Thoracoabdominal chordoma in a pediatric patient. A rare entity.

Autores de IIS La Fe

• Alfredo Marco Macián

  Autor

Participantes ajenos a IIS La Fe

• Negrin, F J
• Beltran, C
• Cortes, J
• Gomez-Chacon, J E
• Bordallo, M F

Grupos

• Investigación Clínica y Traslacional en Cáncer

  

  Leader

  José Gómez Codina

Abstract

INTRODUCTION: Chordoma is a rare, slow-growing notochordal neoplasm typical of adults. Less than 5% of the cases occur in children, where they are located at the skull base. Treatment involves surgical resection with or without radiotherapy. CLINICAL CASE: 14-month-old patient with a left dorsal lumbar mass, pain, and limited mobility in the lower limbs. MRI showed a left paravertebral mass infiltrating the spinal canal (T11-L1), in contact with the parietal pleura, along with abdominal extension. Chordoma diagnosis was established based on percutaneous biopsy. Full resection without safety margins was carried out using the posterior approach. One month later, tumor recurrence caused hemothorax, pleural infiltration, and rib infiltration. Two chemotherapy cycles were administered, with a good response. Reintervention was successfully conducted one month later. After 9 months, the patient is free from disease, under chemotherapy treatment and proton therapy. DISCUSSION: Although unusual, chordoma is included within the differential diagnosis of retroperitoneal masses. Prognosis depends on full resection. INTRODUCCION: El cordoma es una neoplasia de crecimiento lento, infrecuente, tipico de adultos, de origen notocordal. Menos del 5% ocurre en ninos, localizandose en la base craneal. El tratamiento implica la reseccion quirurgica asociando o no radioterapia. CASO CLINICO: Paciente de 14 meses con masa dorsolumbar izquierda, dolor y limitacion motora en miembros inferiores. La resonancia mostro masa paravertebral izquierda introduciendose en canal medular (T11-L1), en contacto con la pleura parietal, y extension abdominal. La biopsia percutanea fue diagnostica para cordoma. Se realizo reseccion completa sin margenes de seguridad, mediante abordaje posterior. Al mes debuto con hemotorax por recidiva tumoral, infiltracion pleural e implante costal. Recibio dos ciclos de quimioterapia con buena respuesta, reinterviniendose al mes exitosamente. Tras 9 meses se encuentra libre de enfermedad, en tratamiento quimioterapico y protonterapia. COMENTARIOS: Aunque inusual, el cordoma forma parte del diagnostico diferencial de masa retroperitoneal. Su pronostico dependera de la reseccion completa.