Hepatic hemangioma overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hepatic Hemangioma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

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Echocardiography or Ultrasound

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

Overview

Hepatic hemangioma is a noncancerous liver tumor made of dilated (widened) blood vessels and is the most common primary liver tumor. The case of spontaneous rupture of a hepatic hemangioma was first described by Van Haefen in 1898.[1] [2] Development of hepatic hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.[3] Hepatic hemangioma may be associated with Kasabach-Merritt syndrome, hereditary hemorrhagic telangiectasia, hepatic arterio-portal shunts, extra hepatic hemangiomata, hemolytic anemia, focal nodular hyperplasia.[4] On gross pathology, variable in size, well circumscribed, classically subcapsular are characteristic findings of hepatic hemangioma.[5] There are no established causes for hepatic hemangioma.[1] The prevalence of hepatic hemangioma is up to 7% in normal adult population.[6] There are no known risk factors for hepatic hemangioma.[7] According to the United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hepatic hemangioma.[8] Hepatic hemangioma must be differentiated from other diseases such as hepatic metastases, hepatocellular carcinoma, hepatic cyst.[9] If left untreated, patients with giant hepatic hemangiomas may progress to develop complications. Pregnancy and estrogen-based medications can cause cavernous hemangiomas to grow. Symptoms of hepatic hemangioma include intermittent right upper quadrant pain, biliary colic, obstructive jaundice.[10] They are frequently asymptomatic and incidentally discovered at imaging, surgery, or autopsy. Hepatic hemangioma is usually not discovered until medical pictures are taken of the liver for some other reason. If a cavernous hemangioma ruptures, the only sign may be an enlarged liver. Some patients with hepatic hemangioma may have elevated concentration of transaminases, bilirubin and alkaline phosphatase even in asymptomatic cases.[10] Patients with small hemangiomas (less than 4 cm) are managed by observation, whereas asymptomatic patients are followed up with periodic radiological examination.[11]Elective surgical resection is recommended for all patients who develop progressive abdominal pain and a size of more than 5cm.[12]

Historical perspective

The case of spontaneous rupture of a hepatic hemangioma was first described by Van Haefen in 1898.[1]

Classification

Hepatic hemangioma may be classified into typical and atypical hemangioma.[1][2]

Pathophysiology

Development of hepatic hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.[3] Hepatic hemangioma may be associated with Kasabach-Merritt syndrome, hereditary hemorrhagic telangiectasia, hepatic arterio-portal shunts, extra hepatic hemangiomata, hemolytic anemia, focal nodular hyperplasia.[4] On gross pathology, variable in size, well circumscribed, classically subcapsular are characteristic findings of hepatic hemangioma.[5] On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are characteristic findings of hepatic hemangioma.[13]

Causes

There are no established causes for hepatic hemangioma.[1]

Differentiating Hepatic hemangioma from other Diseases

Hepatic hemangioma must be differentiated from other diseases such as hepatic abscess, hepatocellular carcinoma, hepatic cyst, hemangioendothelioma.[2]

Epidemiology and Demographics

The prevalence of hepatic hemangioma is estimated to be upto 20% in general population.[14] Hepatic hemangioma commonly affects individuals between 30 to 50 years of age.[14] Females are more commonly affected with hepatic hemangioma than males. The female to male ratio is 3:1.[14]

Risk factors

There are no established risk factors for hepatic hemangioma.[7]

Screening

According to the American Association for the Study of Liver Diseases and United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hepatic hemangioma.[8][15]

Natural History, Complications and Prognosis

If left untreated, patients with giant hepatic hemangiomas may progress to develop complications. Complications of hepatic hemangioma include spontaneous rupture, acute hemorrhagic shock, upper abdominal pain.[16][17]

Diagnosis

History and symptoms

Symptoms of hepatic hemangioma include intermittent right upper quadrant pain, dyspepsia, early satiety, vomiting.[10][7]

Physical Examination

Common physical examination findings of hepatic hemangioma include palpable upper abdominal mass, hepatomegaly, biliary colic.[7][18]

Laboratory Findings

Some patients with hepatic hemangioma may have elevated concentration of transaminases, bilirubin and alkaline phosphatase even in asymptomatic cases.[10]

