]
- Fever of unknown origin (FUO) [9].
Other relevant clinical findings are: weight loss, anemia, fever, thrombocytosis, and elevated fibrinogen and elevated erythrocyte sedimentation rate [10].
Entire Body System
- Asymptomatic
Guidelines for management of the asymptomatic liver hemangioma have been suggested. [ncbi.nlm.nih.gov]
The majority of Liver Hemangiomas is generally asymptomatic and presents no signs and symptoms. [dovemed.com]
- Anemia
The patient showed signs of anemia, inflammatory markers and a transient increase in creatinine levels, which were corrected by conservative treatment. Our patient denied the previous use of estrogen derivatives, smoking or alcohol consumption. [ncbi.nlm.nih.gov]
Sometimes these patients will have anemia or a low platelet count, this is because the red blood cells and platelets may be trapped and destroyed by the malformation in the liver. [novanews.org]
- Congestive Heart Failure
It is possible for congestive heart failure to exist in a patient with a focal liver hemangioma. These lesions are essentially Rapidly Involuting Congenital Hemangioma (RICH ). [novanews.org]
General Benign. [2] Can be hard to differentiate from metastatic disease on imaging. [1] May cause congestive heart failure in infants - if large. [3] May rupture and be life-threatening. [4] Clinical: Do not grow in size - can be followed if small or [librepathology.org]
It is more often found in the child where it may be associated with congestive heart failure. The lesions are large, poorly defined, confluent, replacing almost all of the hepatic parenchyma.” [ctisus.com]
Main complications include torsion due to a long and mobile pedicle, infarction ( 5, 6 ), spontaneous or traumatic rupture, congestive heart failure, and Kasabach-Merritt syndrome ( 2, 6, 7 ). [ncbi.nlm.nih.gov]
Surgeons opt for resection than risk spontaneous rupture and complication such as, congestive heart failure or intratumoral hemorrhage. Liver hemangiomas are usually benign posing no particular health problem for most people. [symptoma.com]
- Swelling
“They did however, let us know that surgery outside the country was an option should I become overwhelmed with the swelling,” said Mrs Nacheri. [checkorphan.org]
Over time, too much copper in the liver can lead to symptoms such as jaundice and swelling of the abdomen and, untreated, lead to acute liver failure, a need for liver transplantation, or death. [uhnj.org]
But they could build up thick enough to add pressure to the liver, they can swell and push against a nerve, causing pain, one of the blood vessels could burst causing bruising, they could result in an enlarged liver, cause nausea. [city-data.com]
Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain [emedicine.medscape.com]
- Chills
Photo Credit Dolly Faibyshev for The New York Times Work Out and Chill? Cool temperature workouts may be the answer for those who want to exercise without becoming a hot mess. [nytimes.com]
Gastrointestinal
- Right Upper Quadrant Pain
Chief complaints are feeling of fullness or right upper quadrant pain, due to pressure on adjacent tissues or organs, hemorrhage, infarcts and thrombosis at the site of lesions. [symptoma.com]
Right upper quadrant pain or fullness is the most common symptom. Examination is usually normal but there may be hepatomegaly and/or an arterial bruit over the right upper quadrant. [patient.info]
[…] enlarge Photo 4 click to enlarge Ishak reviewed 89 cases and found only 13.5% with clinical symptom.7 Right upper quadrant pain or fullness is the most common complain. [abdominalsurg.org]
Adenomas may rupture and bleed, causing right upper quadrant pain. The two most common liver lesions causing hepatic hemorrhage are HA and HCC. [radiologyassistant.nl]
Liver, Gall & Pancreas
- Liver Mass
Native computed tomography identified a liver mass measuring 73×63 mm, located in segment IV and bulging out of the anterior contour of the liver. [ncbi.nlm.nih.gov]
"Because of the opportunity to assess liver masses using other sequences beyond the multiphasic contrast scanning available by CT, MRI imaging is moving towards becoming the standard of care for evaluation of indeterminate liver masses," said Dr. [consultant360.com]
A hepatic hemangioma is a liver mass made of widened (dilated) blood vessels. It is not cancerous. A hepatic hemangioma is the most common type of liver mass that is not caused by cancer. It may be a birth defect. [nlm.nih.gov]
- Hepatomegaly
Physical examination reveals hepatomegaly or arterial bruit on auscultation at the level of the right lobe. Presence of a large abdominal mass is rare. [symptoma.com]
