Choroid plexus tumor explained

Onset:Most common in infants, but can occur at any age[1]
Prognosis:Five-year survival rate

63.9%[2]

Frequency:~124 diagnoses annually (United States)

Choroid plexus tumors are a rare type of cancer that occur from the brain tissue called choroid plexus of the brain.[3] Choroid plexus tumors are uncommon tumors of the central nervous system that account for 0.5–0.6% of intracranial neoplasms in people of all ages.[4] [5] Choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma are the three World Health Organization types for these neoplasms.[6] Children under the age of five account for 10% of cases of choroid plexus tumors.[7] In children and adults, respectively, the lateral ventricle and the fourth ventricle are common locations,[8] [9] [10] About 5% of all choroid plexus tumors are located in the third ventricle.[11] Along with other unusual places such the cerebellopontine angle, the Luschka foramen, or brain parenchyma, the third ventricle is a rare location for choroid plexus tumors.[12] [13] [14] [15] Together, atypical choroid plexus papilloma, and choroid plexus carcinoma make up around 25% of all choroid plexus tumors.[16] 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.[17] [18] Most findings indicate that choroid plexus tumors have no sex predilection.[19] [20]

Clinical features

Symptoms vary depending on the size and location of the tumor and typically include headaches, nausea and vomiting, irritability, and decreased energy. [21] Choroid plexus tumors in the third ventricle region typically manifest symptoms earlier in life than tumors found in other prevalent locations, according to the anatomical characteristics.[22] 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.[23] Rarely, these tumors can cause endocrine problems or the bobbing head doll syndrome.[24]

Imaging characteristics

Choroid plexus tumors 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.[25] 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.[26] Although there are no defined imaging standards for choroid plexus carcinomas, parenchymal invasion or heterogeneous enhancement brought on by necrotic regions, calcifications, or micro-hemorrhages may provide a clue.[27]

Diagnosis

Classification

Treatment

In all choroid plexus tumors, surgical resection is the first line of therapy. Resection of tumors in this region is difficult and requires a high level of skill skilled due to the third ventricle's tiny dimensions and complicated neuroanatomy.[29] 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.[30] An alternate strategy that decreases tumor size and vascularity and makes complete excision easier is neoadjuvant chemotherapy.[31] Although it has conflicting outcomes, chemotherapy is recommended in situations with higher grade malignancies or recurrences. The role of adjuvant treatment in atypical choroid plexus papillomas is the subject of the most controversy in this regard.[32] [33] [34] When a tumor cannot be surgically removed, the best course of treatment is a neuroendoscopic biopsy followed by chemotherapy and radiation.[35]

Outcome

The degree of resection and tumor grade are the primary prognostic variables in choroid plexus tumors. According to several sources, the greatest outcome is anticipated for completely resected choroid plexus papillomas, with a 10-year survival rate of almost 100%.[36] Whereas, following total or partial resection, the 5-year survival rates for choroid plexus carcinoma patients are, respectively, 58 percent and 20 percent.

See also

External links

Notes and References

  1. Web site: Choroid Plexus Tumor. St. Jude Children's Research Hospital. March 8, 2023.
  2. Web site: Choroid Plexus Tumors Diagnosis and Treatment. 17 September 2018 . National Cancer Institute. March 8, 2023.
  3. McEvoy AW, Harding BN, Phipps KP . 20244382 . Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre . Pediatr Neurosurg . 32 . 4 . 192–9 . April 2000 . 10940770 . 10.1159/000028933. etal.
  4. 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.
  5. N. Gupta, Choroid plexus tumors in children, Neurosurg. Clin. N. Am. 14 (4) (2003) 621–631
  6. 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.
  7. D.Y. Suh, T. Mapstone, Pediatric supratentorial intraventricular tumors, FOC 10 (6)(2001) 1–14
  8. 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.
  9. 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.
  10. 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
  11. 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
  12. 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.
  13. 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.
  14. 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.
  15. 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
  16. 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.
  17. I. Nakano, A. Kondo, K. Iwasaki, Choroid plexus papilloma in the posterior third ventricle: case report, Neurosurgery 40 (6) (1997) 1279–1282.
  18. F. Tomasello, V. Albanese, F.P. Bernini, P. Picozzi, Choroid plexus papilloma of the third ventricle, Surg. Neurol. 16 (1) (1981) 69–71.
  19. 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.
  20. 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.
  21. News: Choroid Plexus Tumor. 2017-12-13. en-US.
  22. T. Tomita, D.G. McLone, A.M. Flannery, Choroid plexus papillomas of neonates, infants and children, Pediatric Neurosci. 14 (1) (1988) 23–30
  23. 25
  24. K.K. Koeller, G.D. Sandberg, From the archives of the AFIP. Cerebral
  25. 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
  26. 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.
  27. 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
  28. Book: Central Nervous System Tumours . WHO Classification of Tumours Editorial Board . International Agency for Research on Cancer . 2022 . 9789283245087 . 189–198 . en.
  29. 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
  30. 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
  31. 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.
  32. 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
  33. 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
  34. Web site: Choroid Plexus . American Brain Tumor Association. www.abta.org. 2016-12-03. https://web.archive.org/web/20161220031239/http://www.abta.org/brain-tumor-information/types-of-tumors/choroid-plexus.html?referrer=https%3A%2F%2Fwww.google.co.in%2F. 2016-12-20. dead.
  35. 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, https://doi.org/10.3171/2011.1.FOCUS10326.
  36. 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.

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