Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 3427  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Resource Links
    Similar in PUBMED
    Article in PDF (2,143 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this Article
   Article Figures

 Article Access Statistics
    PDF Downloaded8    
    Comments [Add]    

Recommend this journal


Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 6  |  Page : 1890-1892

“Benign” Choroid Plexus Papilloma with “Atypical” Transformation and Spinal Drop Lesions

1 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Date of Submission10-Oct-2020
Date of Decision29-Mar-2021
Date of Acceptance11-Apr-2021
Date of Web Publication23-Dec-2021

Correspondence Address:
Dr. Madhivanan Karthigeyan
Department of Neurosurgery, PGIMER, Sector 12, Chandigarh - 160 012
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.333457

Rights and Permissions

How to cite this article:
Karthigeyan M, Salunke P, Siroliya A, Gupta K. “Benign” Choroid Plexus Papilloma with “Atypical” Transformation and Spinal Drop Lesions. Neurol India 2021;69:1890-2

How to cite this URL:
Karthigeyan M, Salunke P, Siroliya A, Gupta K. “Benign” Choroid Plexus Papilloma with “Atypical” Transformation and Spinal Drop Lesions. Neurol India [serial online] 2021 [cited 2022 Jan 28];69:1890-2. Available from: https://www.neurologyindia.com/text.asp?2021/69/6/1890/333457

Dear Sir,

Most atypical (grade-II) choroid plexus papillomas (CPP) are of denovo-origin with very few cases documented to temporally transform from grade-I CPP.[1],[2] The related conversion is usually a late phenomenon occurring many years after the initial diagnosis.[1],[2] We present a case of clinically aggressive CPP in cerebellopontine angle region, which recurred and disseminated within short interval with multiple spinal drop-metastases transforming into atypical histology.

A 38-year-old woman presented with 2-month complaints of left hemiparesis, seventh nerve paresis, and cerebellar signs. She had undergone a surgery elsewhere for the left cerebellopontine angle CPP 6 years prior, followed by gamma–knife-radiosurgery (GKRS) for recurrence. The present MRI demonstrated a recurrent lesion, and the patient underwent redo-excision with the postoperative imaging showing complete tumor excision [Figure 1]. The histopathology then was consistent with the previously established diagnosis of CPP (grade-I) [Figure 2].
Figure 1: a & b: Initial preoperative contrast images (a, axial; b, sagittal) show enhancing lesion in left cerebellopontine angle. c: Follow-up MRI shows small recurrence, which was treated by gamma knife radiosurgery. d–f: Increase in the size of the lesion with solid and cystic components for which patient underwent redo surgery. g: Latest follow-up imaging show complete tumor excision

Click here to view
Figure 2: a and b: Low magnification depicting tumor arranged in papillae lined by tall columnar cells (H&E x200). c: Tall columnar cells as seen in high magnification. No multilayering or sheeting pattern is noted (H&E x400). d: Thin papillae with fibrovascular cores. (H&E x400). No mitoses were observed

Click here to view

Five months later, she developed new-onset spastic, left-sided hemiparesis. Although a brain-MRI revealed no recurrence, there were fresh multiple intradural-extramedullary drop-metastasis in cervical/lumbar spine [Figure 3]. The tumors at C2-3/C5-6 levels causing cord-compression were excised in toto. This time, the microscopic features suggested a high-grade transformation into atypical-CPP (grade-II) [Figure 4]. She received craniospinal irradiation. At 14-month follow-up, there was no recurrence with the remaining lesions showing stable disease.
Figure 3: Extensive spinal dissemination. Upper panel (a-c): Pretreatment MRI shows multiple contrast enhancing lesions scattered throughout the cervical and lumbar spine suggestive of drop metastasis. Lower panel (d–f): Posttreatment images. The lesions at C2-3 and C5-6 were totally excised followed by craniospinal irradiation. The other lesions show stable disease at follow up

Click here to view
Figure 4: a: Low magnification depicting fused papillae with sheeting pattern (H&E x200). b: Tall columnar cells in high magnification with multilayering. Mitotic figures were also observed (arrow) (H&E x400). c: Focus of neural tissue invasion was noted (H&E x200). d: Ki67 labeling index revealing mitotically active cells (immunoperoxidase x200)

Click here to view

There have been only very rare instances of grade-I CPP dedifferentiating into atypical variety.[1],[2] These transformations, albeit have been over a period of many years (range, 6–19). The index case is unique in that the atypical transformation that manifested as drop metastasis did occur within few months of the redo surgery at the primary site. The paraffin sections of cranial tumor were subjected to a thorough relook by the same pathologist for possible identification of missed foci of already existing atypical histology; however, it was negative. The case thus illustrates aggressive behavior of CPP. It also provides an insight as to consider routine follow-up screening of craniospinal axis even in benign CPP, particularly those that tend to frequently recur. This way, the cases can be intervened much earlier before a frank neurological deterioration manifests.

As in our case, the reasons behind erratic tumor behavior in CPP are less understood. A remote possibility is previous GKRS to the primary tumor, which could have altered its biologic behavior.[3] The treatment protocol for metastatic CPP is not defined. Redo surgery has been preferred, while a combination of radiotherapy, chemotherapy, temozolomide, and GKRS has also been attempted.[1],[2],[4]

The report helps in understanding the potential for an early malignant conversion, and need for proactive imaging surveillance even in benign CPP, especially in recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Palmer C, Kalsi P, Scoones D, Bradey N, Strachan R. Recurrence of fourth ventricular choroid plexus papilloma with late occurrence of a spinal drop metastasis. Br J Neurosurg 2010;24:482-4.  Back to cited text no. 1
Stuivenvolt M, Mandl E, Verheul J, Fleischeuer R, Tijssen CC. Atypical transformation in sacral drop metastasis from posterior fossa choroid plexus papilloma. BMJ Case Rep 2012;2012:bcr0120125681.  Back to cited text no. 2
Yanamadala V, Williamson RW, Fusco DJ, Eschbacher J, Weisskopf P, Porter RW. Malignant transformation of a vestibular schwannoma after gamma knife radiosurgery. World Neurosurg 2013;79:593.e1-8.  Back to cited text no. 3
Abdulkader MM, Mansour NH, Van Gompel JJ, Bosh GA, Dropcho EJ, Bonnin JM, et al. Disseminated choroid plexus papillomas in adults: A case series and review of the literature. J Clin Neurosci 2016;32:148-54.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


Print this article  Email this article
Online since 20th March '04
Published by Wolters Kluwer - Medknow