In larger lesions, different degrees of hepatomegaly may be found. Generally, when there are specific symptoms, chronic or acute abdominal pain is observed. [scielo.br]
Physical examination revealed soft hepatomegaly with a smooth surface. Laboratory studies including liver function tests and serum alpha-fetoprotein levels were normal. [ijri.org]
Examination is usually normal but there may be hepatomegaly and/or an arterial bruit over the right upper quadrant. [patient.info]
Cardiovascular
- Heart Failure
Although the effect of the treatment was late, the patient is free from heart failure despite a remnant arteriovenous shunt 12 years after treatment. [ncbi.nlm.nih.gov]
Spontaneous rupture can occur, causing life threatening complications such as hemorrhage and heart failure. [symptoma.com]
Surgery can be done even if the child has heart failure. Pregnancy and estrogen-based medicines can cause these tumors to grow. [nlm.nih.gov]
- Thrombosis
This is a much lower incidence than that of native hepatocellular carcinoma (neoplastic thrombosis, 6.5%-44%; bland thrombosis, 42%) [ 10 ], and could show that sorafenib actually reduces the risk of development of portal vein thrombosis. [e-ultrasonography.org]
RELATIVE CONTRAINDICATIONS Anatomic abnormalities of IVC, HV si PV; Recent thrombosis of PV; H ypervascular hepatic tumors; Liver policystic disease 6. [slideshare.net]
subcapsular or deep Tumors collapse when sectioned as blood oozes out Microscopic (histologic) description Variably sized vascular spaces lined by flat endothelial cells and myxoid or fibrous stroma Large fibrous septa may trap bile ducts Variable thrombosis [pathologyoutlines.com]
[…] fatty infiltration Pedunculated hepatic haemangioma Cystic hepatic haemangioma – rare Fluid-fluid level containing hepatic haemangioma – rare This large, atypical haemangioma of the liver may present with abdominal pain or fullness due to haemorrhage, thrombosis [en.wikipedia.org]
[…] enlarge during pregnancy or with estrogen administration Clinical findings Usually asymptomatic and discovered incidentally Large lesions may cause pain, nausea, or vomiting secondary to extrinsic compression of adjacent bowel, rupture, hemorrhage, or thrombosis [learningradiology.com]
Skin
- Angiomatosis
Angiomatosis Angiomatosis is defined as diffuse infiltration of bone or soft tissue by hemangiomatous or lymphangiomatous lesions (, 30 ). [doi.org]
Workup
The following methodologies are currently used in the diagnosis of hepatic angiomas: ultrasound, CT Scan, nuclear medicine procedures, MRI, hepatic arteriography, and digital subtraction angiography.
Ultrasonography
Ultrasound is useful as a relatively inexpensive tool for screening liver hemangiomas, which are echogenic. However, more precise instrumentation is needed for differential diagnosis, such as those used by nuclear medicine studies.
Computed tomography
Dynamic contrast-enhanced CT scanning is more accurate than routine CT scanning, particularly when there is a specific need for a non-enhanced, portal venous, arterial, and delayed imaging, or triple phase CT with delayed imaging.
Magnetic resonance imaging
MRI, although highly sensitive for the detection of liver hemangioma, requires the use of gadolinium as an intravenous contract agent to improve imaging. However, hemangiomas of less than 2 cm may be difficult to distinguish from hypervascular metastasis or hepatocellular carcinoma in late arterial-phase imaging [11].
Nuclear medicine studies
Planar scintigraphic studies using Tc-99m pertechnetate-labeled red blood cells have been reported to have 82% sensitivity and 100% specificity for hemangiomas greater than 2 cm in diameter [12].
Single-photon emission computerized tomography (SPECT) using the same reagent is an alternative to planar imaging. These modalities help validate the results of CT scan and MRI.
Arteriography
The procedure is invasive but serves as an alternative to other diagnostic modalities for certain types of liver hemangiomas.
Imaging of hemangiomas less than 2 cm
The methods of choice for the diagnosis of lesions less than 2 cm are MRI and 99mTc-RBC SPECT.
Ultrasound
- Enlargement of the Liver
However, it is also unknown what causes the possible enlargement of the liver hemangioma in size. [humanitas.net]
Polycystic liver disease rarely causes liver failure, despite the presence of hundreds of cysts. The most common symptoms are enlargement of the abdomen and pain, which sometimes require treatment. [uhnj.org]
Treatment
Hemangiomas which do not grow or cause symptoms, or impinge on adjacent organs, may not require treatment. Large or multiple lesions that cause health problems necessitate medical intervention [13].
