title: The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas.
abstract: BACKGROUND
  We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years.
  
  
  METHODS
  Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months). Except one older patient (15 years old), all patients were younger than 3 years of age. In all patients affected by ACPP, a total surgical resection was achieved. Two children relapsed 12 and 8 months following radical removal. Both of them underwent adjuvant chemotherapy (carboplatin, cyclophosphamide, etoposide, doxorubicin, and methotrexate); a complete remission was maintained in all cases. In all three patients with CPC, it was impossible to achieve complete resection at first surgery. The response to chemotherapy was variable: in one case, it was complete with complete remission following 6 months; in one case, it was partial with reduction on volume (the patient underwent second-look surgery with complete resection); in the third case, there was no response and the patient progressed and finally died with metastatic disease, 8 months after chemotherapy was started. For children with CPC, the OS was 75% at 6 years.
  
  
  RESULTS
  In our series, surgery associated with chemotherapy led to long-term survival in 4/4 patients affected by ACPP and 2/3 patients affected by CPC. Clinical results achieved in our series confirm that our therapeutic regimen is feasible and efficient as a possible adjuvant treatment for both CPC and ACPP. It also suggests that surgery has a pivotal role in the management of most children affected by CPTs.
authors: Department of Translational Medicine Science, University of Naples "Federico II", Naples, Italy.
authors: Annalisa
authors: A
authors: Passariello
authors: Maria
authors: M
authors: Tufano
authors: Pietro
authors: P
authors: Spennato
authors: Lucia
authors: L
authors: Quaglietta
authors: Antonio
authors: A
authors: Verrico
authors: Roberta
authors: R
authors: Migliorati
authors: Giuseppe
authors: G
authors: Cinalli
mesh_terms: D000293:Adolescent; D002277:Carcinoma / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery*; D002675:Child, Preschool; D002831:Choroid Plexus / Q000473:pathology; D016545:Choroid Plexus Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D020288:Papilloma, Choroid Plexus / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery*; D012189:Retrospective Studies