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Choroid Plexus Carcinoma in an Adolescent Male: a Case Report Patel Zeeshan Jameel* , Ashish Varma, Pooja Kumari, Keta Vagha, Jayant Vagha and Sachin Damke

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Choroid Plexus Carcinoma in an Adolescent Male: a Case Report Patel Zeeshan Jameel* , Ashish Varma, Pooja Kumari, Keta Vagha, Jayant Vagha and Sachin Damke Jameel et al. J Med Case Reports (2021) 15:184 https://doi.org/10.1186/s13256-021-02801-w CASE REPORT Open Access Choroid plexus carcinoma in an adolescent male: a case report Patel Zeeshan Jameel* , Ashish Varma, Pooja Kumari, Keta Vagha, Jayant Vagha and Sachin Damke Abstract Introduction/background: Although central nervous system tumors are the most common etiology of malignan- cies in the pediatric age group, choroid plexus carcinomas are rare, with an annual incidence rate of 0.10 per 100,000 children. Case presentation: We report the case of an adolescent male belonging to central India who had presented with a history of persistent headache, projectile vomiting, neck stifness, and an episode of generalized tonic-clonic seizure. Neurological examination was suggestive of a space-occupying lesion. Further neuroimaging was suggestive of a large left-sided choroid plexus carcinoma, later confrmed on pathological examination. Gross total resection was achieved and followed by radiation therapy. His recovery was satisfactory without any major events despite sufering from such a malignancy with a poor prognosis. Conclusion: In the absence of a global consensus on choroid plexus carcinoma management, our patient under- went a successful gross total resection and received postoperative radiotherapy. He made a satisfactory recovery with a further plan to review with gadolinium-enhanced neuroimaging at a later date. We conclude that, when possible, achieving gross total resection is of utmost importance. Keywords: Choroid plexus carcinoma, Brain tumor, Gross total resection Introduction CPC is an exceptionally rare intracranial neoplasm Central nervous system (CNS) tumors are the most characterized histologically by friable papillary or cauli- common etiology of cancer in the pediatric age group fower-like appearance, increased mitotic fgures, pleo- (0–19 years) with an incidence rate of 5.57 per 100,000 morphic nuclei, and necrosis [3]. CPC was found to be population [1]. Among them, choroid plexus tumors more common among males and signifcantly associ- (CPTs) have an average annual age-adjusted incidence ated with younger age (median age 1 year) at diagnosis rate of 0.10 per 100,000 children, being most common in comparison with CPP [4]. Te clinical presentation in the age group 0–4 years [1]. Overall, CNS tumors may is determined by the age of onset, as well as location have grave consequences, with a mortality rate of 0.65 and size of the tumor. Current management strategies, per 100,000 children.1 As per the World Health Organi- although not standardized, include gross total resection zation (WHO) classifcation, CPTs are further subclassi- of the tumor followed by chemotherapy and radiation fed into choroid plexus papilloma (CPP; WHO grade I), therapy (RT). atypical choroid plexus papilloma (WHO grade II), and Here, we describe a rare case of pediatric CPC in the choroid plexus carcinoma (CPC; WHO grade III) [2]. lateral ventricle of an adolescent male and briefy review the current management strategies. *Correspondence: [email protected] Department of Paediatrics, Jawaharlal Nehru Medical College, Sawangi (Meghe), Wardha, Maharashtra 442001, India © The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. The Creative Commons Public Domain Dedication waiver (http:// creat iveco mmons. org/ publi cdoma in/ zero/1. 0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Jameel et al. J Med Case Reports (2021) 15:184 Page 2 of 5 Case presentation cells expressing cytokeratin (focal) and P53 (difuse) but A 14-year-old adolescent male belonging to central India did not express synaptophysin, glial fbrillary acidic pro- presented to our tertiary care health facility in central tein (GFAP), or epithelial membrane antigen (EMA). India with a history of persistent headache for 2 weeks, Also, MIB-1 nuclear labeling was increased markedly, projectile vomiting for 5 days, neck stifness for 5 days, with the index being approximately 40% in areas of and a single episode of generalized tonic-clonic seizure (GTCS) lasting for 10 minutes. Papilledema was present. Systemic examination showed right-sided