For hemangioma that grows and with an ample supply of blood flowing to it, a surgical procedure, called liver artery ligation is performed. The main artery leading to the hemangioma is tied off, leaving the rest of the liver intact, receiving blood from other arteries.
Surgical removal and replacement with a liver transplant may be needed where large or multiple tumors are present that do not respond to other treatment procedures. This option is more of a medical emergency when the surgeon foresees that spontaneous rupture of the angioma is imminent.
Radiation treatments may be prescribed to reduce the size of the tumor or surgical resection for patients with hemangioma greater than 5 cm, with concomitant abdominal pain. In practice, surgical resection and surgical enucleation have been the treatments of choice [14]. An alternative method is arterial embolization. A medication is injected into the hemangioma to block off the blood supply [15].
Management of large hemangiomas
Minimally invasive modalities include radiofrequency ablation, arterial embolization, irradiation and on rare occasions, orthoptopic transplantation.
The principal consideration for either surgery or invasive therapy is alleviation of symptoms but especially in the case of possible spontaneous rupture of the hemangioma. However, high mortality rates have been reported with surgical resection of ruptured hemangioma.
The patient with a ruptured liver hemangioma should be initially managed for hemodynamic stabilization before performing any invasive procedure. Having done so, surgical resection of the liver hemangioma may then proceed [15]. Other recommended treatments are surgical ligation of the hepatic artery or arterial embolization.
Physicians vary in approach to the management of a large (i.e., >10 cm wide) liver hemangioma. In general, large symptomatic hemangiomas require medical intervention. The choice of treatment modality depends on the physical and clinical evaluation of the patient, the results of diagnostic tests, and risks involved. Surgeons opt for resection than risk spontaneous rupture and complication such as, congestive heart failure or intratumoral hemorrhage.
Prognosis
Liver hemangiomas are usually benign posing no particular health problem for most people. On a rare occasion when a liver transplant becomes a medical necessity, the patient needs to be on medication for life to prevent liver transplant rejection under close monitoring by the physician.
Etiology
The precise etiology of liver hemangioma is not known but it is presumed to be congenital in origin. It occurs as a single mass (bolus) of tangled blood vessels, measuring about 4 centimeters or 1.5 inches wide, some may be larger; multiple hemangiomas are rare.
Liver hemangiomas usually do not grow nor cause any signs and symptoms in most affected individuals. There have been cases where the hemangioma has grown, becoming a health problem that needed to be treated.
Epidemiology
The estimated incidence rate of liver hemangiomas is 2%. Cases seen at autopsy are reported at 7.4%. The true incidence of liver hemangiomas is probably higher with the finding of asymptomatic lesions in patients through noninvasive imaging technics.
Liver hemangioma has been diagnosed in fetus in utero [3], as well as in infants. People of all ages are affected. Women (especially multipara, younger women, with large tumors) outnumber men, with a female:male ratio of 4-6:1. The peak age when most cases occur is 30-50 years.
Pathophysiology
There is yet no conclusive evidence that liver hemangioma is hereditary. One particular study in an Italian family found large hemangiomas in three women representing three successive generations. The presumption is that it is either due to a sex-linked genetic transmission or to some predisposing factor relating to female sex hormones.
Pharmacologic practices such as estrogen therapy and steroid therapy [4], and ovarian stimulation in pregnancy can promote tumor growth. A prospective study of 94 women with liver hemangiomas was conducted with a mean follow-up observation of 7.3 years (ranging 1-17 years) [5]. Tumor growth occurred in 23% of women with hormonal therapy versus 10% of control subjects. Spitzer et. al., in 1997 reported hemangioma in a pregnant woman after treatment with human chorionic gonadotropin and clomiphene citrate [6].
Prevention
Preventive measures can not be anticipated since there is no succinct evidence of a hereditary etiology and the disorder is congenital. Likewise, apprehension is unnecessary since liver hemangioma is usually benign and cause no particular signs or symptoms. Large and multiple tumors are uncommon but should they occur, diagnostic and therapeutic interventions are available.
Summary
Angioma of the liver is a benign cavernous growth, with an ample supply of arterial blood as it is in other liver tumors. The lesions are mostly found on the periphery of the liver [1]. Liver hemangioma is presumed to be congenital in origin. Meanwhile, evidence for a hereditary mode of transmission is lacking. As it were, the disorder has been diagnosed in people of all ages, but more in women than among men. Angiomas are usually small, occur singly and remain undetected. Most affected persons are asymptomatic. However, there are cases where the tumor grows, causing complications from an exacerbated disease. At its worst, spontaneous rupture of an enlarged angioma is an immediate consideration of the clinician.
