LOADING...

Choroid Plexus Papilloma

Choroid Plexus Papilloma

No Results

No Results

processing….

Choroid plexus papillomas (CPPs) are benign neoplasms of the choroid plexus, a structure made from tufts of villi within the ventricular system that produces cerebrospinal fluid (CSF). [1, 2, 3] CPPs are commonly observed in the lateral ventricles of children, but they can be encountered in adults. While the vast majority of these neoplasms are benign, a small percentage can be malignant. [4, 5, 6, 7, 8, 9]

CPPs comprise about 1% of intracranial neoplasms but 2-4% in children. The most common location is the atrium of the lateral ventricle in children and the fourth ventricle in adults. Rare locations include the third ventricle, cerebellopontine (CP) angle, and cerebral parenchyma. [7]

An image depicting a choroid plexus papilloma can be seen below.

The Surveillance and End Results (SEER) database was reviewed for population-based outcomes of choroid plexus tumors (CPTs), including choroid plexus papillomas (CPP), atypical CPPs (aCPP), and choroid plexus carcinomas (CPC). A total of 349 patients with CPTs were identified (120 CPCs, 26 aCPPs, and 203 CPPs). Patients with CPC presented at a younger age (median, 3 yr; mean, 14.8 yr) relative to CPP (median, 25 yr; mean, 28.4 yr). Histology was a significant predictor of overall survival (OS), with 5-year OS rates of 90% for CPP, 77% for aCPP, and 58% for CPC. Older age and male sex were prognostic for worse OS and cause-specific survival for CPP. Only extent of surgery had a significant impact on survival for CPC. [10]

In another review of SEER data, of 107 CPPs and 95 CPCs, more than 75% of CPCs were diagnosed in patients younger than 5 years, versus 48% for CPPs; and 65% of CPCs and 57% of CPPs occurred in males. In both groups, at least 90% of children underwent surgical resection, and gross total resection (GTR) was achieved in 67% of CPCs and 63.6% of CPPs. Almost 17% of CPCs were treated with radiation, versus only 0.9% of CPPs. More than 98% of patients with CPP were alive at the last follow-up, whereas only 62% of CPC patients were. [11, 12]

The choroid plexus is a neuroepithelial-lined papillary projection of the ventricular ependyma. The papillae consist of cores of fibrovascular tissue lined by low-cuboidal neuroepithelial cells. While benign cystic lesions of the choroid plexus are not uncommon, neoplasms are rare. Although most choroid plexus neoplasms are benign, they can become symptomatic by obstructing CSF flow, eventually leading to generalized increased intracranial pressure or mass effect.

CPPs are rare, comprising less than 1% of brain tumors in patients of all ages. However, CPPs most often occur in children and constitute 2-4% of childhood intracranial neoplasms with a predilection for younger ages. [6] CPPs comprise 4-6% of the intracranial neoplasms in children younger than 2 years and 12-13% of intracranial neoplasms in children younger than 1 year.

CPPs have been associated with von Hippel-Lindau syndrome and Li-Fraumeni syndrome. [13]

The frequency of CPPs in children is similar in China (1.5%) [14] and France (2.3%) [15] .

The male-to-female incidence ratio of CPP is 2.8:1.

No distribution by race has been described.

CPPs arise from the single layer of cuboidal epithelial cells lining the papillae of the choroid plexus. The choroid plexus is associated with the ventricular lining of the body, trigone, and inferior horn of the lateral ventricles; the foramen of Monro; the roof of the third ventricle; and the posterior portion of the roof of the fourth ventricle. The typical locations of normal choroid plexus correspond to the most common locations for a CPP to occur.

A recent study points to the role of a transmembrane receptor protein (Notch3) in the pathogenesis of human choroid plexus tumors. The Notch pathway helps regulate development of the mammalian nervous system, and activation of the Notch pathway has been increasingly recognized in human cancers. Notch3 is expressed in ventricular zone progenitor cells in the fetal brain and, when activated, can function as an oncogene. [16]

CPPs are associated with the Li-Fraumeni cancer syndrome (an autosomal dominant syndrome characterized by a germline mutation in the TP53 gene) and the Aicardi syndrome (a rare X-linked dominant condition observed in females, characterized by visual impairment, developmental delay, and seizures).

