Malignant ectomesenchymoma is an uncommon neoplasm composed of neuroectodermal elements and one or more mesenchymal neoplastic elements. It is believed to arise from remnants of migratory neural crest cells (ectomesenchyme). The authors report the clinical and pathological findings of a 3-year-old girl with malignant ectomesenchymoma of the CNS. Embryogenesis of this tumor is discussed, and a review of the literature with 39 other cases is done.
Fibrous dysplasia (FD) is a rare tumour, representing 2.5% of all bone tumours and 7% of benign tumours. Aneurysmal bone cyst (ABC) is also an uncommon pathology, usually associated with a secondary vascular lesion consisting of an arteriovenous malformation. In this article, we relate a case report of a young female with a rare combination of FD with aneurysmal bone cyst presenting as a subarachnoid haemorrhage (SAH). Despite the possibility of clinical treatment of these lesions, this report demonstrates that symptomatic lesions may be successfully managed by surgical resection. The authors' opinion is that this treatment should be individualised depending on particularities of each case, such as localisation of the lesion, biopsy results and image exams features. We also present a critical literature review of diagnostic methods and therapeutical options for both ABC and FD, with emphasis on controversial topics surrounding these issues.
Surgery followed by radiotherapy seems to be a good alternative for the treatment of this specific type of metastasis. Thorough clinical and radiological evaluation, followed by genetic analysis of the surgical specimen, particularly with respect to the potential for tumor invasion under specific conditions, is recommended. The information obtained contributes to better management and better overall long-term outcomes for these patients.
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