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical therapy

Surgery

Prevention

Patients with small hemangiomas (less than 4 cm) are managed by observation, whereas asymptomatic patients are followed up with periodic radiological examination.[11]Elective surgical resection is recommended for all patients who develop progressive abdominal pain and a size of more than 5cm.[12]

References

  1. 1.0 1.1 1.2 1.3 1.4 Jr, Marcelo AF Ribeiro (2010). "Spontaneous rupture of hepatic hemangiomas: A review of the literature". World Journal of Hepatology. 2 (12): 428. doi:10.4254/wjh.v2.i12.428. ISSN 1948-5182.
  2. 2.0 2.1 2.2 Subtypes of hepatic hemangioma. Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatic-haemangioma-3. Accessed on October 26, 2015
  3. 3.0 3.1 Papafragkakis, Haris; Moehlen, Martin; Garcia-Buitrago, Monica T.; Madrazo, Beatrice; Island, Eddie; Martin, Paul (2011). "A Case of a Ruptured Sclerosing Liver Hemangioma". International Journal of Hepatology. 2011: 1–5. doi:10.4061/2011/942360. ISSN 2090-3456.
  4. 4.0 4.1 Associations of hepatic hemangioma. Dr Yuranga Weerakkody et al. Radiopaedia (2015). http://radiopaedia.org/articles/hepatic-haemangioma-3. Accessed on November 7, 2015
  5. 5.0 5.1 Gross pathology of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015
  6. Heiken, Jay P. (2007). "Distinguishing benign from malignant liver tumours". Cancer Imaging. 7 (Special Issue A): S1–S14. doi:10.1102/1470-7330.2007.9084. ISSN 1470-7330.
  7. 7.0 7.1 7.2 7.3 Marrero, Jorge A; Ahn, Joseph; Rajender Reddy, K (2014). "ACG Clinical Guideline: The Diagnosis and Management of Focal Liver Lesions". The American Journal of Gastroenterology. 109 (9): 1328–1347. doi:10.1038/ajg.2014.213. ISSN 0002-9270.
  8. 8.0 8.1 Hepatic hemangioma. USPSTF. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=hepatic+hemangioma
  9. Radiopaedia 2015 Hepatic hemangioma "Radiopedia 2015 Hepatic hemangioma [Dr Yuranga Weerakkody]".
  10. 10.0 10.1 10.2 10.3 Jr MA, Papaiordanou F, Gonçalves JM, Chaib E (2010). "Spontaneous rupture of hepatic hemangiomas: A review of the literature". World J Hepatol. 2 (12): 428–33. doi:10.4254/wjh.v2.i12.428. PMC 3010512. PMID 21191518.
  11. 11.0 11.1 Jones BE, Moore RY (1977). "Ascending projections of the locus coeruleus in the rat. II. Autoradiographic study". Brain Res. 127 (1): 25–53. PMID http://www.ncbi.nlm.nih.gov/pmc/articles/PMC301051 Check |pmid= value (help).
  12. 12.0 12.1 Erdogan D, Busch OR, van Delden OM, Bennink RJ, ten Kate FJ, Gouma DJ; et al. (2007). "Management of liver hemangiomas according to size and symptoms". J Gastroenterol Hepatol. 22 (11): 1953–8. doi:10.1111/j.1440-1746.2006.04794.x. PMID 17914976.
  13. Microscopic features of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015
  14. 14.0 14.1 14.2 "Adult hepatic hemangioma: an updated review with focus on the natural course and treatment options". Abdomen. 2015. doi:10.14800/abdomen.908. ISSN 2378-1351.
  15. Hepatic hemangioma. AASLD. https://www.aasld.org/search/node/hepatic%20hemangioma
  16. Assy, Nimer (2009). "Characteristics of common solid liver lesions and recommendations for diagnostic workup". World Journal of Gastroenterology. 15 (26): 3217. doi:10.3748/wjg.15.3217. ISSN 1007-9327.
  17. Ehrl, Denis; Rothaug, Katharina; Herzog, Peter; Hofer, Bernhard; Rau, Horst-Günter (2012). ""Incidentaloma" of the Liver: Management of a Diagnostic and Therapeutic Dilemma". HPB Surgery. 2012: 1–14. doi:10.1155/2012/891787. ISSN 0894-8569.
  18. "Adult hepatic hemangioma: an updated review with focus on the natural course and treatment options". Abdomen. 2015. doi:10.14800/abdomen.908. ISSN 2378-1351.