Boukas Alexandros, Joshi Abhijit, Jenkins Alistair, Holliman Damian
Department of Neurosurgery, Regional Neurosciences Centre, Royal Victoria Infirmary, Newcastle Upon Tyne, UK.
Pediatr Neurosurg. 2013;49(2):93-8. doi: 10.1159/000356931. Epub 2013 Dec 21.
Cartilaginous metaplasia in ependymomas is extremely rare and only few cases have been reported in the literature. We describe a case of a 5-year-old patient with a 5th recurrence of 4th ventricle ependymoma. He was previously treated with 4 resections, chemotherapy and radiotherapy. Histopathology revealed well-differentiated chondroid tissue occupying almost the entire lesion. Near total resection was achieved for the 5th time, but the patient died 3 months later achieving a total survival of 48 months, the 3rd longest reported in literature. Multiple resections of tumour recurrence provided a new insight in this very rare tumour, as it gave us the opportunity to observe the progression of tumour aggressiveness from grade II to grade III and finally to chondroid metaplasia. Cartilaginous metaplasia in posterior fossa ependymomas is a very atypical and challenging tumour with poor overall prognosis.
室管膜瘤中的软骨化生极为罕见，文献中仅报道过少数病例。我们描述了一例5岁的第四脑室室管膜瘤第5次复发的患者。他此前接受过4次手术切除、化疗和放疗。组织病理学显示，分化良好的软骨样组织几乎占据了整个病变。第5次手术实现了近全切除，但患者在3个月后死亡，总生存期为48个月，这是文献报道中第三长的生存期。肿瘤复发的多次切除为这种极为罕见的肿瘤提供了新的见解，因为这使我们有机会观察肿瘤侵袭性从二级进展到三级，最终发展为软骨化生的过程。后颅窝室管膜瘤中的软骨化生是一种非常不典型且具有挑战性的肿瘤，总体预后较差。
