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Agressive pediatric myofibromatosis in a two-year-old child

C Letelier1, M Gunther1, A Alarcon2

  • 1Department of buccal and maxillo-facial surgery, faculty of dentistry, university of Chile, Chile.

Journal of Stomatology, Oral and Maxillofacial Surgery
|December 24, 2017
PubMed

Insights

Aggressive paediatric myofibromatosis, a rare fibroblastic tumor, was successfully treated with conservative surgery in a 2-year-old. This approach preserved jaw structure with no relapse after one year.

Area of Science:

  • Pediatric Oncology
  • Surgical Pathology

Background:

  • Aggressive paediatric myofibromatosis is a rare autosomal recessive disease of unknown etiology, characterized by rapid fibroblastic proliferation.
  • Tumors typically present in children, exhibiting painless growth and attachment to muscle or bone, with conservative surgical excision as the standard treatment despite potential for early relapse.

Observation:

  • A 2-year-old presented with a rapidly enlarging left submandibular mass, confirmed as aggressive paediatric myofibromatosis via histopathology.
  • Imaging revealed significant mandibular bone compromise and pharyngeal extension, indicating an advanced-stage lesion.

Findings:

  • Histopathology showed elongated fibroblastic and ovoid cells in a fibromyxoid stroma, consistent with myofibromatosis.
  • Conservative surgical excision preserved the mandible, with no signs of relapse one year post-operation.

Implications:

  • Conservative management can be effective for aggressive paediatric myofibromatosis, even with extensive bone involvement.
  • Long-term follow-up is crucial for monitoring these aggressive pediatric tumors and ensuring structural restoration.
Abstract

Keywords:
Aggressive myofibromatosisChildhoodSurgery

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