Hemangioma

Synonyms

Infantile haemangioma, strawberry nevus, hemangioma of infancy (HI).

Epidemiology

Relatively frequent (4-5% of infants).

Definition

Benign vascular tumours that appear in the first weeks after birth.

Aetiology & Pathogenesis

Unknown but has been discussed.

Signs & Symptoms

Dome-shaped circumscribed bright red nodules (cutaneous haemangioma), or pink to blue soft swelling (subcutaneous haemangioma), or both (mixed type) or superficial.

Localisation

They can be located in any area of the body.

Classification

Superficial (cutaneous), deep (subcutaneous) or mixed (superficial and deep).

Laboratory & other workups

Laboratory tests are not needed.

Dermatopathology

Histology is not needed for the diagnosis. A proliferation of dermal mature vessels without inflammatory infiltrate is observed if a biopsy is performed. However, growing HI express more GLU-1 receptor by immunohistochemistry. This should prompt the clinician to consider intervention.

Course

Childhood haemangiomas are not present at birth, appear in the first weeks of life, grow during a few months and then spontaneously regress in the first or first years of life. Those which are rapidly and continuously growing need intervention.

Clinical presentation

Complications

Ulceration (infection, pain), scar with esthetic consequences, bleeding (uncommon). Depending on hemangioma location:

Eye: amblyopia

Nose: cartilage deformation

Lips: feeding difficulties

Lower jaw: associated subglottic hemangioma with possible stridor and respiratory obstruction

Segmentary facial hemangiomas and perineal/lombo-sacral hemangiomas may be associated with underlying malformations (rare); miliary hemangiomatosis may be associated with visceral hemangiomas and high output cardiac insufficiency (rare).

Diagnosis

Most cases: clinical. Diagnostic uncertainty: ultrasonography. Suspicious subglottic hemangioma: referral to ENT (ear-nose-throat) specialist. Suspicious PHACES or PELVIS syndrome: brain or medullary MRI, respectively. Glut-1 staining.

Differential diagnosis

Differential diagnoses include vascular malformations (such as: nevus flammeus), granuloma pyogenicum, angiosarcoma, other malignant tumors (rhabdomyosarcoma, infantile fibrosarcoma). Kasabach-Merritt syndrome is characterized by profound thrombocytopenia, consumptive coagulopathy with a hemorrhagic risk, in association with vascular tumors (rare).

Prevention & Therapy

In most cases: no treatment. In case of complications related to localization or rapid growth: beta-blockers (propranolol).

Special

Aggressive early treatment (laser) may be needed in fast growing lesions in anatomical risky localisations (i.e. eyes), however, in this situation, systemic ß-blocker administration as an inpatient may be required.

3.2.6 Hemangioma