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Successful treatment with vincristine in PHACES syndrome

A Herrero Hernández1, O Escobosa Sánchez, T Acha García

  • 1Division of Pediatric Oncology, Hospital Materno-Infantil Carlos Haya, Málaga, Spain. antonio.herrero.sspa@juntadeandalucia.es

Clinical & Translational Oncology : Official Publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
|April 28, 2007
PubMed

Insights

Vincristine effectively treated PHACES syndrome in an infant with a rapidly growing mandibular hemangioma. This treatment reduced hemangioma size, offering a potential alternative for complex pediatric vascular anomalies.

Area of Science:

  • Pediatric Hematology/Oncology
  • Dermatology
  • Genetics

Background:

  • PHACES syndrome is a complex disorder characterized by posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and eye abnormalities.
  • Segmental mandibular hemangiomas can cause significant facial disfigurement and functional issues, including airway obstruction.

Observation:

  • A female infant presented at 8 months with a rapidly growing segmental mandibular hemangioma causing facial disfigurement.
  • Initial treatment with oral prednisone showed a clinical response but led to obstructive sleep apnea.

Findings:

  • Vincristine therapy was initiated at 24 months and administered for 4 months.
  • Treatment resulted in a marked decrease in the size of the mandibular hemangioma.
  • The primary side effect observed was constipation.

Implications:

  • Vincristine demonstrates efficacy in managing challenging infantile hemangiomas associated with PHACES syndrome.
  • This case highlights vincristine as a viable therapeutic option when conventional treatments like corticosteroids are insufficient or cause adverse effects.
  • Further research into vincristine's role in PHACES syndrome and other complex hemangiomas is warranted.

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