Both somatic and germline abnormalities that involve multiple genetic loci have been associated with the development of choroid plexus tumors. Recent genomic hybridization data shows that choroid plexus papillomas and choroid plexus carcinomas have characteristic chromosomal additions and deletions, which suggests that the genetic basis for these tumors is distinct. [17]

The polyoma viruses SV40, JC, and BK have also been implicated in the development of choroid plexus tumors. Choroid plexus tumors have been induced experimentally in transgenic mice using the polyomavirus common gene product, T antigen. The mechanism is thought to involve the binding of T antigen with both pRb and p53 tumor suppressor proteins, as these complexes have been identified in humans with choroid plexus tumors. [18] Research is ongoing to further elucidate the relationship between polyoma viruses and human CNS tumors.

Recent research has also demonstrated differential expression of several genes in choroid papilloma tumor cells using DNA microarray techniques on cells from 7 choroid plexus papillomas. Among the abnormalities identified was up-regulation of the TWIST-1 transcription factor, which was shown to promote proliferation and in vitro invasion. TWIST-1 is involved in the p53 tumor suppressor pathway as an inhibitor. [19]

Symptoms from choroid plexus tumors generally result from secretion of CSF by tumor cells, leading to an increased amount of fluid and, eventually, to hydrocephalus. Not infrequently, the tumor itself can cause mass effect, with symptoms depending on tumor location. In either case, eventual progression and increased intracranial pressure can occur. Cases of hydrocephalus occasionally do not resolve with surgery, possibly because of derangement of reabsorption mechanisms or blockage at other sites in the ventricular system.

Patients usually present with the following signs of increased intracranial pressure: headache, nausea and vomiting, drowsiness, ocular or gaze palsies (cranial nerves [CN] III and VI), papilledema, visual disturbances, and, eventually, blindness.

Infants, especially those with a tumor located in the third ventricle, can present with hydrocephalus or macrocephalus, as well as with associated increased intracranial pressure.

Unusual presentations include trochlear palsies (CN IV), psychosis, or occasionally, seizures.

As CPPs grow, they eventually obstruct the flow of CSF. Once the intracranial space can no longer compensate for the increase in pressure, a tension-obstruction type of hydrocephalus develops. Persistently increased intracranial pressure is not compatible with life. The pressure is alleviated by resection of the tumor or a ventricular shunting procedure.

Because the choroid plexus is located within the ventricles, the CPP can expand into a space-occupying lesion that may not cause symptoms until either the flow of CSF is blocked or the papilloma becomes large enough to press against the ventricular walls and, subsequently, the brain parenchyma.

These tumors most often occur in the lateral ventricles in children and in the fourth ventricle or cerebellopontine angle (CPA) of adults. Bilateral CPA choroid plexus papillomas have also been reported in the setting of neurofibromatosis Type 2. [20] Rarely, CPPs can also be found in the third ventricle. Other unusual or rare sites include the sella and primary intraparenchymal sites. [21, 22] Occasionally, CPPs show extensive calcification or even ossification or may lack their usual radiographic contrast enhancement. [23, 24]

In some instances, choroid plexus can be found in the cerebellopontine angle, where it has escaped the ventricle via the lateral foramen of Luschka. From this unusual placement of the choroid, or from exophytic growth of the papilloma through the foramen of Luschka, CPPs sometimes manifest in the cerebellopontine angle.

The appearance of CPPs in unusual sites most frequently occurs in the setting of von Hippel-Lindau syndrome.

Grossly, these tumors are tan and lobulated. They fill the ventricles and compress the walls; when they are benign, they do not generally invade brain parenchyma.

Contraindications to surgical correction of CPP are based on the patient’s comorbidities and his or her ability to tolerate surgery. However, watchful waiting is inappropriate in most cases. As choroid plexus tumors grow, the resulting hydrocephalus and other complications usually result in greater morbidity than occurs if tumors are removed when they are first discovered and smaller.

