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eISSN: 2300-8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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5/2019
vol. 94
 
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Opis przypadku

A rare case of mediastinal fibromatosis in a child – case report

Dorota Aleksandra Nadbrzeżna
1
,
Aleksandra Raźnikiewicz
2
,
Andrzej Grabowski
2
,
Wojciech Korlacki
2
,
Tomasz Szczepański
3
,
Joanna Bulsa
3
,
Małgorzata Walaszczyk
4

1.
Department of General Surgery, Municipal Hospital Complex in Chorzow, Poland
2.
Department of Children’s Developmental Defects Surgery and Traumatology, School of Medicine with the Division of Dentistry in Zabrze, Medical University of Silesia in Katowice, Zabrze, Poland
3.
Department of Paediatric Haematology and Oncology, School of Medicine with the Division of Dentistry in Zabrze, Medical University of Silesia in Katowice, Zabrze, Poland
4.
Department of Anaesthesiology and Intensive Therapy, School of Medicine with the Division of Dentistry in Zabrze, Medical University of Silesia in Katowice, Zabrze, Poland
DOI: https://doi.org/10.5114/polp.2019.89868
Data publikacji online: 2019/11/19
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Introduction
Fibromatosis is a very rare desmoid tumour. It is characterised by local invasion and infiltration of close structures. The treatment is mainly surgical.

Material and methods
We report the case of 1.5-year-old boy admitted to the Paediatric Hospital, with a diagnosis of mediastinal desmoid fibromatosis. He was treated surgically (thoracoscopy and thoracotomy), chemotherapy was used, and he was hospitalised in the Intensive Care Unit more than once. Despite intensive treatment, the patient perished.

Results and conclusions
Fibromatosis is classified as a desmoid tumour. Rare tumour localisation and invasive tumour characteristics causes considerable difficulty in the diagnosis, despite the use of many diagnostic methods.