choroid plexus tumor
{{short description|Cancer of the cells that produce cerebrospinal fluid in the ventricles of the brain}}
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|prognosis = Five-year survival rate: 63.9%{{Cite web|url=https://www.cancer.gov/rare-brain-spine-tumor/tumors/choroid-plexus-tumors|title=Choroid Plexus Tumors Diagnosis and Treatment|date=17 September 2018 |publisher=National Cancer Institute|access-date=March 8, 2023}}
|frequency = ~124 diagnoses annually (United States)
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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.{{cite journal |last1=King |first1=David |last2=Bison |first2=Brigitte |last3=Cruz |first3=Ofelia |last4=Faria |first4=Claudia C. |last5=Filipek |first5=Iwona |last6=Finlay |first6=Jonathan L. |last7=Garami |first7=Miklós |last8=Kordes |first8=Uwe R. |last9=Löbel |first9=Ulrike |last10=Maclean |first10=Jilly |last11=Mikkelsen |first11=Torben Stamm |last12=Obrecht-Sturm |first12=Denise |last13=Solanki |first13=Guirish |last14=Thomas |first14=Christian |last15=Timmerman |first15=Beate |last16=Zapotocky |first16=Michal |last17=Adamski |first17=Jenny |title=An overview of the European standard clinical practice recommendations for choroid plexus tumours |journal=EJC Paediatric Oncology |date=March 2025 |pages=100226 |doi=10.1016/j.ejcped.2025.100226|doi-access=free }}{{cite journal |vauthors=McEvoy AW, Harding BN, Phipps KP |s2cid=20244382 |title=Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre |journal=Pediatr Neurosurg |volume=32 |issue=4 |pages=192–9 |date=April 2000 |pmid=10940770 |doi= 10.1159/000028933|url=http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=pne32192|display-authors=etal|url-access=subscription }} 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.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.N. Gupta, Choroid plexus tumors in children, Neurosurg. Clin. N. Am. 14 (4) (2003) 621–631 Choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma are the three World Health Organization types for these neoplasms.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. Children under the age of five account for 10% of cases of choroid plexus tumors.D.Y. Suh, T. Mapstone, Pediatric supratentorial intraventricular tumors, FOC 10 (6)
(2001) 1–14 In children and adults, respectively, the lateral ventricle and the fourth ventricle are common locations,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.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.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 About 5% of all choroid plexus tumors are located in the third ventricle.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 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.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.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.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.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 Together, atypical choroid plexus papilloma, and choroid plexus carcinoma make up around 25% of all choroid plexus tumors.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. 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.I. Nakano, A. Kondo, K. Iwasaki, Choroid plexus papilloma in the posterior third ventricle: case report, Neurosurgery 40 (6) (1997) 1279–1282. F. Tomasello, V. Albanese, F.P. Bernini, P. Picozzi, Choroid plexus papilloma of the third ventricle, Surg. Neurol. 16 (1) (1981) 69–71. Most findings indicate that choroid plexus tumors have no sex predilection.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.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.
Clinical features
Symptoms vary depending on the size and location of the tumor and typically include headaches, nausea and vomiting, irritability, and decreased energy. {{Cite news|url=https://www.stjude.org/disease/choroid-plexus-tumor.html|title=Choroid Plexus Tumor|access-date=2017-12-13|language=en-US}} 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.T. Tomita, D.G. McLone, A.M. Flannery, Choroid plexus papillomas of neonates, infants and children, Pediatric Neurosci. 14 (1) (1988) 23–30 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.[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 Rarely, these tumors can cause endocrine problems or the bobbing head doll syndrome.K.K. Koeller, G.D. Sandberg, From the archives of the AFIP. Cerebral
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.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 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.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. 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.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
Diagnosis
=Classification=
- Choroid plexus carcinoma (WHO grade 3)
- Atypical choroid plexus papilloma (WHO grade 2)
- Choroid plexus papilloma (WHO grade 1){{Cite book |title=Central Nervous System Tumours |collaboration=WHO Classification of Tumours Editorial Board |publisher=International Agency for Research on Cancer |year=2022 |isbn=9789283245087 |pages=189–198 |language=en}}
Treatment
In all choroid plexus tumors, maximal surgical resection is the first line of therapy. Resection of tumors in this region is difficult and requires a high level of expertise.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 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.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 An alternative strategy that decreases tumor size and vascularity and makes complete excision easier is neoadjuvant chemotherapy.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. Chemotherapy confers a definite survival benefit in choroid plexus carcinoma and maybe beneficial in selected cases of atypical choroid plexus papillomas.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 When a tumor cannot be surgically removed, the best course of treatment is a neuroendoscopic biopsy followed by chemotherapy and radiation.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.
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%.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. 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
References
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External links
- [http://www.cancer.gov/dictionary?CdrID=46185 Choroid plexus tumor] in the NCI Dictionary of Cancer Terms
{{Central nervous system tumors}}
{{NCI-cancer-dict}}
{{DEFAULTSORT:Choroid Plexus Tumor}}