Hemangioma – Types, Causes and Treatment

Last Updated on January 27, 2024

Hemangioma is a benign vascular tumor and one of the most common soft tissue tumors. It is the most common tumor of infancy and childhood.

Note* -Vascular anomalies are broadly classified by the International Society for the study group into vascular tumors and vascular malformations.

Most of the hemangiomas are superficial in the head and neck region but they can occur anywhere. The liver is another common site.

These show endothelial hyperplasia on microscopy. This is in contrast to vascular malformations where the endothelium is normal but the flow is abnormal.

Hemangiomas are formed due to abnormalities in the formation of blood vessels [angiogenesis]  that allow overproliferation. An increase in factors responsible for blood vessel formation such as [fibroblast growth factor [FGF], vascular endothelial growth factor [VEGF] etc.) are increased and theorized to be responsible but a clear cause is not established yet.

The head and neck region is the most common location. About 60% of them are found in this region. This is followed by the trunk and limbs. Lower limbs are more commonly involved than the upper.

Usually, the lesions are small but occasionally cover a large area.

Deep lesions, depending on their locations can cause severe problems like effects on vision, speech, and hearing.

Visceral lesions are found to occur in the liver, intestines, airway, and brain.

In some cases, these can be life-threatening.

Sometimes, these can be part of a syndrome too. Generally speaking, hemangiomas are more common in females.

Types of Hemangioma

Hemangioma are part of the classification of vascular tumors, under benign vascular tumors. Part of that classification is listed below [based on the International Society for the Study of Vascular Anomalies (ISSVA) classification 2018]

Benign vascular tumors

• Infantile hemangioma
• Congenital hemangioma
  • Rapidly involuting (RICH)
  • Noninvoluting (NICH)
  • Partially involuting (PICH)
• Tufted angioma
• Spindle-cell angioma
• Epithelioid angioma
• Pyogenic granuloma (Lobular capillary hemangioma)
• Others

These are discusseed below one by one.

Infantile Hemangioma

These are the most common benign tumors of infancy. Strawberry marks is the colloquial term given to them due to their appearance clinically.

These lesions are the most common tumors of infancy and are marked by early growth and followed by spontaneous regression.

These may be found in the skin or other organs.

Cutaneous lesions are seen mainly in

• Head and neck – most common, about 60 % of skin lesions
• Trunk
• Extremities

Other sites of occurrence are

• Liver
• GI tract
• Larynx
• Central nervous system
• Lymphatic system – Thymus, spleen, lymph nod
• Lung
• Urinary bladder

Maximum lesions are small, less than 1 cm. However, the size can range from a pinhead to 20 cm.

They show rapid growth in the neonatal period. Quite a lot of them appear in this period in contrast to the congenital type where the child is born with the lesion.

The growth can continue up to one year of life.

However, 50% of the lesions shrink and fade by 5 years. Others may take more time.

The diagnosis is usually evident on clinical examination but in doubtful cases, a biopsy can be done.

On immunohistochemically, they express the Glut-1 marker that can be used to confirm the diagnosis

MRI can locate and tell the extent of deep hemangiomas.

As the lesions regress mostly, the majority do not need any active treatment, just observation.

As and when required propranolol and oral corticosteroids can help with hemangiomas which cause disfigurement or are affecting function.

Laser surgery can be considered for those lesions that do not respond to drugs or in cases where the drugs cannot be given for some reason.

Surgical removal is indicated where the function such as vision is threatened [ocular hemangioma]. In such cases, early timely surgery may help to save the vision.

Early removal may also be considered in cases of significant disfigurement.

Congenital hemangioma

Congenital hemangiomas are lesions that are fully formed at birth. Unlike the infantile, they are grown to the maximum achievable size at birth and do not show any further fast growth.

Like the infantile, these occur mainly in the skin but can occur in other organs too.

There does not seem to be a gender predilection.

Congenital hemangiomas are of three subtypes

• Rapidly involuting congenital hemangioma (RICH)
  • Regresses within the first year of life
  • By 1 to 1.5 years of age, the lesion is almost completely gone
  • A loose scar tissue may be left behind.
• Noninvoluting congenital hemangioma (NICH)
  • These do not reduce in size after the baby is born
• Partially involuting congenital hemangioma (PICH)
  • This involute incompletely.
  • These involute in the first 1-2 years before stabilizing and then remaining as such.

Just by looking at the lesion at birth, it is difficult to say what is the subtype. It is only the behavior afterward

RICH and NICH demonstrate overlapping features.

Congenital lesions can be detected in utero by antenatal ultrasound.

All congenital hemangiomas are negative for glucose transporter-1 (GLUT1) [a feature that differentiates from the infantile which is positive]

RICH lesions should be observed as no other treatment is required except in case of complications. These could be

• Thrombocytopenia
• Heart failure
• Anemia

Treatment for NICH and PICH is considered when the lesion does not regress after 18 months of age and  would be dictated by their size and location.

Drugs are not effective.

In all cases where treatment is required, the  following are the available options

• Embolization
• Surgery

Tufted Angioma

Tuft literally means a bunch. These lesions are called so because these benign tumors are characterized by tightly packed capillary lobules. These are found in the skin and subcutaneous tissue.

