Introduction

“This looks different.” “Is this normal?”, “Is it getting bigger?” “Could this be cancer?” - these are normal, day questions that we ask ourselves when we see a sudden growth on our body that isn’t a typical spot or mole. They could be in the form of vascular anomalies, which are atypical growths from blood vessels (such as the veins, arteries, capillaries, and lymphatic vessels) that can form into birthmarks, malformations, or tumours, which could be cancerous.¹ This report focuses on two different types of vascular anomalies, lymphangioma and haemangioma (hemangioma), due to the similarities in appearance. However, treatment for both conditions differs greatly, so it is important to distinguish between them in order for the right treatment to be provided. 

Lymphangioma

Lymphangioma is a non-cancerous and uncommon vascular disease that can occur at birth or from an early age (before the age of two). The prevalence of the condition in infants is between 0.025% to 0.05%, in which both genders can equally be affected, and the race of the patient has no impact on the disease development.²

Symptoms and presentation

Lymphangioma can happen anywhere on the skin, though the head and neck are the most common sites to be affected. In addition, the chest and armpit and more rarely, the abdomen (such as the spleen, pancreas, and liver) are also affected areas. Although rare, lymphangioma can also develop in areas of the mouth, such as the lips, tongue, the soft palate, the floor of the mouth, as well as the insides of the cheek. 

Lymphangioma that is present on the skin can appear as raised, fluid-filled growths that can be grouped together or scattered and can be transparent or bleed. It can appear purple in colour and look similar to frogspawn (frog eggs that are clumped together and jelly-like). Those with lymphangioma may also experience symptoms of itchiness, pain, burning sensation, infection, and lymphatic drainage.^3,4,5

Pathophysiology

Lymphangioma can occur when there is an abnormality in the lymphatic system, which is an accumulation of tissues, vessels, and organs that are part of your immune system, to prevent infection and maintain the normal fluid balance throughout the body. It can either be congenital (obtained at birth) or acquired (developed later after birth). In congenital lymphangioma, there is a connection fault between the lymphatic channel and the main lymphatic drainage duct. In comparison, in acquired lymphangioma, it can develop from a series of circumstances such as surgery, cancer, trauma, and radiation therapy that can disrupt the normal lymphatic drainage.³

Types of lymphangioma

There are various types of lymphangioma. For example, cystic lymphangioma is more prevalent in infancy and childhood and is commonly located on the head and neck. Cystic lymphangioma can be categorised depending on its size. For instance, macrocystic lymphangioma has a diameter of more than one centimetre, whilst microcystic lymphangioma has a diameter of less than one centimetre. There is also a mixed cystic lymphangioma, along with more complex types of lymphangiomas that present themselves on various body parts, such as the bone.²

Treatment

Overall, lymphangiomas can be hard to treat regardless of whether they are deep or just on the surface (superficial). Surgery is the first line of treatment due to the increased probability of recurrence. Compared to deep lymphangiomas, superficial types have a higher surgical success rate, especially if they are small. Other treatment options are also available, such as lasers with carbon dioxide, long-pulsed Nd-YAG lasers, and electrosurgery, which can help to lessen the appearance of the growths. Other treatment options include cryotherapy (using extreme cold temperatures to destroy tissues), superficial radiotherapy and sclerotherapy (with the antibiotic doxycycline, or chemotherapy drugs such as bleomycin or pingyangmycin, ethanol or sodium tetradecyl sulfate). 

Medicines have also been trialled in patients with lymphangiomas, for example, sildenafil, propranolol, and sirolimus. Currently, there are numerous clinical trials to determine other suitable medicines to treat lymphangioma; however, surgery is generally the first choice in patients.^2,3

Haemangioma

Haemangiomas are non-cancerous vascular tumours that can occur at birth and are called infantile haemangiomas. They are common, with 4% to 5% of infants being affected with the condition. Infants who are caucasian are more affected than other races, with a higher prevalence in those assigned female at birth (AFAB) compared to those assigned male at birth (AMAB). Those born prematurely with a low birth weight or babies that have been born with insufficient oxygen are also more susceptible. Babies born to older parents are also more prone to developing haemangiomas.⁶

Symptoms and presentation

Infantile haemangiomas most commonly develop on the face and neck, but can also affect the trunk and the limbs. The condition can also impact other areas of the body, but is less common in the following regions: the genitals, lower lip, armpit, eyes, mouth, nose, nasal passage, and the ears.

