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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 6 | Page : 1890-1892
“Benign” Choroid Plexus Papilloma with “Atypical” Transformation and Spinal Drop Lesions
Madhivanan Karthigeyan1, Pravin Salunke1, Anshul Siroliya1, Kirti Gupta2
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 Submission||10-Oct-2020|
|Date of Decision||29-Mar-2021|
|Date of Acceptance||11-Apr-2021|
|Date of Web Publication||23-Dec-2021|
Dr. Madhivanan Karthigeyan
Department of Neurosurgery, PGIMER, Sector 12, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
|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
Most atypical (grade-II) choroid plexus papillomas (CPP) are of denovo-origin with very few cases documented to temporally transform from grade-I CPP., The related conversion is usually a late phenomenon occurring many years after the initial diagnosis., 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|
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|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|
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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|
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|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)|
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There have been only very rare instances of grade-I CPP dedifferentiating into atypical variety., 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. 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.,,
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]