We present a patient with an intraventricular hemorrhage. Imaging identified a left atrial intraventricular mass and a vague adjacent second periventricular cystic lesion. A guided trans-sulcal approach via a left parietal craniotomy resulted in a gross total resection of both lesions. These represented two distinct lesions, the periventricular cystic lesion was an extraventricular neurocytoma (EVN) and a World Health Organization grade 1 choroid plexus papilloma (CPP). The neurocytoma required methylation studies for confirmatory diagnosis. The patient had an uneventful recovery with a normal neurological exam at 12-weeks. This documents the occurrence of two distinct central nervous system tumors, a CPP and an EVN presenting with an intraventricular hemorrhage

Craver, Randall

Derouen, Kaleb

Roberts, Oritsejolomi A.

Shelvin, Kierany

Shoap, Wesley

Volk, Jerome

English

LSU Health Digital Scholar (Louisiana State Univ.)

LSU Health Science Center LSU Health Digital Scholar School of Medicine Faculty Publications School of Medicine 1-11-2024 Clinical rarity: Simultaneous choroid plexus papilloma and extraventricular neurocytoma presenting as intraventricular hemorrhage in an adolescent Kaleb Derouen LSU Health Sciences Center - New Orleans, kdero3@lsuhsc.edu Kierany Shelvin LSU Health Sciences Center - New Orleans, kshel1@lsuhsc.edu Wesley Shoap LSU Health Sciences Center - New Orleans, wshoap@lsuhsc.edu Randall Craver LSU Health Sciences Center - New Orleans, RCrave@lsuhsc.edu Jerome Volk LSU Health Sciences Center - New Orleans, jvolk@lsuhsc.edu See next page for additional authors Follow this and additional works at: https://digitalscholar.lsuhsc.edu/som_facpubs  Part of the Neurology Commons, Neurosurgery Commons, and the Pediatrics Commons Recommended Citation Derouen, Kaleb; Shelvin, Kierany; Shoap, Wesley; Craver, Randall; Volk, Jerome; and Roberts, Oritsejolomi A., "Clinical rarity: Simultaneous choroid plexus papilloma and extraventricular neurocytoma presenting as intraventricular hemorrhage in an adolescent" (2024). School of Medicine Faculty Publications. 2532. https://digitalscholar.lsuhsc.edu/som_facpubs/2532 10.1093/jscr/rjad699 This Article is brought to you for free and open access by the School of Medicine at LSU Health Digital Scholar. It has been accepted for inclusion in School of Medicine Faculty Publications by an authorized administrator of LSU Health Digital Scholar. For more information, please contact DigitalScholar@lsuhsc.edu. Authors Kaleb Derouen, Kierany Shelvin, Wesley Shoap, Randall Craver, Jerome Volk, and Oritsejolomi A. Roberts This article is available at LSU Health Digital Scholar: https://digitalscholar.lsuhsc.edu/som_facpubs/2532 Received: October 18, 2023. Accepted: December 5, 2023Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), whichpermits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.Journal of Surgical Case Reports, 2024, 1, 1–5https://doi.org/10.1093/jscr/rjad699Case ReportCase ReportClinical rarity: simultaneous choroid plexus papillomaand extraventricular neurocytoma presenting asintraventricular hemorrhage in an adolescentKaleb Derouen*, Kierany Shelvin, Wesley Shoap, Randall Craver, Jerome Volk, Oritsejolomi A. RobertsDepartment of Neurosurgery, Children’s Hospital of New Orleans, New Orleans, LA, United States*Corresponding author. Louisiana State University School of Medicine, New Orleans, LA, United States. E-mail: kdero3@lsuhsc.eduAbstractWe present a patient with an intraventricular hemorrhage. Imaging identified a left atrial intraventricular mass and a vague adjacentsecond periventricular cystic lesion. A guided trans-sulcal approach via a left parietal craniotomy resulted in a gross total resection ofboth lesions. These represented two distinct lesions, the periventricular cystic lesion was an extraventricular neurocytoma (EVN) anda World Health Organization grade 1 choroid plexus papilloma (CPP). The neurocytoma required methylation studies for confirmatorydiagnosis. The patient had an uneventful recovery with a normal neurological exam at 12-weeks. This documents the occurrence oftwo distinct central nervous system tumors, a CPP and an EVN presenting with an intraventricular hemorrhage.Keywords: