Introduction:
Malignant tumours of the Head & Neck in a paediatric population are rare, representing only a fraction of all paediatric malignancies (12%). They are most often derived from mesenchymal and endothelial cells, as distinct from adult malignancies where epithelial tumours prevail. Management of these tumours is often challenging due to the developing anatomy, altered physiology, rarity, and effects on growth/development. We present two cases of paediatric malignant tumours.
Case Reports:
Patient A presented at five-months-old with a rapidly enlarging swelling in the anterior mandible. Biopsy demonstrated an undifferentiated round cell sarcoma. Immunohistochemistry confirmed a BCOR-associated sarcoma. Definitive surgical management and upfront systemic anti-cancer therapy was advised at MDT discussion. Following vincristine doxorubicin cyclophosphamide/ifosfamide etoposide (VDC/IE) chemotherapy, the patient underwent surgery in the form of segmental mandibulectomy and reconstruction with autologous rib graft.
Patient B presented at 7 years with an enlarging right maxillary swelling with initial tissue sampling confirming a spindle cell malignant neoplasm. The patient proceeded to hemi-maxillectomy with the ultimate diagnosis being established as a spindle cell malignant melanoma.
Conclusion:
These malignant paediatric tumours represent exceptionally rare presentations. Comprehensive management requires a nuanced inter-disciplinary approach and treatment strategy.