Congenital sacrococcygeal PNET and chemotherapy.

Abstract

We present the case of a congenital localised sacrococcygeal primitive neuroectodermal tumor treated aggressively with surgical resection and modified age-appropriate adjuvant chemotherapy. The conventional combination chemotherapy of vincristine, adriamycin, cyclophosphamide, ifosfamide and etoposide was modified to a regimen including vincristine, adriamicin, cyclophosphamide and actinomycin in order to minimise the predicted toxicity in this age group. Adjuvant "induction" chemotherapy commenced at 4 weeks of age and consisted of four cycles of vincristine, adriamycin and cyclophosphamide at 50%, 75%, 75% and 100% of recommended doses (vincristine 0.05 mg/kg, adriamycin 0.83 mg/kg daily × 2, cyclophosphamide 40 mg/kg) at 3-weekly intervals. This was followed by four cycles of "maintenance" chemotherapy with vincristine (0.025 mg/kg), actinomycin (0.025 mg/kg) and cyclophosphamide (36 mg/kg) at full recommended doses. Cardioxane at a dose of 16.6 mg/kg was infused immediately prior to the adriamycin. Our patient is thriving at 19 months out from end of treatment.

• Authors: Colin Patrick Hawkes, David R Betts, John O'Brien, Maureen J O'Sullivan, Michael Capra
• Journal: Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology (Indian J Med Paediatr Oncol) Vol. 33 Issue 3 Pg. 182-4 (Jul 2012) ISSN: 0971-5851 [Print] Germany
• PMID: 23248428 (Publication Type: Case Reports)