pediatric neurosurgery, choroid plexus papilloma, neurosurgeryIntroductionThe choroid plexus, a complex network of neuroepithelial cellssituated within the cerebral ventricles, plays a critical role inthe production and secretion of cerebrospinal fluid [1]. Choroidplexus neoplasms manifest in three distinct forms, distinguishedby their World Health Organization (WHO) grading: papilloma(WHO grade 1), atypical (WHO grade 2), and carcinoma (WHOgrade 3). Choroid plexus tumors represent a subset of brain malig-nancies, with an annual incidence of merely 0.3 cases per mil-lion [2]. From a comprehensive meta-analysis, the median ageof diagnosis is 3.5 years [2]. In children, these tumors predom-inantly arise in the lateral ventricles, while in adults, their pri-mary site is the fourth ventricle [3]. While traditionally consid-ered sporadic, emerging evidence has demonstrated associationsbetween choroid plexus papilloma (CPP) and various genetic con-ditions, such as Li-Fraumeni syndrome and Aicardi syndrome[4]. Diagnosis routinely relies on a battery of imaging modalities,including neurosonography, computed tomography (CT) scans,and magnetic resonance image (MRI), aimed at characterizingtumor location, dimensions, and suitability for surgical inter-vention. Presently, the first-line therapeutic approach for CPP issurgical resection, with documented recurrence frequencies beinglow, with cures approaching 100%.Central neurocytomas are often located within the ventricularcavities, and the less frequent extraventricular neurocytomas(EVN) can be located throughout the central nervous system.In 2007, WHO officially classified EVN as a distinct tumor sub-type, separating it from central neurocytomas [5]. Beyond theirdiffering anatomical locations, these two variants also displaycontrasting histological characteristics, with EVNs typically pre-senting as less cellular and exhibiting a more heterogeneoushistological appearance [6]. Given the limited number of reportedcases, the literature surrounding EVNs remains scant, offeringlimited insights into risk factors, clinical manifestations, diag-nostic strategies, and treatment modalities [7]. EVNs have nopathognomonic radiographic signs, and often mimic other astro-cytic glial tumors [8, 9]. Due to their extremely low incidence,there is no established consensus on the treatment of EVNs [9].Nevertheless, current literature suggests that complete tumorresection is considered the optimal treatment approach [10, 11].Case illustrationA 13-year-old female presented with an abrupt onset of a severeheadache accompanied by two episodes of vomiting, left-sidedjaw pain, nausea, and nasal congestion. She did not report anynumbness, tingling, muscle weakness, or changes in vision.An initial non-contrast CT scan of the head revealed a leftlateral intraventricular hemorrhage, (Fig. 1a). There were nointraparenchymal hemorrhages, midline shifts, or extra-axialfluid accumulations. MRI revealed a hemorrhagic lesion mea-suring 2.1 cm × 1.6 cm × 2.6 cm, involving the left atrium and theadjacent periventricular deep white matter (Fig. 1b). There wasthickening of the choroid plexus, with no significant vasogenicedema or acute extra-axial fluid collection, and no signs of brainherniation.Downloaded from https://academic.oup.com/jscr/article/2024/1/rjad699/7516944 by LSU Health Sciences Ctr user on 24 June 20242 | Derouen et al.Figure 1. (a)–(d) Demonstrates evidence of a hemorrhagic lesion involving the left atrium and adjacent periventricular deep white matter with amoderate amount of intraventricular hemorrhage within the left ventricle.On neurological exam, she exhibited intact cognition andappropriate mental status. Pupils were equal, round, and reactiveto light. She experienced pain upon extraocular movements.There were no discernible cranial nerve deficits, sensoryabnormalities, motor weakness, and her gait was within normalparameters.The child underwent right frontal external ventricular drainplacement. The following day, a cerebral angiogram suggested thepresence of a left intraventricular mass. The angiogram revealeda 9 mm × 15 mm left choroidal plexus tumor blush, located in theposterior aspect of the choroid plexus, with no arteriovenous fis-tulas or arteriovenous