These are rare tumors and occur in infants and children though adult-onset cases are known as well.

The lesion is suspected in an infant or young child red/ violet vascular lesion with ill-defined borders on the trunk or extremities..These are associated with pain, tenderness, and increased sweating [hyperhidrosis]. Usually, these are slow growers and expand over a few months to years but occasionally may expand rapidly.

A biopsy can confirm the diagnosis.

For deep-seated angiomas, MRI is able to evaluate the depth of invasion and extent of the tumor.

Kasabach-Merritt syndrome [severely low platelets and coagulopathy] has been seen to occur in ten percent of these lesions.

The treatment of tufted angioma depends on the extent of the tumor,  tumor size, and the presence or absence of Kasabach-Merritt syndrome.

For small lesions without symptoms, observation only is warranted.

In the case of Kasabach-Meritt syndrome aggressive medical management to address the critical issues should be followed.

Various treatment approaches are

• Surgical excision,
• Pulsed-dye laser for superficial lesions
• Drugs
  • Oral propranolol
    • followed by foam sclerotherapy with  sodium tetradecyl sulfate
    • Topical tacrolimus

Spindle-cell Angioma

Spindle cell hemangiomas are rare tumors composed of a mixture of cavernous blood vessels and Kaposi’s sarcoma-like spindle cells. These tumors occur in children and young adults. Association with other diseases has been reported. These are namely

• Maffucci syndrome
• Klippel-Trénaunay syndrome
• Venous malformations
• Congenital lymphedema

These lesions typically involve distal extremities but occur in the proximal part of the limbs, trunk, head, and neck region. Generally, the lesion is smaller than 2 cm but can vary from a few millimeters to 10 cm. The oral cavity is another rare location.

Histologically, they show dilated blood vessels and accumulated spindle cells. Thrombi or phleboliths may be found.

The exact cause is not known.

Surgical removal is the best treatment. About half of them are known to recur after removal.

The following measures are associated with lesser chances of recurrence

• Post-surgery radiotherapy
• Low-dose interferon α-2b
• Recombinant interleukin 2
• Epithelioid angioma

Epithelioid Angioma

Epithelioid hemangiomas occur on the head [forehead, the preauricular region, and the scalp], and distal extremities [the digits]. Less commonly they are found in bone and deep soft tissue. The lesion is seen as a slow-growing, subcutaneous, or dermal nodule.

Microscopically, they appear as the sharply defined proliferation of capillary-like blood vessels, lined by plump epithelioid endothelial cells.

Epithleloid or histiocytoid cells are cells with abundant cytoplasm and prominent cytoplasmic vacuolization and have large, pleomorphic nuclei.

The biopsy is confirmatory and makes a definitive diagnosis.

The cause of the lesion is unknown.

The treatment options are

• Complete removal surgically
• Chemoembolization
• Microwave ablation for bony lesions

Pyogenic granuloma (Lobular capillary hemangioma)

Pyogenic granulomas or granuloma pyogenicum are benign small, raised, and red bumps on the skin that often appear following an injury on the hands, arms, or face.

These occur on hands, face, and arms and are known to bleed easily.

The lesions are actually misnamed. They are neither infections nor they are granulomatous. Therefore, lobular capillary hemangioma is a better usage.

Pregnancy tumor is the name given to lesions that occur on the gums or oral mucosa in pregnancy.

In addition to the skin and oral cavity, the pyogenic granuloma is reported to be present in

• Gastrointestinal tract
• Airway
• Eye
• Central nervous system
• Bladder

The lesion appears as a solitary, red, papule that is often pedunculated [has a stem] or less commonly sessile [no stem]. It is very friable and bleeds easily. Sometimes more lesions may develop around [satellite lesions]

It may show fast growth and ulceration.

The exact cause is not unknown. Hormonal changes and medications have been theorized as probable causes.

Disseminated pyogenic granulomas are quite rare but have been reported with burns.

Medical treatment is not effective

Where feasible, complete excision is the treatment of choice.

Shave excision or curettage &  electrocautery can be considered for areas with cosmetic concerns. However, the rate of recurrence could be high.

Ulceration, hemorrhage, and infections are possible complications.

References

• ISSVA Classification of Vascular Anomalies. 2018 International Society for the Study of Vascular Anomalies Available at “issva.org/classification” Accessed 14/09/2023.
• Dubois J, Patriquin HB, Garel L, et al. Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography. AJR Am J Roentgenol. 1998 Jul. 171(1):247-52.
• Bruckner AL , Frieden IJ . Hemangiomas of infancy. J Am Acad Dermatol 2003;48:477–96[Link]
• Papke DJ Jr, Jagannathan J, Dong F, Dickson BC, Mertens F, Hornick JL, Fletcher CDM. Epithelioid and Spindle Cell Hemangioma: Clinicopathologic Analysis of 18 Primary Bone and Soft Tissue Tumors Highlighting a Predilection for the Hands and Feet, Frequent Multicentricity, and Benign Behavior. Am J Surg Pathol. 2023 Feb 1;47(2):147-156. doi: 10.1097/PAS.0000000000001974.