Choroid plexus tumor

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Choroid plexus tumor
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Choroid plexus tumors are a rare type of cancer that occur from the brain tissue called choroid plexus of the brain.[1] Choroid plexus tumors (CPTs) are uncommon CNS tumors that account for 0.5–0.6% of intracranial neoplasms in people of all ages.[2][3] Choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC) are the three WHO grades for these cancers, respectively.[4] Children under the age of five account for 10% of CPT cases.[5] In children and adults, respectively, the lateral ventricle and the fourth ventricle are common locations,[6][7][8] About 5% of all CPTs are located in the third ventricle.[9] Along with other unusual places such the cerebellopontine angle, the Luschka foramen, or brain parenchyma, the third ventricle is a rare location for CPTs.[10][11][12][13] Together, aCPP and CPC make up around 25% of all CPTs.[14] Although there have been reports of third ventricle choroid plexus papillomas in people in their fifth decade of life, only 14% of choroid plexus tumors are reported to arise in infants[15][16].Most findings indicate that CPTs have no known sex preferences.[17][18]

Signs and symptoms

Symptoms vary depending on the size and location of the tumor and typically include headaches, nausea and vomiting, irritability, and decreased energy. [19] CPTs in the third ventricle region typically manifest symptoms earlier in life than tumors found in other prevalent locations, according to the anatomical characteristics.[20] Macrocephaly, splayed cranial sutures, fontanel widening/bulging, and forced downward look, often known as sunset eyes, are common presentations of hydrocephalus in the pediatric population, Headaches, nauseousness, vomiting, and vision abnormalities are common in older individuals.[21] Rarely, these tumors can cause endocrine problems or the bobbing head doll syndrome.[22]


The etiology of the majority of choroid plexus tumors is unknown[23]



Imaging characteristics

Sheet of choroid plexus carcinoma invading adjacent brain parenchyma

CPTs appear as well-defined, large lobulated masses on CT and MR imaging. Due to the micro-hemorrhages and micro-calcifications, they show as hyperdense structures on CT scans.[24] Tumor signal seems to be isointense on T1 and isointense to moderately hyperintense on T2 sequences on MR imaging, respectively. These tumors show up brightly and uniformly following the injection of contrast agent on both CT and MRI because the high vascularity of structures originating from the choroid plexus causes these enhancements. Calcifications in children are uncommon, however they can occur in 14–25 percent of instances in people of all ages.[25] Although there are no defined imaging standards for CPCs, parenchymal invasion or heterogeneous enhancement brought on by necrotic regions, calcifications, or micro-hemorrhages may provide a clue.[26]


In all CPTs, surgical resection is the first line of therapy. Resection of tumors in this region is difficult and highly skilled due to the third ventricle's tiny dimensions and complicated neuroanatomy.[27] There have been a few reports of preoperative feeder artery embolization, however this method is difficult and carries a risk of vascular damage or stroke.[28] An alternate strategy that decreases tumor size and vascularity and makes complete excision easier is neoadjuvant chemotherapy.[29] Although it has conflicting outcomes, chemotherapy is recommended in situations with higher grade malignancies or recurrences. The role of adjuvant treatment in aCPPs is the subject of the most controversy in this regard.[30][31][32] When a tumor cannot be surgically removed, the best course of treatment is a neuroendoscopic biopsy followed by chemotherapy and radiation.[33]


The degree of resection and tumor grade are the primary prognostic variables in CPTs. According to several sources, the greatest outcome is anticipated for completely resected CPPs, with a 10-year survival rate of almost 100%.[34] Whereas, following total or partial resection, the 5-year survival rates for CPC patients are, respectively, 58 percent and 20 percent.[26]