malformations. Nevertheless, it remainedunclear whether two separate lesions were present.A left parietal craniotomy with neuro-monitoring, stereotacticguidance, and intraoperative ultrasound resected both lesions.A “U” shaped dural opening was created, oriented toward thesagittal sinus, allowing for the identification of the primary motorand sensory cortices. Guided dissection enabled access to theatrium of the lateral ventricle, where the choroid plexus had aglobe shape tumor. The adjacent cystic tumor was also accessedDownloaded from https://academic.oup.com/jscr/article/2024/1/rjad699/7516944 by LSU Health Sciences Ctr user on 24 June 2024Simultaneous choroid plexus papilloma and extraventricular neurocytoma | 3Figure 2. (a) and (b) The touch preparation and frozen section controlshows similar histologic features. The cystic lesion shows a moderatelyhypercellular tumor, with uniform round nuclei, irregular distribution ofchromatin, and a moderate amount of eosinophilic cytoplasm. There isno distinct fibrillary background (Hematoxylin and eosin, 400×).by this approach. Both lesions were completely removed. Therewere no intraoperative complications.PathologyOnly one small piece of the cystic lesion was obtained andentirely submitted for touch preparation and frozen section(Fig. 2), which demonstrated a hypercellular lesion composedof uniform ovoid cells with salt-and-pepper chromatin, lackingpleomorphism, grooves, nucleoli, increased mitotic activity, andhad no papillary architecture. The intraventricular lesion wassubmitted separately and had the morphology of a benignpapilloma (Fig. 3), staining with cytokeratin AE1/AE3, with nostaining with GFAP. Because of the histological discrepancy of thefirst and second specimens, and the limited amount of tissue fromthe cystic lesion, the paraffin embedded tissue of the cystic lesionwas submitted for methylation profiling by NIH. The methylationprofiling had a maximum score of 1 for both class and subclassfor EVN, WHO grade 2 (Fig. 4).Post-operative courseAt her 12-week post-operative evaluation, follow-up MRI scansshowed no residual or recurrent disease, as illustrated in Fig. 5.This patient’s genetic workup has demonstrated no propensityfor neoplastic disease so far and she has resumed customaryactivities and routines.Figure 3. The intraventricular tumor consisted cuboidal epitheliumoverlying vascularized papillae. The nuclei are uniform without atypia.A psammomatous calcification is present in the upper portion of thefield (Hematoxylin and eosin, 100×).However, it is worth noting that early in her post-operativecourse she did encounter challenges related to visual acuity. Shehas since been receiving care by an ophthalmologist and nowutilizes corrective glasses to manage her visual concerns. Shecontinued to have intact cranial nerve function and 5/5 bilateralstrength post-operatively.DiscussionChoroid plexus tumors account for <1% of all reported cases,with a slightly higher frequency observed in the pediatric popu-lation, where they manifest in ∼3–4% of cases [10]. The precisefrequency of EVN in children remains unknown. Despite theirbenign nature, both CPPs and EVNs can result in significant mor-bidity, often associated with heightened intracranial pressure, andthe development of hydrocephalus. Consequently, patients withthese tumors typically present with a spectrum of symptoms,including nausea, vomiting, visual disturbances, unsteady gait,and pulsatile headaches [11].Our patient’s presentation was consistent with expectationsand characterized by a sudden-onset severe headache. To the bestof our knowledge, no prior documented case in the existing liter-ature has reported the simultaneous presentation of these twodistinct tumor types culminating in intraventricular hemorrhage.The occurrence of intraventricular hemorrhage in an adolescentpatient is a rare clinical finding. Awareness of the varied and oftenunusual clinical manifestations of pediatric brain tumors enablesprompt recognition and targeted intervention for this complexpathology.Existing literature underscores that due to their benigncharacter, CPPs and EVNs can be effectively cured through grosstotal resection. Surgical resection of these tumors can poseformidable