hemiparesis (power 2/5) with exaggerated deep tendon refexes and a positive Babinski sign suggestive of an intracranial space- occupying lesion (SOL). After initial stabilization, brain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass (6.2 × 5.2 × 4 cm), located in the left lateral ventri- cle extending difusely into the ipsilateral parietooccipi- tal region with perilesional edema with a midline shift of 6 mm (Fig. 1). Tere were no signs of spinal cord involve- ment. Magnetic resonance (MR) spectroscopy showed reduced N-acetyl-aspartate (NAA) and increased cho- line, with a lactate peak suggestive of a malignant mass. Gross total excision of the tumor was achieved with coagulation of the choroid plexus. Intraoperative fnd- ings revealed a soft, cystic, necrotic, and highly vascular tumor with intraventricular extension. Histopathologi- cal examination revealed crowded and branched papillae merging to form sheets of tumor cells disrupted by areas of necrosis with tumor cells exhibiting nuclear pleomor- phism (multilobulated, multinucleated hyperchromatic Fig. 2 Histological examination showing crowded and branched nuclei). Mitoses were readily identifed (15–20/10 hpf). papillae merging to form sheets of tumor cells disrupted by areas Surrounding brain parenchyma infltration was also of necrosis with tumor cells exhibiting nuclear pleomorphism with noted (Fig. 2). Immunohistochemistry showed tumor readily identifable mitoses Fig. 1 a T2 weighted image (T2WI) showing heterogeneous space occupying lesion (SOL) with necrotic areas with epicenter in left trigone of lateral ventricle infltrating into adjacent white matter with surrounding vasogenic edema. (b) Postcontrast T1WI showing heterogeneous enhancement with multiple nonenhancing necrotic areas Jameel et al. J Med Case Reports (2021) 15:184 Page 3 of 5 highest proliferative activity. Hence, a diagnosis of cho- Surgery remains to be the cornerstone of management, roid plexus carcinoma (CPC) was rendered. and the completeness of resection infuences patient sur- Te patient’s postoperative recovery was satisfactory vival. Achieving gross total resection (GTR) is the most with improved power (5/5 on the right side) and resolu- decisive factor for a patient’s long-term survival and tion of all symptoms. Te patient was planned for adju- prognosis. Various studies have determined that patients vant radiotherapy. He received 59 Gy of cranial radiation who have undergone GTR have signifcantly better sur- in 33 fractions using intensity-modulated radiation ther- vival rates [5, 6]. Furthermore, Mallick et al. [7] observed apy (IMRT). Follow-up imaging after 3 months showed that progression-free survival was signifcantly higher for no residual neoplastic lesion with no metastatic lesion patients with GTR in comparison with subtotal resec- elsewhere on CNS imaging (Fig. 3). Te further manage- tion (60 months versus 11 months) after eliminating the ment plan includes close follow-up for the next 6 months impact of adjuvant therapy. In addition, the extent of with neuroimaging again 1 year postsurgery. surgical resection remained a signifcant factor afecting survival [7]. Despite the merits of GTR, it is often dif- cult to achieve complete resection and it has increased Discussion morbidity. Te large size, high vascularity, difuse infl- Choroid plexus carcinoma (CPC) is a rare cause of a trative nature, and excessive friability of CPC present a hemispheric cerebral tumor arising from lateral ventri- formidable challenge for complete resection. In the pedi- cles in children. Te possible diferential diagnosis for atric population, blood loss may be life threatening as the such a hemispheric brain tumor includes choroid plexus entire circulating blood volume may be lost during the papilloma (CPP), ependymoma, atypical teratoid rhab- resection of these vascular tumors. Te surgical approach doid tumor, glioma, astrocytoma, and primitive neuroe- planned should allow good visual access to vascular sup- ctodermal tumor (PNET). Radiopathological correlation ply and maximal exposure of the tumor mass. An efec- with tissue immunohistochemistry is of essence in difer- tive intraoperative surgical strategy is to identify and entiating and establishing a confrmatory diagnosis. ligate the feeding choroidal vessel, thus facilitating the en Te extremely low incidence of CPC in children has bloc tumor mass removal. In patients with large tumors been a major obstacle in the development of standard- where the tumor is resected in
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