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Liver Hemangioma

Liver Cavernoma

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].

Wound Infection
  • There were three postoperative complications, 1 in the enucleation group (pleural effusion), 2 in the resection group (liver abscess and wound infection). There was no surgery-related mortality in either group.[ncbi.nlm.nih.gov]
Colic
  • Abdominal colic, melena/hematemesis, and jaundice represent the pathognomonic triad for hemobilia, but clinical presentation and etiology of this entity are varying.[ncbi.nlm.nih.gov]
  • No signs of biliary channel necrosis (hemobilia, increase in serum transaminases, serum bilirubin, serum alkaline phosphatase, biliary colic) were noted. The patient was discharged on the seventh day in a satisfactory condition.[ijri.org]
Melena
  • Abdominal colic, melena/hematemesis, and jaundice represent the pathognomonic triad for hemobilia, but clinical presentation and etiology of this entity are varying.[ncbi.nlm.nih.gov]
Hepatosplenomegaly
  • Clinical examination revealed hepatosplenomegaly, palpable mass in the right lower abdomen, and signs of neurofibromatosis type 1 (Morbus von Recklinghausen). Computed tomography revealed a giant tumor in the right lobe of the liver.[ncbi.nlm.nih.gov]

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.

Thrombocytosis
  • Other relevant clinical findings are: weight loss, anemia, fever, thrombocytosis, and elevated fibrinogen and elevated erythrocyte sedimentation rate.[symptoma.com]

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.

Sex distribution
Age distribution

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:

  1. Typical liver hemangioma and
  2. 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

Article

  1. 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.
  2. Vilgrain V, Boulos L, Vullierme MP, et-al. Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics. 2000; 20(2):379-97.
  3. Dreyfus M, Baldauf JJ, Dadoun K, et al. Prenatal diagnosis of hepatic hemangioma. Fetal Diagn Ther. 1996 Jan-Feb; 11(1):57-60.
  4. 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.
  5. Glinkova V, Shevah O, Boaz M, et al. Hepatic haemangiomas: possible association with female sex hormones. Gut. 2004 Sep; 53(9):1352-5.
  6. 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.
  7. Goodman Z. Benign tumors of the liver. Okuda K, Ishak KG. Neoplasms of the liver. Tokyo: Springer-Verlag; 1987; 105-125.

  8. Mikami T, Hirata K, Oikawa I, et al. Hemobilia caused by a giant benign hemangioma of the liver: report of a case. Surg Today. 1998; 28(9):948-52.

  9. Lee CW, Chung YH, Lee GC, et al. A case of giant hemangioma of the liver presenting with fever of unknown origin. J Korean Med Sci. 1994 Apr; 9(2):200-4.

  10. Pol B, Disdier P, Le Treut YP, et al. Inflammatory process complicating giant hemangioma of the liver: report of three cases. Liver Transpl Surg. 1998 May; 4(3):204-7.
  11. Vossen JA, Buijs M, Liapi E, et al. Receiver operating characteristic analysis of diffusion-weighted magnetic resonance imaging in differentiating hepatic hemangioma from other hypervascular liver lesions. J Comput Assist Tomogr. 2008 Sep-Oct; 32(5):750-6.
  12. Kinnard MF, Alavi A, Rubin RA, et al. Nuclear imaging of solid hepatic masses. Semin Roentgenol. 1995 Oct; 30(4):375-95.
  13. Erdogan D, Busch OR, van Delden OM et-al. Management of liver hemangiomas according to size and symptoms. J Gastroenterol Hepatol. 2007; 22(11):1953-8.
  14. Belli G, D'Agostino A, Fantini C, et al. Surgical treatment of giant liver hemangiomas by enucleation using an ultrasonically activated device (USAD). Hepatogastroenterology. 2009 Jan-Feb; 56(89):236-9. 
  15. 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-.

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Last updated: 2018-06-22 08:17