Surgical considerations in a paediatric case of a large skull-base epithelioid haemangioendothelioma

Intracranial epithelioid haemangioendothelioma (EHE) is a rare intermediate grade vascular tumour with heterogeneous clinical and histopathological behaviour. We present the surgical considerations of an exceptionally large skull-based EHE in an 11-year old female who presented to our institution with headaches and a protuberance over the left parietal area. Magnetic resonance imaging (MRI) demonstrated a left sided 10.5 × 6.6 × 11.1 cm extra-axial tumour arising from the parieto-temporaloccipital region which was continuous with the calvarium. An initial biopsy confirmed EHE. Staged treatment involved preoperative angiography and embolization. The patient underwent an extensive tumour excision and acrylic cranioplasty. Residual tumour persists in the petrous temporal bone. No neurological deficit was sustained. Postoperatively, we proceeded to tumour surveillance rather than adjuvant therapies, and follow-up imaging up to 36 months postoperatively has shown no tumour progression. We illustrate our surgical management of this large EHE and review the literature of this rare pathological entity with variable tumour behaviour and potential role for adjuvant therapy.