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

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.

• Authors: Annalisa Passariello, Maria Tufano, Pietro Spennato, Lucia Quaglietta, Antonio Verrico, Roberta Migliorati, Giuseppe Cinalli
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 31 Issue 7 Pg. 1079-88 (Jul 2015) ISSN: 1433-0350 [Electronic] Germany
• PMID: 25863950 (Publication Type: Journal Article)

Topics

• Adolescent
• Carcinoma (diagnosis, drug therapy, surgery)
• Child, Preschool
• Choroid Plexus (pathology)
• Choroid Plexus Neoplasms (diagnosis, drug therapy, surgery)
• Female
• Follow-Up Studies
• Humans
• Infant
• Magnetic Resonance Imaging
• Male
• Papilloma, Choroid Plexus (diagnosis, drug therapy, surgery)
• Retrospective Studies