Haemangioma

§ overviewOverview

Benign neoplasm of vascular endothelium characterised by GLUT-1 positivity (infantile type), rapid proliferation then spontaneous involution.

§ epidemiologyEpidemiology

Most common tumour of infancy; 4–10% of infants; F:M 3:1; more common in Caucasians, preterm infants.

§ pathogenesisPathogenesis

Proliferation phase 0–12 mo → plateau → involution over 5–10 y; leaves residual telangiectasia or fibrofatty tissue.

§ clinicalClinical Features

• 01Bright red 'strawberry' cutaneous lesion or bluish deep swelling
• 02Blanches on pressure
• 03Head & neck in 60%
• 04Beard distribution → airway involvement
• 05PHACES syndrome (Posterior fossa, Haemangioma, Arterial, Cardiac, Eye, Sternal)

§ differentialDifferential Diagnosis

• 01Vascular malformation (present at birth, no involution)
• 02Pyogenic granuloma
• 03Kaposi sarcoma
• 04Rhabdomyosarcoma

§ investigationsInvestigations

• 01Clinical diagnosis usually sufficient
• 02USG with Doppler: high-flow lesion
• 03MRI for deep/segmental lesions
• 04Biopsy rarely required; GLUT-1 IHC differentiates from malformation

§ ihcIHC / Special Stains

• 01GLUT-1 positive (infantile haemangioma)

§ classificationClassification

• 01Infantile haemangioma (GLUT-1+)
• 02Congenital haemangioma (RICH/NICH — GLUT-1−)
• 03ISSVA 2018 classification separates tumours from vascular malformations

§ treatmentTreatment

• 01Observation for uncomplicated lesions (spontaneous involution)
• 02First-line pharmacotherapy: oral propranolol 2–3 mg/kg/day
• 03Topical timolol for superficial
• 04Systemic steroids (historical, second-line)
• 05Pulsed-dye laser for residual telangiectasia
• 06Surgical excision for functional/aesthetic residuum after involution

§ complicationsComplications

• 01Ulceration, bleeding
• 02Airway or visual obstruction
• 03High-output cardiac failure (large hepatic)
• 04Kasabach–Merritt phenomenon (with kaposiform haemangioendothelioma)

§ prognosisPrognosis

Excellent; 50% resolve by age 5, 90% by 9 years.

§ examKey Examination Points

• 01Differentiate from vascular malformation (history since birth, no involution)
• 02Assess airway and vision in periorbital/segmental lesions

§ revisionQuick Revision Summary

• 01GLUT-1+ · propranolol first line · 90% involute by age 9

§ vivaBDS Viva Questions

• 01Haemangioma vs vascular malformation?
• 02Mechanism of propranolol?
• 03What is Kasabach–Merritt syndrome?

§ mcqsMCQs — Assessment (3)

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