Hemangioma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Hemangioma was first described by Dr. Robert Liston, a British surgeon, in 1843.[1] Hemangioma may be classified according to International Society for the Study of Vascular Anomalies into six subtypes: Infantile hemangioma, congenital hemangioma, tufted angioma, spindle-cell hemangioma, epithelioid hemangioma, and lobular capillary hemangioma.[2][3] Development of hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.[4] Hemangioma may be associated with POEMS syndrome and Castleman disease. On gross pathology, spongy with vascular compartments of various sizes separated by fibrous tissue are findings of hemangioma.[4] On microscopic histopathological analysis, channels lined by benign endothelium containing red blood cells are findings of hemangioma.[5] There are no established causes for hemangioma.[6] Hemangioma must be differentiated from other diseases such as: Congenital hemangioma, kaposiform hemangioendothelioma, tufted angioma, and nevus flammeus, and pyogenic granuloma.[7] The prevalence of infantile hemangioma is estimated to be up to 10% in general population.[3] Hemangioma commonly affects infants.[6] Females are more commonly affected with hemangioma than males.[6] Common risk factors in the development of hemangioma are female gender, prematurity, low birth weight, and fair skin.[3] According to the United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hemangioma.[8] If left untreated, 20% of patients with hemangioma may progress to develop ulceration, hemorrhage, infection, and high output cardiac failure.[9] Common complications of hemangioma include ischemia, necrosis, ulceration, and bleeding.[3] Prognosis is generally good. Physical examination findings of superficial hemangioma include well-demarcated, flat, and erythematous red patches.[3] The majority of cases of hemangioma are self-limited. Patients with small, stable hemangiomas in non-vital sites are treated with "wait and see" approach, whereas patients with fast growth of hemangioma are treated medically.[6] Surgery is not the first-line treatment option for patients with hemangioma. It is usually reserved for patients with either massive protuberant proliferating hemangioma and lesions that are refractory to less invasive treatments.[7][3]

Historical Perspective

Hemangioma was first described by Dr. Robert Liston, a British surgeon, in 1843.[1]

Classification

Hemangioma may be classified according to International Society for the Study of Vascular Anomalies into six subtypes: Infantile hemangioma, congenital hemangioma, tufted angioma, spindle-cell hemangioma, epithelioid hemangioma, and lobular capillary hemangioma.[2][3]

Pathophysiology

Development of hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.[4] Hemangioma may be associated with POEMS syndrome and Castleman disease. On gross pathology, spongy with vascular compartments of various sizes separated by fibrous tissue are findings of hemangioma.[4] On microscopic histopathological analysis, channels lined by benign endothelium containing red blood cells are findings of hemangioma.[5]

Causes

There are no established causes for hemangioma.[6]

Differentiating Hemangioma from other Diseases

Hemangioma must be differentiated from other diseases such as: Congenital hemangioma, kaposiform hemangioendothelioma, tufted angioma, and nevus flammeus, and pyogenic granuloma.[7]

Epidemiology and Demographics

The prevalence of infantile hemangioma is estimated to be up to 10% in general population.[3] Hemangioma commonly affects infants.[6] Females are more commonly affected with hemangioma than males.[6]

Risk Factors

Common risk factors in the development of hemangioma are female gender, prematurity, low birth weight, and fair skin.[3]

Screening

According to the United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hemangioma.[8]

Natural History, Complications and Prognosis

If left untreated, 20% of patients with hemangioma may progress to develop ulceration, hemorrhage, infection, and high output cardiac failure.[9] Common complications of hemangioma include ischemia, necrosis, ulceration, and bleeding.[3] Prognosis is generally good.

Diagnosis

Staging

There is no established system for the staging of hemangioma.

History and symptoms

Symptoms of hemangioma include: flat, and erythematous red patches.[3]

Physical Examination

Physical examination findings of superficial hemangioma include well-demarcated, flat, and erythematous red patches.[3]

Laboratory Findings

There are no diagnostic lab findings associated with hemangioma.

CT

On CT scan, hemangioma of the liver is characterized by dynamic enhancement pattern related to the size of its vascular space.[2]

MRI

Ultrasound

Ultrasound may be helpful in the diagnosis of hemangioma. Findings on ultrasound suggestive of hemangioma include fat, phleboliths or prominent vascular channels.[10]

Treatment

Medical therapy

The majority of cases of hemangioma are self-limited. Patients with small, stable hemangiomas in non-vital sites are treated with "wait and see" approach, whereas patients with fast growth of hemangioma are treated medically.[6]

Surgery

Surgery is not the first-line treatment option for patients with hemangioma. It is usually reserved for patients with either massive protuberant proliferating hemangioma and lesions that are refractory to less invasive treatments.[7][3]

Primary Prevention

There are no primary or secondary preventive measures available for hemangioma.

References

  1. 1.0 1.1 Liston R. Case of erectile tumour in the popliteal space.-Removal. Med Chir Trans. 1843;26:120-32.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2116921/pdf/medcht00056-0152.pdf
  2. 2.0 2.1 2.2 Hemangioma. Dr Tim Luijkx and Dr Donna D'Souza et al. Radiopaedia (2015). http://radiopaedia.org/articles/haemangioma. Accessed on November 12, 2015
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 Richter, Gresham T.; Friedman, Adva B. (2012). "Hemangiomas and Vascular Malformations: Current Theory and Management". International Journal of Pediatrics. 2012: 1–10. doi:10.1155/2012/645678. ISSN 1687-9740.
  4. 4.0 4.1 4.2 4.3 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.
  5. 5.0 5.1 Microscopic features of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015
  6. 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 Zheng JW, Zhang L, Zhou Q, et al. A practical guide to treatment of infantile hemangiomas of the head and neck. Int J Clin Exp Med. 2013;6(10):851-60.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3832322/?report=classic#
  7. 7.0 7.1 7.2 7.3 Callahan, Alison B.; Yoon, Michael K. (2012). "Infantile hemangiomas: A review". Saudi Journal of Ophthalmology. 26 (3): 283–291. doi:10.1016/j.sjopt.2012.05.004. ISSN 1319-4534.
  8. 8.0 8.1 Hemangioma. USPSTF. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=hemangioma Accessed on November 10, 2015
  9. 9.0 9.1 Hassan, Basheir A.; Shreef, Khalid S. (2014). "Propranolol in Treatment of Huge and Complicated Infantile Hemangiomas in Egyptian Children". Dermatology Research and Practice. 2014: 1–5. doi:10.1155/2014/541810. ISSN 1687-6105.
  10. Ultrasound of Facial hemangioma. Dr Sinéad Culleton. Radiopaedia (2015). http://radiopaedia.org/cases/facial-haemangioma. Accessed on November 17, 2015

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