See also


  1. McEvoy AW, Harding BN, Phipps KP, et al. (April 2000). "Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre". Pediatr Neurosurg. 32 (4): 192–9. doi:10.1159/000028933. PMID 10940770. S2CID 20244382. Archived from the original on 2011-11-22. Retrieved 2022-07-19.
  2. I. Felix, S. Phudhichareonrat, W.C. Halliday, L.E. Becker, Choroid plexus tumors in children: immunohistochemical and scanning-electron-microscopic features, Pediatric Neurosci. 13 (5) (1987) 263–269.
  3. N. Gupta, Choroid plexus tumors in children, Neurosurg. Clin. N. Am. 14 (4) (2003) 621–631
  4. S. Chhabda, O. Carney, F. D'Arco, T.S. Jacques, K. Mankad, The 2016 world health organization classification of tumours of the central nervous system: what the paediatric neuroradiologist needs to know, Quantitative Imag. Med. Surgery 6 (5) (2016) 486–489.
  5. D.Y. Suh, T. Mapstone, Pediatric supratentorial intraventricular tumors, FOC 10 (6) (2001) 1–14
  6. C. Bettegowda, O. Adogwa, V. Mehta, K.L. Chaichana, J. Weingart, B.S. Carson, G.I. Jallo, E.S. Ahn, Treatment of choroid plexus tumors: a 20-year single institutional experience, J. Neurosurgery. Pediatrics 10 (5) (2012) 398–405.
  7. A.K. Jaiswal, S. Jaiswal, R.N. Sahu, K.B. Das, V.K. Jain, S. Behari, Choroid plexus papilloma in children: diagnostic and surgical considerations, J. Pediatric Neurosci.4 (1) (2009) 10–16.
  8. H. Ogiwara, A.J. Dipatri Jr, T.D. Alden, R.M. Bowman, T. Tomita, Choroid plexus tumors in pediatric patients, Br. J. Neurosurg. 26 (1) (2012) 32–37
  9. M.L. Levy, A. Goldfarb, D.J. Hyder, I. Gonzales-Gomez, M. Nelson, F.H. Gilles, J.G. McComb, Choroid plexus tumors in children: significance of stromal invasion, Neurosurgery 48 (2) (2001) 303–309
  10. R. Kumar, G. Achari, D. Benerji, V.K. Jain, D.K. Chhabra, Choroid plexus papillomas of the cerebellopontine angle, Neurology India 50 (3) (2002) 352–358.
  11. A. Mishra, B.K. Ojha, A. Chandra, S.K. Singh, N. Chandra, C. Srivastava, Choroid plexus papilloma of posterior third ventricle: a case report and review of literature, Asian J. Neurosurgery 9 (4) (2014) 238.
  12. S.J. Pawar, R.R. Sharma, A.K. Mahapatra, S.D. Lad, M.M. Musa, Choroid plexus papilloma of the posterior third ventricle during infancy & childhood: report of two cases with management morbidities, Neurology India 51 (3) (2003) 379–382.
  13. A. Tavallaii, E. Keykhosravi, H. Rezaee, Telovelar approach for microsurgical resection of an unusually located choroid plexus papilloma in the luschka foramen of an infant – case report and review of literature, Interdisciplinary Neurosurgery 20 (2020) 100693
  14. D.N. Louis, H. Ohgaki, O.D. Wiestler, W.K. Cavenee, P.C. Burger, A. Jouvet, B.W. Scheithauer, P. Kleihues, The 2007 WHO classification of tumours of the central nervous system, Acta Neuropathol. 114 (2) (2007) 97–109.
  15. I. Nakano, A. Kondo, K. Iwasaki, Choroid plexus papilloma in the posterior third ventricle: case report, Neurosurgery 40 (6) (1997) 1279–1282.
  16. F. Tomasello, V. Albanese, F.P. Bernini, P. Picozzi, Choroid plexus papilloma of the third ventricle, Surg. Neurol. 16 (1) (1981) 69–71.
  17. M. Safaee, A.J. Clark, O. Bloch, M.C. Oh, A. Singh, K.I. Auguste, N. Gupta, M.W. McDermott, M.K. Aghi, M.S. Berger, A.T. Parsa, Surgical outcomes in choroid plexus papillomas: an institutional experience, J. Neurooncol. 113 (1) (2013) 117–125.
