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The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas

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Abstract

Introduction

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.

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Authors and Affiliations

  1. Department of Translational Medicine Science, University of Naples “Federico II”, Naples, Italy

    Annalisa Passariello, Maria Tufano & Antonio Verrico

  2. Department of Pediatric Neurosurgery, Santobono-Pausilipon Children’s Hospital of Naples, Via Mario Fiore n. 6, 80129, Naples, Italy

    Pietro Spennato & Giuseppe Cinalli

  3. Department of Oncology, Santobono-Pausilipon Children’s Hospital of Naples, Naples, Italy

    Lucia Quaglietta & Roberta Migliorati

Authors
  1. Annalisa Passariello
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  2. Maria Tufano
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  3. Pietro Spennato
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  4. Lucia Quaglietta
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  5. Antonio Verrico
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  6. Roberta Migliorati
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  7. Giuseppe Cinalli
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Corresponding author

Correspondence to Pietro Spennato.

Additional information

This paper is dedicated to Roberta Migliorati MD (1955-2014), Chief of the Department of Pediatric Oncology Santobono-Pausilipon Children’s Hospital of Naples, Italy.

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Passariello, A., Tufano, M., Spennato, P. et al. The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas. Childs Nerv Syst 31, 1079–1088 (2015). https://doi.org/10.1007/s00381-015-2697-3

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  • DOI: https://doi.org/10.1007/s00381-015-2697-3

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