challenges, attributable to their anatomical location,size, marked vascularity, and the relatively small blood volume inyoung patients [11].This case report contributes to the limited body of litera-ture addressing the management of co-occurring benign tumors,specifically CPP (WHO grade 1) and EVN (WHO grade 2). Therefore,we believe that the data presented in our single-center case reportcan enhance our understanding of these exceedingly rare tumors.This contribution is valuable not only for the existing literaturebut also for future meta-analyses of this medical condition.Downloaded from https://academic.oup.com/jscr/article/2024/1/rjad699/7516944 by LSU Health Sciences Ctr user on 24 June 20244 | Derouen et al.Figure 4. Methylation study results.Figure 5. (a) and (b) Stable postsurgical findings compatible with grosstotal resection of left atrial and adjacent periatrial tumor. No MRIevidence of residual/recurrent mass, with normal ventricular size.Author contributionsKD analyzed and interpreted the data and was the major contrib-utor in writing the manuscript. All other authors reviewed andapproved the manuscript.AcknowledgmentsThank you to Tangi Butler for performing the methylation studies.Conflict of interest statementThe authors declare that they have no competing interests.FundingNone of the authors received any funding for this study.Data availabilityThe authors declare that all of the data is available within thisarticle.References1. Hosmann A, Hinker F, Dorfer C., et al. Management of choroidplexus tumors—an institutional experience. Acta Neurochir2019;161:745–54. https://doi.org/10.1007/s00701-019-03832-5.2. Wolff JEA, Sajedi M, Brant R., et al. Choroid plexus tumours. Br JCancer 2002;87:1086–91. https://doi.org/10.1038/sj.bjc.6600609.3. Bahar M, Hashem H, Tekautz T., et al. Choroid plexus tumorsin adult and pediatric populations: the Cleveland Clinic anduniversity hospitals experience. J Neuro-Oncol 2017;132:427–32.https://doi.org/10.1007/s11060-017-2384-1.4. Gozali AE, Britt B, Shane L., et al. Choroid plexus tumors;management, outcome, and association with the li-Fraumenisyndrome: the Children’s hospital Los Angeles (CHLA) experi-ence, 1991-2010. Pediatr Blood Cancer 2012;58:905–9. https://doi.org/10.1002/pbc.23349.5. Han L, Niu H, Wang J., et al. Extraventricular neurocytoma inpediatric populations: a case report and review of the literature.Oncol Lett 2013;6:1397–405. https://doi.org/10.3892/ol.2013.1583.Downloaded from https://academic.oup.com/jscr/article/2024/1/rjad699/7516944 by LSU Health Sciences Ctr user on 24 June 2024Simultaneous choroid plexus papilloma and extraventricular neurocytoma | 56. Tortori-Donati P, Fondelli MP, Rossi A., et al. Extraventricularneurocytoma with ganglionic differentiation associated withcomplex partial seizures. Am J Neuroradiol 1999;20:724–7.7. Phi JH, Kim DG. Rare Pediatric central Neurocytomas.Neurosurg Clin N Am 2015;26:105–8. https://doi.org/10.1016/j.nec.2014.09.015.8. Byun J, Hong SH, Yoon MJ., et al. Prognosis and treatment out-comes of central neurocytomas: clinical interrogation basedon a single center experience. J Neuro-Oncol 2018;140:669–77.https://doi.org/10.1007/s11060-018-2997-z.9. Rades D, Schild SE, Fehlauer F. Defining the best available treat-ment for neurocytomas in children. Cancer 2004;101:2629–32.https://doi.org/10.1002/cncr.20695.10. Jaiswal AK, Jaiswal S, Sahu RN., et al. Choroid plexus papil-loma in children: diagnostic and surgical considerations. JPediatr Neurosci 2009;4:10–6. https://doi.org/10.4103/1817-1745.49100.11. Ogiwara H, Dipatri AJ, Alden TD., et al. Choroid plexus tumorsin pediatric patients. Br J Neurosurg 2012;26:32–7. https://doi.org/10.3109/02688697.2011.601820. Downloaded from https://academic.oup.com/jscr/article/2024/1/rjad699/7516944 by LSU Health Sciences Ctr user on 24 June 2024

Clinical rarity: Simultaneous choroid plexus papilloma and extraventricular neurocytoma presenting as intraventricular hemorrhage in an adolescent

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Clinical rarity: Simultaneous choroid plexus papilloma and extraventricular neurocytoma presenting as intraventricular hemorrhage in an adolescent

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