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Hemangioma of Infancy

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Vein & Vascular Health
Heart & Vascular Health
+4
Contributed byKrish Tangella MD, MBASep 25, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Benign Infantile Hemangioendothelioma
  • Juvenile Hemangioma
  • Strawberry Nevus

What is Hemangioma of Infancy? (Definition/Background Information)

  • Hemangioma of Infancy is a benign proliferation of small blood vessels (capillaries) that is present either at birth (congenital onset), or occurs right after birth (perinatal onset)
  • These benign vascular proliferations usually get larger during the first year of life and slowly disappear (spontaneous regression) as the baby gets older
  • A majority of the cases (57% of them) occur in the head and neck region, 20% of the cases occur in the trunk region, and about 16% on the arms and legs
  • No treatment is usually necessary in a majority of the cases, since the tumors disappear spontaneously with time

Who gets Hemangioma of Infancy? (Age and Sex Distribution)

  • Hemangioma of Infancy occurs in both male and female, infants and children. It is more common in females than compared to males (female-male ratio 3:1)
  • Higher incidents of the tumor have been noted in premature infants
  • The tumor is common in western countries, and among the Caucasian races
  • The probable tumor incidence is 4-5% of all births in Caucasian children; though some reports have stated the incidence to be as high as 12%

What are the Risk Factors for Hemangioma of Infancy? (Predisposing Factors)

The risk factors for Hemangioma of Infancy include:

  • Premature infants
  • Family history of the tumor (an autosomal dominant inheritance in some families is noted)
  • The risk factors for Infantile Hemangiomas also include chorionic villus sampling during pregnancy (a prenatal diagnostic test) and ischemic type of pathology occurring in the placenta
  • Hemangioma of Infancy can be associated with certain specific syndromes, such as PHACES syndrome. PHACES syndrome consists of Posterior fossa malformation of the skull bone, Heart defects, Arterial abnormalities, Coarctation of aorta, Eye abnormalities, and Sternal clefting
  • The tumor, present in the lumbosacral areas (in the back), is known to be associated with spinal abnormalities, such as spinal dysraphism and tethered cord syndrome. The presence of such syndromes, increases ones risk for Hemangioma of Infancy

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hemangioma of Infancy? (Etiology)

  • The exact cause of Hemangioma of Infancy occurrence is unknown
  • Current research indicates that this tumor is caused due to a somatic mutation
  • A majority of the cases are sporadic, which means that they occur randomly and are not caused as an inherited disorder
  • Occasionally, they are seen as an autosomal dominant inheritance pattern, with the abnormality localized to the chromosome 5q region
  • Sometimes, multiple Infantile Hemangiomas have been observed. In such cases, a specific type of chromosomal translocation abnormality, called translocation (3q:4p) has been reported

What are the Signs and Symptoms of Hemangioma of Infancy?

Over one-third of the tumor cases are present at birth. The signs and symptoms of Hemangioma of Infancy include:

  • Red patch on skin that occurs either at birth, or noticed within the first 14 days after birth. The tumors usually grow very fast becoming larger and more reddish in color
  • Tumor growth usually occurs up to age 1½ years after which, the tumor slowly starts to disappear. The tumor may take almost 5-7 years, to completely regress and disappear 
  • This vascular skin tumor is usually found in the head and neck region. The next common sites are the chest, back, arms, and legs
  • Infrequently, these tumors have been noted in non-skin areas, such as liver, lung, brain, parotid gland, and the gastrointestinal tract
  • In some cases Infantile Hemangiomas can have multiple occurrences; hence, other areas of the body should also be thoroughly examined
  • Increased incidence of moles (congenital melanocytic nevi) has been reported with Infantile Hemangiomas 

A variant of Hemangioma of Infancy tumor, called Rapidly Involuting Congenital Hemangioma (RICH) is described:

In this tumor, decreased platelets (thrombocytopenia), low levels of fibrinogen in the blood, and increased fibrin degradation product (elevated FDP) are noted. RICH tumors are well-developed at the time of birth and rapidly disappear within the first 30 days of life. In contrast, the Non-Involuting Capillary Hemangioma (called NICH), grows as the child grows.

