Tufted angioma
Angioblastoma of Nakagawa; progressive capillary haemangioma
Tufted angioma is an uncommon ISSVA benign vascular tumour that sits on a spectrum with its locally aggressive partner kaposiform haemangioendothelioma (KHE) — they share histological features (cannonball / lobular tufts of capillaries) and clinical risk of Kasabach-Merritt phenomenon (consumptive coagulopathy + thrombocytopenia + microangiopathic haemolytic anaemia). Tufted angioma typically presents in infancy / early childhood as a slowly enlarging red-violaceous indurated plaque, often associated with pain, hyperhidrosis and hypertrichosis over the lesion. Symptomatic, progressive or KMP-associated disease should be managed through a specialist vascular-anomalies MDT; systemic sirolimus is the main modern first-line option, with vincristine and corticosteroid-based regimens used as alternatives or adjuncts. Propranolol has a variable response and should not be presented as standard first-line KMP therapy.
Clinical features
- Slowly enlarging red-violaceous indurated plaque or nodule, typically on the neck, shoulders, upper trunk, thighs.
- Onset — infancy or early childhood; rarely adult-onset.
- Often associated features within the lesion:
- Pain.
- Hyperhidrosis.
- Hypertrichosis.
- Slow expansion; does not involute (contrasts with infantile haemangioma).
- Kasabach-Merritt phenomenon (KMP) may complicate larger / younger lesions — severe consumptive thrombocytopenia, hypofibrinogenaemia, microangiopathic haemolytic anaemia.
Kasabach-Merritt phenomenon (KMP)
- Severe consumptive coagulopathy due to platelet trapping within tufted angioma / KHE.
- Profound thrombocytopenia (often < 10 × 10⁹/L), hypofibrinogenaemia (< 1 g/L), elevated D-dimer, microangiopathic haemolytic anaemia.
- Tumour enlargement, dramatic deepening of colour, bruising, bleeding.
- Mortality 20–30% historically; modern outcomes improved with sirolimus.
- NOT a complication of typical infantile haemangioma — KMP defines KHE / tufted-angioma spectrum.
Histology
- Discrete lobules ("cannonballs") of capillary tufts scattered through the dermis and subcutis.
- Sometimes referred to as "lobular capillary tufts in cannonball distribution" — pathognomonic.
- Endothelial cells, pericytes, surrounding stromal lymphatics positive for D2-40 / podoplanin.
- Differential — KHE (overlapping features; KHE has more spindled morphology), pyogenic granuloma, infantile haemangioma (GLUT1+).
Management
- Multidisciplinary vascular-anomalies MDT; specialist tertiary referral for progressive lesions, deep disease or any KMP features.
- Systemic sirolimus (rapamycin) — main modern first-line option for symptomatic / progressive disease and KMP; substantially improved outcomes since 2010s.
- Vincristine ± corticosteroids — alternative / adjunctive systemic regimen for KMP; specialist haematology-oncology input.
- Propranolol — variable response and less reliable than in infantile haemangioma; may be considered only in selected non-KMP cases or under specialist advice.
- Embolisation — selected large / vascular lesions.
- Surgical excision — small accessible lesions without KMP.
- Radiotherapy — historical; carries late-effect risk; rarely used now.
- Aspirin / pentoxifylline — selected low-grade chronic coagulopathy under specialist direction.
References
- Wassef M et al. Vascular anomalies classification — recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics; 2015.
- Adams DM et al. Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics; 2016.
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