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Clinical outcome of adult choroid plexus tumors: retrospective analysis of a single institute

  • Clinical Article - Brain Tumors
  • Published:
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Abstract

Background

Choroid plexus tumors are rare brain tumors with clinical features that vary according to the histological grade. We reviewed the treatment outcomes of 15 adult patients with choroid plexus tumors, focusing on surgical outcomes and current therapeutic strategies.

Method

Patient demographic and clinical characteristics, operative findings, adjuvant therapies, disease progression and survival rates were reviewed.

Results

The median age at diagnosis was 33.7 ± 10 years (19–59 years) for patients with choroid plexus tumors. Postoperative chemotherapy was given to 26.7 % of patients, and 13.3 % of patients received radiotherapy. The Ki-67 labeling index and mitotic index increased at higher histological grades. All of the choroid plexus papilloma and atypical choroid plexus papilloma patients have survived. The overall survival rate of patients with choroid plexus carcinoma was 50 % in the first year, but none of the patients survived to the second year. Five patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion.

Conclusions

Choroid plexus papilloma and atypical choroid plexus papilloma patients can be treated with complete surgical resection. Choroid plexus carcinoma has a poor prognosis, and aggressive multi-modal treatments are generally needed for treatment. Chemotherapy and radiotherapy are important adjuvant therapies for choroid plexus carcinoma. If hydrocephalus and/or subdural effusion occur, permanent cerebrospinal fluid (CSF) diversion should be added to the therapeutic strategy.

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Conflicts of interest

None.

Author information

Authors and Affiliations

  1. Neurosurgery Clinic, Ministry of Health, Diskapi Yildirim Beyazit Research and Educational Hospital, 06610, Ankara, Turkey

    Erhan Turkoglu, Hayri Kertmen, Ahmet Metin Sanli, Ahmet Gunaydin, Levent Gurses, Behzat R. Ergun & Zeki Sekerci

  2. Pathology Clinic, Ministry of Health, Diskapi Yildirim Beyazit Research and Educational Hospital, 06610, Ankara, Turkey

    Evrim Onder

  3. Cukurambar Mah. Ogretmenler Cad. Kardelen Apt. No:5/6, 06290, Ankara, Turkey

    Erhan Turkoglu

Authors
  1. Erhan Turkoglu
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Correspondence to Erhan Turkoglu.

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Comment

In this manuscript, the Authors report retrospectively their experience of 15 adult patients with choroid plexus tumors treated during a period of 10 years. This represents a huge number of cases for a single center, if you consider the very low incidence of this tumor in the non-pediatric population worldwide (0.3 /1 million population/1 year), and together with a few other case series (Tacconi et al., Talacchi and al., and Boström et al.), this is one of the largest adult series published until now. The median follow-up was 5 years, and all patients temporarily received an EVD. The paper is well written and provides technical and management details of neurosurgical interest. If possible, the Authors suggest a radical tumor resection in adult patients with choroid plexus papillomas, because of its association with excellent long-term outcomes and tumor-progression free rates. Choroid plexus carcinomas have a poor prognosis, and multimodal treatment seems to improve survival rates after surgical excision. An open point of discussion remains the relatively new entity known as atypical choroid plexus papilloma and the need of adjuvant therapies or merely long-term follow-up for this category of tumor.

Alex Alfieri

Neuruppin, Germany

In this article, the Authors have collected a remarkable series of adult patients with choroid plexus tumors, which are rare and mostly occurring in childhood. Choroid plexus tumors are classified as gliomas deriving from choroid plexus epithelium. As clearly stated in this article and in the pertinent literature, the extent of resection and histologic grade are the most important prognostic factors determining recurrence-free and overall survival. Gross total resection must be pursued in all choroid plexus tumors. Choroid plexus papillomas (WHO grade I) can be cured by total resection alone. According to the Authors’ results, gross total resection appears achievable in most instances due to a lesser infiltrative behavior and less pathologic vascularization of these tumors, as compared to choroid plexus carcinomas (WHO grade III). These latter are significantly more aggressive, with a greater tendency for leptomeningeal dissemination and recurrence. Also, carcinomas are infiltrative tumors with a critical vascularization that often prevents total resection. An important role seems to be played by adjuvant chemo-radiation treatments, but a standard treatment protocol has not yet been defined.

We have less information regarding atypical choroid plexus papillomas (grade II) (see the literature review table in the article). Such tumors seem to have a favorable prognosis, but a greater risk of local recurrence as compared with papillomas. Increased mitotic activity is the only histologic feature to be independently associated with recurrence. Choroid plexus papillomas with an increased number of mitoses carry nearly five times the risk of recurrence as compared with tumors with less proliferative features. Therefore, this index should be taken into great consideration in planning the follow-up of patients with choroid plexus tumors.

Alfredo Conti,

Messina, Italy

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Turkoglu, E., Kertmen, H., Sanli, A.M. et al. Clinical outcome of adult choroid plexus tumors: retrospective analysis of a single institute. Acta Neurochir 156, 1461–1468 (2014). https://doi.org/10.1007/s00701-014-2138-1

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  • DOI: https://doi.org/10.1007/s00701-014-2138-1

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