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|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 4 | Page : 619-621
Lateral ventricular cystic choroid plexus papilloma presenting with recurrent drop attacks: A rare manifestation of a rare variant
Ankit Bansal1, Amandeep Kumar1, Mehar C Sharma2, Suvendu Purkait2, Bhawani S Sharma1
1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||4-Aug-2015|
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bansal A, Kumar A, Sharma MC, Purkait S, Sharma BS. Lateral ventricular cystic choroid plexus papilloma presenting with recurrent drop attacks: A rare manifestation of a rare variant. Neurol India 2015;63:619-21
|How to cite this URL:|
Bansal A, Kumar A, Sharma MC, Purkait S, Sharma BS. Lateral ventricular cystic choroid plexus papilloma presenting with recurrent drop attacks: A rare manifestation of a rare variant. Neurol India [serial online] 2015 [cited 2020 Jun 2];63:619-21. Available from: http://www.neurologyindia.com/text.asp?2015/63/4/619/162095
Choroid plexus papillomas (CPPs) are uncommon tumors of the central nervous system, accounting for 1-5% of all pediatric brain tumors. CPPs are usually solid tumors and commonly present with features of raised intracranial pressure (ICP) due to either increased cerebrospinal fluid (CSF) production or blockade of CSF pathways. We hereby present a case of cystic lateral ventricular CPP in a 5-year-old child who presented with recurrent drop attacks. To the best of our knowledge, a lateral ventricular cystic CPP presenting with drop attacks has not been previously reported. The case is being reported for its unusual clinical and imaging features.
A 5-year-old male child presented in our outpatient department with complaints of multiple episodes of drop attacks for the past 3 months. There was no history of headache or vomiting. His developmental history and clinical examination were within normal range.
Magnetic resonance imaging revealed a large lateral ventricular cystic lesion with a mural nodule in the right temporo-parietal region. The cyst contents had an intensity similar to CSF. The mural nodule was iso-intense on both T1- and T2-weighted (T1W and T2W) images and showed an intense homogenous enhancement on contrast images [Figure 1]a-d. It was located medially in the region of the temporal choroidal fissure. There was associated mass effect and midline shift.
|Figure 1: A large temporo - parietal cystic lesion with a mural nodule located medially. The mural nodule is isointense on T1 - weighted (a) and T2 - weighted (b) images and shows homogenous enhancement on contrast images (c - d). Photomicrograph showing the papillary architecture of the tumor (×100) (e). The papillae are lined by single layer of columnar cells with a fibro - vascular core (e). Immunohistochemistry for pancytokeratin (f), S100 (g) and vimentin (h) is positive|
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The patient underwent a right temporal craniotomy and excision of the mural nodule via the middle temporal gyrus approach. The postoperative course was uneventful. There was complete subsidence of drop attacks after surgery. Histopathological examination revealed features of a CPP [Figure 1]e-h.
CPPs are usually solid tumors. They are isodense to hyperdense on CT scan and appear isointense on T1W and hyperintense on T2W images and show homogenous contrast enhancement. The cystic variants are very rare and can present as cystic tumors with or without enhancing mural nodule. The mural nodule, when present shows a homogenous enhancement and can be single or multiple. It is usually located in the medial part of the cyst, corresponding to the location of choroid plexus in the choroidal fissure of the temporal horn.  In the absence of a mural nodule, the cyst wall shows enhancement. The cyst contents radiologically appear similar to CSF.
Classically, a cyst with a mural nodule as a radiological feature is observed in pilocytic astrocytomas, pleomorphic xanthoastrocytomas, gangliogliomas, and hemangioblastomas. It is very unusual for a CPP to present as a cystic lesion with a mural nodule.
The cystic variant of CPPs, just like the solid CPPs, usually present with features of raised ICP due to increased CSF production or blockade of CSF pathways. A drop attack as a presenting symptom is a very unusual feature of cystic CPP, and only one such case report has been described previously.  Pollack et al.  had described a case of cystic CPP with bobble-head syndrome and recurrent drop attacks. However, in their case, the third ventricle rather than the lateral ventricle was involved.
Drop attacks or acute neurological deterioration is typically seen in intraventricular lesions that cause obstruction of the foramen of Monro. Colloid cyst and neurocysticercosis are two such intraventricular lesions known to present in this manner. Pollack et al.  had described a case of third ventricular cystic CPP that presented with recurrent drop attacks. The mechanism of drop attacks in the present case appears to be similar to that of the aforementioned intraventricular lesions, that is, intermittent obstruction of the foramen of Monro. The large cyst caused partial obstruction of bilateral foramina of Monro. A change in head position probably caused further obstruction of the already compromised foramina, leading to a transient rise in ICP that manifested as drop attacks.
The present case describes a rare radiological and clinical manifestation of CPP. CPPs should be considered in the differential diagnosis of cystic lesions with a mural nodule.
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Pollack IF, Schor NF, Martinez AJ, Towbin R. Bobble-head doll syndrome and drop attacks in a child with a cystic choroid plexus papilloma of the third ventricle. Case report. J Neurosurg 1995;83:729-32.