Primary cerebral rhabdomyosarcoma presenting as haemorrhagic stroke.

Abstract

Intracerebral haemorrhage (ICH) occurs mostly in the context of arterial hypertension, with typical localisations. Tumour-associated bleeding is the cause of 6-10% of ICHs, mostly from metastases. We present the case of a 40-year-old female admitted originally for neck pain of sudden onset, accompanied by nausea and marked right arm paresis. A CT-scan revealed left fronto-central cortico-subcortical haemorrhage. Cerebral angiography was normal. Two months after the initial event the residual paresis worsened and the patient developed neuropsychological deficits. A CT-scan showed oedema around the original bleeding site, on MRI a solid lesion with a diameter of 5 cm could be seen, with some cystic alterations and contact to the meninges. The tumour was surgically removed, and removal at the time was considered complete. Histological analysis proved it to be an embryonal rhabdomyosarcoma. The patient's neurological deficits gradually improved. Almost three months after the operation she complained of intense left-sided headache. On CT a hyperdense left fronto-central lesion with positive enhancement could be seen; MRI confirmed a relapse tumour and showed bleeding in the rostral portion of the tumour as well as oedema. The patient started radiation therapy with a total dose of 60 Gy. Whole body image studies at the time failed to reveal any other neoplastic lesions. Two months later a CT-scan showed continued tumour growth. We present this case as a rare aetiology of intracerebral haemorrhage, more frequently associated with arterial hypertension or vascular pathology, as well as being an unusual manifestation of embryonal rhabdomyosarcoma, rarely found in the brain. The case also serves to illustrate the importance of a thorough diagnosis including MRI imaging in patients with so-called atypical ICH.