On the Horizon for Supratentorial Choroid Plexus Tumors in Children

  • The International CPT-SIOP-Registry (2000 and 2009): This current study is collecting information to analyze the survival rates and the responses to surgery, radiotherapy, and chemotherapy for all choroid plexus tumors. The first analysis show that maximal resection followed by radiation seems to yield the best prognosis for patients with choroid plexus carcinoma; and VP16 followed by vincristine yields the best chemotherapy response. This analysis led to a treatment protocol for the rest of the study: A total of 6 courses of chemotherapy (VP16, 100 mg/m2 d1-5, vincristine, 1.5 mg/m2 d1, and either carboplatin, 350 mg/m2 d1+2, or cyclophosphamide, 1000 mg/m2 d1+2) was administered, and additional irradiation after the second course for those patients older than 3 years of age was given.
  • CPT-SIOP Study: Four different chemotherapy protocols are being studied in the CPT-SIOP-2009 study; recruitment is still ongoing. The different protocols are as follows: 1) standard: cyclophosphamide, carboplatin, vincristine, etoposide; 2) doxorubicin, actinomycin D, cisplatin; 3) high dose methotrexate; and 4) temozolomide, irinotecan. Radiation and intrathecal cytarabine are given to all treatment arms following a risk-adapted algorithm uniform in all treatment arms (http://www.choroidplexustumors.com/CptRegistry.aspx).
  • Combined endoscopic and microscopic surgery: Combined approaches with neuroendoscopy are increasingly being used. Being that the tumor is intraventricular, the vascular supply could be identified with the endoscope and coagulated and/or clipped, then the tumor can be resected either endoscopically or combined with an open approach with decreased blood loss. With technological advances of endoscopic tools, miniaturized ultrasonic aspirators, better coagulation, endoscopic clipping tools, a total endoscopic resection might become possible.