Research Article

Rapid Increase in Volume of a Neuroepithelial Cyst: An Unusual Case with High Proliferative Index

Alick Durao¹, Giacomo Piaser Guerrato², Marco Zemella² and Luca Massimi^2,3*

¹Department of Neurosurgery, Hospital de Força Aérea do Galeão, Rio de Janeiro, Brazil

²Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

³Catholic University Medical School, Rome, Italy

Submission: February 12, 2022; Published: March 08, 2023

*Corresponding author: Luca Massimi, M.D., Institute of Neurosurgery, Catholic University Medical School, Largo A. Gemelli, 8, 00168, Rome, Italy; Tel: +393397584217

How to cite this article:Alick Durao, Giacomo Piaser Guerrato, Marco Zemella and Luca Massimi*. Rapid Increase in Volume of a Neuroepithelial Cyst: An Unusual Case with High Proliferative Index. Acad J Ped Neonatol 2023; 12(2): 555897. 10.19080/AJPN.2022.12.555897

Abstract

Neuroepithelial cysts (NC) are rare cystic lesions of neuroectodermal origin, usually differentiated into ependymal and choroidal plexus variant. They can be found at any age and in any region of the central nervous system, although young age and intracranial space are the most commonly involved. In spite of their possible incidental diagnosis and their frequently benign course (with indolent or slow growth over the time), NC may show a rapid growth causing symptoms due to the mass effect. Herein, we present a rare case of quickly growing NC characterized by hyperactive cystic wall secretion.

Keywords: neuroepithelial cysts, choroid plexus cysts, ependymal cysts, microsurgery, proliferative index

Introduction

Neuroepithelial cysts (NC) are rare cystic lesions with ependymal or epithelial lining, which include ependymal and choroidal plexus cysts [1]. NC correspond to 0.4-1% of all intracranial cysts [2,3]. Both the choroidal and the ependymal variant have a neuroectodermal origin that leads to the development of a cyst starting from ectopic ependymal cells [4]. They can be found in any region of the central nervous system, but the intracranial space is the most common location [5]. The clinical course is usually benign, most of NC often remaining asymptomatic or showing an indolent growth over the time [6]. Herein, we present a case with an unusually rapid clinical course and with apparently “aggressive” pathological features.

Case presentation

This three-month old girl was admitted to our department fifteen days after the birth with a fetal ultrasounds and MRI diagnosis of intracranial cystic lesions (Figure 1). The child was in good general condition, with regular head circumference and slightly bulging anterior fontanel. No neurological or ophthalmological deficits were detected at physical examination. Postnatal brain MRI showed a large posterior midline cystic lesion with a bulky multi-lobulated left temporo-occipital component and a small right occipito-mesial cyst, associated to left temporal and occipital lobes hypoplasia (Figure 2-A,B). The cysts appeared as hypointense on T1-weighted and hyperintense on T2-weighted images and showed an enhancement of their inner wall following Gadolinium administration. The child was discharged with planned, serial clinical and radiological follow-up.

However, in the first days following the discharge, an abnormally quick head growth was noticed. The child remained in good clinical condition apart from an isolated epileptic seizure. A new MRI, performed ten days after the previous one, showed a remarkable enlargement of left cystic components with dislocation and compression of the homo-lateral cerebral peduncle (Figure 2-C,D). Therefore, the child underwent a left temporo-parieto-occipital craniotomy with wide removal of the left-sided cyst wall and marsupialisation into the sylvian and midline cisterns. At operation, a clear cleavage with brain surface was evident; the cyst wall appeared thick, tenacious and opaque (Figure 3). The post-operative course was uneventful and following neuroimaging investigation confirmed a remarkable reduction of the cyst volume, stable over the time. At current follow-up (6 years after the operation), the child shows good clinical condition and mild psychomotor delay. No recurrence of the cyst has been detected (Figure 4).

References

1. Robles LA, Paez JM, Ayala D, Boleaga-Duran B (2018) Intracranial glioependymal (neuroglial) cysts: a systematic review. Acta Neurochir (Wien) 160(7): 1439-1449.
2. Baysefer A, Erdoğan E, Gezen F, Sirin S, Timurkaynak E, et al. (1997) Cyst of the mesencephalon (neuroepithelial cyst?): review and case report. Minim Invasive Neurosurg 40(4): 148-150.
3. Wahyuhadi J, Pratama M, Wathoni R, Basuki H (2021) The Indonesian Central Nervous System Tumors Registry (INA-CTR): 7 years result from single institution of primary brain tumor epidemiology. IJN 4: 25–35.
4. Hasegawa M, Nouri M, Nagahisa S, Hayashi T, Adachi K, et al. (2015) Neuroepithelial cyst of the fourth ventricle. Childs Nerv Syst 31(1): 155-159.
5. Gankpe G, Ndekha G, Okacha N, Chakour K, Chaoui M, et al. (2019) Intracranial ependymal cyst with unsusual presentation: case report and review Literature. Interdiscipl Neurosurg 17: 45-48.
6. Niwa R, Ayako I, Komori T, Matsuo T (2022) Symptomatic Change of an Intracranial Neuroepithelial Cyst 7 Years After Its Incidental Finding. Brain Tumor Res Treat 10(1): 34-37.
7. Morigaki R, Shinno K, Pooh KH, Nakagawa Y (2011) Giant glioependymal cyst in an infant. J Neurosurg Pediatr 7(2): 175-178.
8. Kim E (2021) Cerebellar Glioependymal Cyst. Brain Tumor Res Treat 9(1): 31-34.
9. Draghi R, Mongardi L, Panzacchi R, Godano U, Barni I, et al. (2020) Choroid Plexus Cyst of the Fourth Ventricle Associated with Intermittent Obstructive Hydrocephalus. Case Reports World Neurosurg 143: 152-157.
10. Cavalheiro S, Canullo ML, Silva da Costa MD, Dastoli PA, Mendonça Nicácio J, et al. (2019) Glioependymal cyst on the third cranial nerve: case report. J Neurosurg Pediatr 1-5, doi: 10.3171/2019.8.PEDS19317.
11. Desita F, Ferriastuti W, Fauziah D (2022) Unusual presentation of a neuroepithelial cyst: A case report. Radiol Case Rep 17(11): 4384-4387.
12. Uematsu Y, Kubo K, Nishibayashi T, Ozaki F, Nakai K, et al. (2000) Interhemispheric neuroepithelial cyst associated with agenesis of the corpus callosum. A case report and review of the literature. Pediatr Neurosurg 33(1): 31-36.
13. Choque-Velasquez J, Hernesniemi J (2019) Unedited microneurosurgery of a pineal region neuroepithelial cyst. Surg Neurol Int 10: 27.
14. Ozgural O, Dogan I, Solmaz S, Morali Guler T, Kahilogullari G (2019) Transcranial endoscopic treatment of thalamic neuroepithelial cyst: case report and review of the literature. Br J Neurosurg 1-4, doi: 10.1080/02688697.2018.1552753.
15. Heran NS, Berk C, Constantoyannis C, Honey CR (2003) Neuroepithelial cysts presenting with movement disorders: two cases. Can J Neurol Sci 30(4): 393-396.