  18. J.H. Shin, H.K. Lee, A.K. Jeong, S.H. Park, C.G. Choi, D.C. Suh, Choroid plexus papilloma in the posterior cranial fossa: MR, CT, and angiographic findings, Clinical Imaging 25 (3) (2001) 154–162.
  19. "Choroid Plexus Tumor". Archived from the original on 2017-12-14. Retrieved 2017-12-13.
  20. T. Tomita, D.G. McLone, A.M. Flannery, Choroid plexus papillomas of neonates, infants and children, Pediatric Neurosci. 14 (1) (1988) 23–30
  21. [25] G. Menon, S.N. Nair, S.S. Baldawa, R.B. Rao, K.P. Krishnakumar, C.V. Gopalakrishnan, Choroid plexus tumors: an institutional series of 25 patients, Neurology India 58 (3) (2010) 429–435
  22. K.K. Koeller, G.D. Sandberg, From the archives of the AFIP. Cerebral
  23. "Choroid Plexus Tumors Diagnosis and Treatment - NCI". 17 September 2018. Archived from the original on 13 May 2022. Retrieved 1 October 2022.
  24. T.L. Coates, D.B. Hinshaw Jr., N. Peckman, J.R. Thompson, A.N. Hasso, B.A. Holshouser, D.S. Knierim, Pediatric choroid plexus neoplasms: MR, CT, and pathologic correlation, Radiology 173 (1) (1989) 81–88
  25. A.W. McEvoy, B.N. Harding, K.P. Phipps, D.W. Ellison, A.J. Elsmore, D. Thompson, W. Harkness, R.D. Hayward, Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre, Pediatr. Neurosurg. 32 (4) (2000) 192–199.
  26. 26.0 26.1 M.Z. Sun, M.C. Oh, M.E. Ivan, G. Kaur, M. Safaee, J.M. Kim, J.J. Phillips, K.I. Auguste, A.T. Parsa, Current management of choroid plexus carcinomas, Neurosurg. Rev. 37 (2) (2014) 179–192
  27. H.M. Do, W.F. Marx, H. Khanam, M.E. Jensen, Choroid plexus papilloma of the third ventricle: angiography, preoperative embolization, and histology, Neuroradiology 43 (6) (2001) 503–506
  28. F.P. Trivelato, L.B. Manzato, M.T. Rezende, P.M. Barroso, R.M. Faleiro, A.C. Ulhoa, Preoperative embolization of choroid plexus papilloma with Onyx via the anterior choroidal artery: technical note, Child'sNervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery 28 (11) (2012) 1955–1958
  29. ] E.J. Koh, K.C. Wang, J.H. Phi, J.Y. Lee, J.W. Choi, S.H. Park, K.D. Park, I.H. Kim, B.K. Cho, S.K. Kim, Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute, Childs Nerv. Syst. 30 (2) (2014) 217–225.
  30. ] F.G. Kamar, V.F. Kairouz, S.M. Nasser, S.G. Faddoul, I.C. Saikali, Atypical choroid plexus papilloma treated with single agent bevacizumab, Rare Tumors 6 (1) (2014)33–35
  31. ] A. Passariello, M. Tufano, P. Spennato, L. Quaglietta, A. Verrico, R. Migliorati, G. Cinalli, The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas, Childs Nerv. Syst. 31 (7) (2015) 1079–1088
  32. "Choroid Plexus | American Brain Tumor Association". Archived from the original on 2016-12-20. Retrieved 2016-12-03.
  33. P.A. Oppido, A. Fiorindi, L. Benvenuti, F. Cattani, S. Cipri, M. Gangemi, U. Godano,P. Longatti, C. Mascari, E. Morace, L. Tosatto, Neuroendoscopic biopsy of ventricular tumors: a multicentric experience, FOC 30 (4) (2011) E2, Archived 2022-10-01 at the Wayback Machine.
  34. ] S.J. McGirr, M.J. Ebersold, B.W. Scheithauer, L.M. Quast, E.G. Shaw, Choroid plexus papillomas: long-term follow-up results in a surgically treated series, J.Neurosurg. 69 (6) (1988) 843–849.

External links

 This article incorporates public domain material from the U.S. National Cancer Institute document: "Dictionary of Cancer Terms".