Haemangiomas are small clusters of blood vessels that appear like “strawberry marks”, where superficial haemangiomas are raised and bright red in colour. In comparison to deep haemangiomas, which are bluish in colour and can appear as a plaque or tumour. They are generally asymptomatic; however, in serious cases, they can form an ulcer, bleed, and may cause blurry vision, especially if it is located near the eyes.^6,7,9

Pathophysiology

Haemangiomas develop due to endothelial cell proliferation. Endothelial cells are cells which line the blood vessels and are responsible for the development and growth of the connective tissue that makes up the wall of the blood vessels. When these cells grow in number and multiply, it can lead to tumour production. However, the reason why this occurs remains unclear. Some hypotheses suggest that there is a fault in the angiogenic factors (protein molecules that are involved in creating new blood vessels). 

The angiogenic factors that appear to play a possible role in developing haemangiomas include vascular endothelial growth factor (VEGF), basic growth factor of fibroblasts (bFGF), and matrix metalloproteinases (MMPs) 2 and 9. In addition, there is a possible genetic predisposition in those with haemangiomas, with higher incidences in multiple subsequent births, twins, or triplets.^6,7,8

Types of haemangiomas

There are two types of haemangiomas: congenital, which appear at birth and infantile, which presents itself later during infancy. They can also be deep (located in the subcutaneous fatty tissue), superficial (located in the superficial dermis), or mixed (have features in both areas). Haemangiomas can also be referred to as focal, multifocal, or segmental depending on their presentation. Focal haemangiomas are distinct and individual growths with well-defined borders, in comparison to multifocal haemangiomas that are present in different areas of the body. Segmental haemangiomas cover a larger area of the body and are plaque-like. They are more serious in nature and can increase the risk of developing complications such as an ulceration.⁹

Treatment

The majority of infantile haemangiomas can clear on their own without treatment. However, those that are more complex can be treated with beta blockers such as propranolol, which is considered the first line of treatment. Another treatment option is high doses of oral prednisolone (a corticosteroid). Timolol is another beta blocker that can be used on the skin topically to treat smaller and superficial haemangiomas. Other treatment options include: topical imiquimod (a drug that stimulates the immune system to fight infections), pingyangmycin (an antibiotic that induces cell death in the haemangiomas, reducing their size and further growth), subcutaneous injections of interferon α-2a or 2b (an antiviral drug), cryotherapy, laser therapy, and radiation therapy.^6,10

Similarities and differences

Similarities

• Both are noncancerous types of birthmarks that happen during early birth
• Both commonly occur in the neck
• Similar treatment options (propranolol, pingyangmycin, laser therapy, and cryotherapy)
• Both can be congenital and can also develop at an early age

Differences

Summary

Lymphangiomas and haemangiomas occur early at birth. However, they are two different conditions that should be treated separately. They are both non-cancerous and can either be superficial or deep. An abnormal lymphatic system is involved in the production of lymphangiomas, whereas in haemangiomas, there is a fault in cell proliferation, which causes an overproduction of new blood vessels, especially the capillaries. On the skin, lymphangiomas can appear as fluid-filled vesicles that are purple in colour, in comparison to haemangiomas, where growth can be bright red or bluish and not fluid-filled. 

Patients with lymphangiomas may also experience itchiness; however, this does not occur in those with haemangiomas. Treatment also differs between the two conditions; surgery is considered first-line in lymphangioma. In contrast, propranolol is the ideal treatment for those with haemangioma. Post treatment, lymphangiomas have the possibility of recurring in comparison to haemangiomas, which do not. It is recommended to speak to your healthcare provider if you are concerned about a growth developing on your child, as early intervention can prevent more serious complications from occurring.