How is Hemangioma of Infancy Diagnosed?

The diagnosis of Hemangioma of Infancy is as follows:

  • A thorough physical examination and evaluation of complete medical history. A thorough physical examination will help diagnose multiple hemangiomas (some cases may be multiple) and any variant of the tumor
  • Biopsy will reveal proliferation of blood vessels
  • MRI of the tumor will show vascular areas.
  • It is important to note that Hemangioma of Infancy can be associated with other conditions, including some specific syndromes, such as PHACES syndrome. PHACES syndrome consists of Posterior fossa malformation of the skull bone, Heart defects, Arterial abnormalities, Coarctation of aorta, Eye abnormalities, and Sternal clefting
  • Hemangioma of Infancy present in the lumbosacral areas (in the back) can be associated with spinal abnormalities, such as spinal dysraphism and tethered cord syndrome
  • Hence, the healthcare provider may perform additional tests to rule out any associated conditions, when they are suspected

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hemangioma of Infancy?

Most cases of Infantile Hemangiomas do not have any life-threatening complications. However, some possible complications could be:

  • Ulceration of the tumor: This is a very common complication occurring in 1 out of 5 such tumors
  • Such infections may result in formation of scar tissue, causing cosmetic issues

How is Hemangioma of Infancy Treated?

Hemangioma of Infancy is a benign condition, which is sometimes difficult to diagnose, resulting in a misdiagnosis. The misdiagnosed cases may result in receiving inappropriate treatment. 

In a majority of the cases no specific treatment is needed, other than monitor of the tumor. With time, the tumor will become smaller and eventually may disappear, leaving behind some scar tissue.

Treatment measures for Infantile Hemangiomas include:

  • Laser techniques and cryosurgery:

A specific type of laser treatment called pulsed dye laser treatment is used to treat tumors, which have ulcerated. Ulceration of the tumor is a common complication that occurs in about 1 in 5 cases. The treatment of ulcerated Infantile Hemangioma is very important, since these tumors can significantly bleed and may become infected. Also, an infection may result in formation of scar tissues that may cause cosmetic issues.

  • Ulcerated Infantile Hemangioma can be treated with topical creams, topical antibiotics, local steroid therapy, and aggressive wound care
  • Occasionally, embolization of the tumor is also used as a treatment modality
  • It is extremely important to treat the hemangiomas around the eye (Periorbital Infantile Hemangiomas), because these tumors can affect the vision

How can Hemangioma of Infancy be Prevented?

Currently, there are no known methods to prevent Hemangioma of Infancy occurrence.

What is the Prognosis of Hemangioma of Infancy? (Outcomes/Resolutions)

  • The prognosis of Hemangioma of Infancy is excellent, since it is a benign tumor
  • In most of the cases, the tumor will disappear with time, leaving behind scar tissue

Additional and Relevant Useful Information for Hemangioma of Infancy:

Following findings are noted by a pathologist when examining a biopsy under a microscope:

  • The tumor on biopsy shows proliferation of blood vessels, which are surrounded by prominent basement membranes. Admixed within the proliferation of blood vessels are a significant number of mast cells. As the tumor regresses, less blood vessels and more intervening stroma is noted
  • During the involution process, the blood vessels may become larger, giving an appearance of arteriovenous malformation
  • Immunohistochemical staining shows the tumor to be positive for GLUT-1, Lewis Y antigen, FCGR-2 and IGF-2. The stroma surrounding the blood vessels (the basement membrane) is positive for immunohistochemical stain called Merosin. The positivity for GLUT-1 immunohistochemical stain, is characteristic of Hemangioma of Infancy
  • Other immunohistochemical stains that these tumors are positive for include: CD31, smooth muscle actin, CD54 (ICAM-I), CD15, and WT-1

The differential diagnosis of Hemangioma of Infancy includes, but is not limited to, tufted angioma, pyogenic granuloma, congenital non-progressive hemangioma, and intramuscular hemangioma

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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