[Paediatric myofibroblastic tumours. A presentation of three cases]
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M Oscoz Lizarbe1, J Hualde Olascoaga, E Rúperez García
1Hospital Virgen del Camino, Irunlarrea, Pamplona, España.
Abstract:
There cases of paediatric inflammatory myofibroblastic tumours in different locations are presented. This a rare benign disease, that can develop into malignant forms. Clinical features are associated with the location. Fever was the main symptom in all our cases. The symptoms that indicated the location of the tumour were, haematemesis in the first case, and respiratory disease in the third. In the third case our patient was diagnosed with tuberculosis due to mycobacterium avium. All cases had increased CRP, ESR, thrombocytosis and high levels of gamma globulins Surgery is curative if total resection is possible, and the diagnosis is made by histopathology. We would like to emphasise the early development in the second case as this was a 3 months-old infant.
Insights
Paediatric inflammatory myofibroblastic tumours are rare and can be benign or malignant. Fever is a key symptom, with location-specific signs like haematemesis or respiratory issues aiding diagnosis.
Area of Science:
- Pediatric Oncology
- Pathology
Background:
- Paediatric inflammatory myofibroblastic tumours (IMT) are rare neoplasms with unpredictable behavior.
- While often benign, IMT can exhibit malignant potential, necessitating accurate diagnosis and management.
Observation:
- Fever was the predominant symptom across all presented cases.
- Tumor location influenced clinical presentation, with haematemesis and respiratory disease indicating specific sites.
- One case was initially misdiagnosed as tuberculosis due to Mycobacterium avium.
Findings:
- All patients exhibited elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), thrombocytosis, and hypergammaglobulinemia.
- Histopathological diagnosis is crucial for definitive identification.
- Early onset was noted in a 3-month-old infant, highlighting the disease's potential for rapid development.
Implications:
- Complete surgical resection offers a curative pathway for paediatric IMT.
- Early diagnosis and prompt treatment are vital for favorable outcomes.
- Awareness of IMT's varied presentation is essential for pediatricians and oncologists.
