Atypowy nowotwór teratoidny/rabdoidny kanału kręgowego (AT/RT) u dwuletniego dziecka

Abstract

Author(s): Anna Mosiewicz, Robert Kaczmarczyk, Bożena Jarosz, Marzena Janczarek, Hanna Wiśniewska-Ślusarz, Barbara Mosiewicz, Tomasz Trojanowski

Atypical Teratoid/Rhabdoid Tumour (AT/RT) is an uncommon tumour with an extremely aggressive natural history. AT/RT primary localisation within the spinal canal is very rare. The aim of the study was to describe a 26-month-old boy admitted to the hospital because of low back pain lasting 3 weeks, limping, less frequent mictions followed by involuntary mictions and paraparesis of 3rd grade according to Lovett’s scale. MRI of the spine showed a pathological mass sized 52x18x17 mm located intradural extramedullary at the level Th11-L2. Laminectomy Th11-L2 was performed. The bright red soft tumour with haemorrhagic foci was resected radically without damage to the roots. Histopathologic examination showed predomination of the rhabdoid component within the texture of the tumour. Sparse small groups of neoplastic cells constitute second component of the tumour. Necrosis with distrophic calcifications were seen locally. The child was treated with chemotherapy. After third course of the treatment MRI showed recurrence of the tumour. Patient suffered from hydrocephalus, external ventriculostomy and the following ventriculoperitoneostomy were performed. After initial improvement, the clinical condition of the child began to deteriorate, he became unconscious and died four weeks after the ventriculoperitoneostomy. The time from the first symptoms to the death of the patient was only five months. The clinical course and the MRI image of AT/RT is not specific enough. The only characteristic of this tumour is its extremely rapid progress. Even the quickly undertaken surgical treatment and chemotherapy did not stop the progress of the tumour in the described patient.

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