Multicentric infantile myofibromatosis: two perinatal cases
Fanny Pelluard-Nehmé1, Frederic Coatleven, Dominique Carles
1Unité de Foetopathologie, Service d'Anatomie et Cytologie Pathologique, Centre Hospitalo-Universitaire de Bordeaux, Place Amélie Raba Léon, 33076, Bordeaux Cedex, France. fanny.pelluard@chu-bordeaux.fr
European Journal of Pediatrics
|December 23, 2006
Summary
Infantile myofibromatosis, a rare fibrous tumor in infants, can cause severe complications. This report details two cases with in utero growth, leading to fetal and infant death, highlighting a previously undocumented cause of fetal demise.
Area of Science:
- Pediatric Pathology
- Developmental Biology
- Oncology
Background:
- Infantile myofibromatosis is the most common fibrous tumor in infancy.
- It presents in solitary, multiple, or generalized forms with varying prognoses.
- Characterized by mesenchymal proliferation in various tissues, including skin, muscles, viscera, bones, and subcutaneous areas.
Observation:
- Two cases of multicentric infantile myofibromatosis with significant intrauterine lesional growth were observed.
- Visceral lesions in infantile myofibromatosis are linked to high morbidity and mortality in early infancy.
- These lesions can cause failure to thrive, infection, hemorrhage, and organ obstruction.
Findings:
- Significant in utero lesional growth in multicentric infantile myofibromatosis led to fetal demise in one case.
- Post-natal demise occurred in the second case due to complications from the condition.
- This represents the first reported instance of fetal death attributed to infantile myofibromatosis.
Implications:
- The findings underscore the critical and potentially fatal impact of infantile myofibromatosis, even before birth.
- Early recognition and understanding of intrauterine lesional growth are crucial for managing this condition.
- This case report expands the known spectrum of infantile myofibromatosis complications and its prognostic implications.