Initially, diagnosis is based on thorough physical and clinical evaluation of the patient. There are two sub types, namely:
- Typical liver hemangioma and
- Atypical liver hemangioma [2].
Atypical liver hemangiomas are further classified into: Fast filling liver hemangioma, representing up to 16% of all liver hemangiomas; Slow fill liver hemangioma, constituting 8-16% of all hemangiomas; Giant liver hemangioma; Hyalinised or sclerosed liver hemangioma; and calcified liver hemangioma.
Other unusual imaging patterns are:
- Pedunculated liver hemangioma
- Liver hemagioma with fatty infiltration
- Liver hemangioma with capsular retraction
- Liver hemangioma with surrounding regional nodular hyperplasia
- Cystic liver hemangioma: rare
- Fluid-fluid level containing liver hemangioma: rare
A number of non-invasive imaging technologies are currently available to determine the precise nature and extent of the disease. Likewise, several options are available in terms of treatment, from the so-called minimally invasive modalities to surgical removal and replacement (liver transplant).
Patient Information
Seven percent (7%) of apparently healthy persons may harbor angioma of the liver without any sign or symptom. The female:male ratio among positive cases is 4-6:1. Female hormones have been linked to the development of hemangiomas, but which are not known to be carcinogenic. Tumors in women are usually larger than those that occur in men. Angiomas are not necessarily confined to the liver, these may occur in other parts of the body.
Causes
Liver hemangioma is essentially a congenital defect of unknown etiology.
These tumors have been found in people of all ages, but mostly in adults aged 30 to 50 years old.
Tumors in babies are called benign infantile hemangioendothelioma or multinodular liver hemangiomatosis. The disorder becomes clinically manifested in infants at 6 months of age. Though rare and non carcinogenic, these have been linked to heart failure and high mortality rates in infants.
Symptoms
Hemangiomas are mostly benign, small, less than an inch wide and clinically insignificant. Some may grow, become large and cause health problems necessitating appropriate intervention. Most angiomas become evident only during routine medical examination for some other purpose or at autopsy. Initial screening is by abdominal ultrasound imaging or CT (computerized tomography) scanning. Large hemangiomas can impinge on adjacent organs, causing symptoms, such as pain, feeling of fullness and nausea. Spontaneous rupture can occur, causing life threatening complications such as hemorrhage and heart failure.
Diagnosis
A strong index of suspicion of liver hemangioma warrants the use of reliable diagnostic tools to rule out malignancy or some other type of tumor. This is likewise important in assuring the patient of the nature and extent of the disorder. An array of confirmatory tests are available with varying levels of sensitivity and specificity to establish the diagnosis of hemangioma. These are: ultrasonography, CT scan, MRI, and scintigraphy, Biopsy is to be avoided because of the possible risk of hemorrhage.
Treatment
Most liver hemangiomas do not necessitate treatment. For patients with large or multiple tumors, causing symptoms, a number of minimally invasive procedures are available depending upon the discretion of the physician and patient's consent. Surgical intervention and/or replacement with liver transplant are options only after the benefits and risks involved are properly assessed.
References
- Vilanova JC, Barceló J, Smirniotopoulos JG, et al. Hemangioma from head to toe: MR imaging with pathologic correlation. Radiographics. 2004 Mar-Apr; 24(2):367-85.
- Vilgrain V, Boulos L, Vullierme MP, et-al. Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics. 2000; 20(2):379-97.
- Dreyfus M, Baldauf JJ, Dadoun K, et al. Prenatal diagnosis of hepatic hemangioma. Fetal Diagn Ther. 1996 Jan-Feb; 11(1):57-60.
- Takahashi T, Kuwao S, Katagiri H, et al. Multiple liver hemangiomas enlargement during long-term steroid therapy for myasthenia gravis. Dig Dis Sci. 1998 Jul; 43(7):1553-61.
- Glinkova V, Shevah O, Boaz M, et al. Hepatic haemangiomas: possible association with female sex hormones. Gut. 2004 Sep; 53(9):1352-5.
- Spitzer D, Krainz R, Graf AH, et al. Pregnancy after ovarian stimulation and intrauterine insemination in a woman with cavernous macrohemangioma of the liver. A case report. J Reprod Med. 1997 Dec; 42(12):809-12.
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- Jain V, Ramachandran V, Garg R, Pal S, et al. Spontaneous rupture of a giant hepatic hemangioma - sequential management with transcatheter arterial embolization and resection. Saudi J Gastroenterol. 2010 Apr-Jun; 16(2):116-.