Choroid Plexus Carcinoma of Third Ventricle: A Rare Case Report
Abstract
Choroid plexus tumors are very rare tumors accounting for 0.5% of all brain tumors. They most commonly occur in childhood and present with features of raised intra-cranial pressure. World Health Organization (WHO) classifies them into 3 broad categories namely papilloma (grade 1), atypical papilloma (grade 2) and carcinoma (grade 3). These tumors are most commonly seen in the supratentorial compartment with lateral ventricle followed by third ventricle, being the most common site. Rarely, they may present in infratentorial region in adults. Dissemination through cerebrospinal fluid space is the inevitable natural course of the disease. Treatment requires total surgical resection with adjuvant chemoradiotherapy being controversial. In this study, we present a case of 7-year-old child who visited to the out-patient department of our center with progressive quadriparesis and altered sensorium and on imaging was found to have a well-defined, lobulated mass lesion with intense post contrast enhancement in posterior part of third ventricle with resultant obstructive hydrocephalus. The patient underwent craniotomy and on immuno-histopathological examination was diagnosed as a case of choroid plexus carcinoma. However, the patient succumbed to his illness a month after his surgery. The aim of this report is to highlight a rare entity, its diagnostic challenge and effect of early management in the form of surgery and chemo-radiotherapy.References
Paulus W, Brandner S. Choroid plexus tumours. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. Lyon, France: IARC Press; 2007. pp. 81–5
Hasselblatt M, Bohm C, Tatenhorst L, Dinh V, Newrzella D, Keyvani K, et al. Identification of novel diagnostic markers for choroid plexus tumors, a microarray-based approach. Am J Surg Pathol. 2006; 30:66–74
Kim T, Park MR, Hong EK, et al. Choroid Plexus Carcinoma in Adults: Two Case Reports. Brain Tumor Res Treat, 2019; 7(1): 48-52
Rickert CH, Paulus W. Tumors of the choroid plexus. Microsc Res Tech. 2001;52:104.
Newbould MJ, Kelsey AM, Arango JC, Ironside JW. The choroids plexus carcinomas of childhood: Histopathology, immunocytochemistry and clinicopathological correlations. Histopathology 1995; 26:137-43
Krutilkova, V., Trkova, M., Fleitz, J., Gregor, V., Novotna, K., Krepelova, A.,Sedlacek, Z. Identification of five new families strengthens the link between childhood choroid plexus carcinoma and germline TP53 mutations. European Journal of Cancer, 2005;41(11): 1597-1603
Meyers, S. P., Khademian, Z. P., Chuang, S. H., Pollack, I. F., Korones, D. N., & Zimmerman, R. A. Choroid plexus carcinomas in children: MRI features and patient outcomes. Neuroradiology. 2004;46(9): 770–780.
Crea, A., Bianco, A., Cossandi, C., Forgnone, S., Fornaro, R., Crobeddu, E., Panzarasa, G. Choroid plexus carcinoma in adults: literature review and first report of a location into the third ventricle. World Neurosurgery. 2019
Louis D et al. WHO Classification of Tumours of the Central nervous System. 5th edition. 2021.
Pawel, B. R. SMARCB1-deficient Tumors of Childhood: A Practical Guide. Pediatric and Developmental Pathology,2017;21(1), 6–28.
Singh A, Vermani S, Sharma S, Chand K. Papillary meningioma: a rare but distinct variant of malignant meningioma. Diagn Pathol. 2007;2:3
Sun, M. Z, Oh, M. C, Ivan, M. E, Kaur, G, Safaee, M, Kim, J. M,Parsa, A. T. Current management of choroid plexus carcinomas. Neurosurgical Review. 2013; 37(2): 179–192.
Zaky, W., Dhall, G., Khatua, S., Brown, R. J., Ginn, K. F., Gardner, S. L., Finlay, J. L. Choroid plexus carcinoma in children: The Head Start experience. Pediatric Blood & Cancer. 2015; 62(5): 784–789.
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