Lun MP, Monuki ES, Lehtinen MK. Development and functions of the choroid plexus-cerebrospinal fluid system. Nat Rev Neurosci. 2015 Aug. 16 (8):445-57. [Medline].

Liddelow SA. Development of the choroid plexus and blood-CSF barrier. Front Neurosci. 2015. 9:32. [Medline].

Spector R, Keep RF, Robert Snodgrass S, Smith QR, Johanson CE. A balanced view of choroid plexus structure and function: Focus on adult humans. Exp Neurol. 2015 May. 267:78-86. [Medline].

Rickert CH, Paulus W. Tumors of the choroid plexus. Microsc Res Tech. 2001 Jan 1. 52(1):104-11. [Medline].

Gupta N. Choroid plexus tumors in children. Neurosurg Clin N Am. 2003 Oct. 14(4):621-31. [Medline].

Abdulkader MM, Mansour NH, Van Gompel JJ, Bosh GA, Dropcho EJ, Bonnin JM, et al. Disseminated choroid plexus papillomas in adults: A case series and review of the literature. J Clin Neurosci. 2016 Oct. 32:148-54. [Medline].

Prasad GL, Mahapatra AK. Case series of choroid plexus papilloma in children at uncommon locations and review of the literature. Surg Neurol Int. 2015. 6:151. [Medline].

Bohara M, Hirabaru M, Fujio S, Higashi M, Yonezawa H, Karki P, et al. Choroid Plexus Tumors: Experience of 10 Cases with Special References to Adult Cases. Neurol Med Chir (Tokyo). 2015. 55 (12):891-900. [Medline].

Dudley RW, Torok MR, Gallegos D, Liu AK, Handler MH, Hankinson TC. Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol. 2015 Jan. 121 (1):201-7. [Medline].

Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2014 Oct 1. [Medline].

Dudley RW, Torok MR, Gallegos D, Liu AK, Handler MH, Hankinson TC. Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol. 2014 Oct 9. [Medline].

Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015 Jan. 121 (1):151-7. [Medline].

Gozali AE, Britt B, Shane L, et al. Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: The Children’s Hospital Los Angeles (CHLA) experience, 1991-2010. Pediatr Blood Cancer. 2011 Oct 11. [Medline].

Zhou D, Zhang Y, Liu H, Luo S, Luo L, Dai K. Epidemiology of nervous system tumors in children: a survey of 1,485 cases in Beijing Tiantan Hospital from 2001 to 2005. Pediatr Neurosurg. 2008. 44(2):97-103. [Medline].

Bauchet L, Rigau V, Mathieu-Daudé H, Fabbro-Peray P, Palenzuela G, Figarella-Branger D, et al. Clinical epidemiology for childhood primary central nervous system tumors. J Neurooncol. 2009 Mar. 92(1):87-98. [Medline].

Dang L, Fan X, Chaudhry A, Wang M, Gaiano N, Eberhart CG. Notch3 signaling initiates choroid plexus tumor formation. Oncogene. 2006 Jan 19. 25(3):487-91. [Medline].

Kamaly-Asl ID, Shams N, Taylor MD. Genetics of choroid plexus tumors. Neurosurg Focus. 2006 Jan 15. 20(1):E10. [Medline].

Zhen HN, Zhang X, Bu XY, Zhang ZW, Huang WJ, Zhang P, et al. Expression of the simian virus 40 large tumor antigen (Tag) and formation of Tag-p53 and Tag-pRb complexes in human brain tumors. Cancer. 1999 Nov 15. 86(10):2124-32. [Medline].

Hasselblatt M, Mertsch S, Koos B, Riesmeier B, Stegemann H, Jeibmann A. TWIST-1 is overexpressed in neoplastic choroid plexus epithelial cells and promotes proliferation and invasion. Cancer Res. 2009 Mar 15. 69(6):2219-23. [Medline].

Casar Borota O, Jacobsen EA, Scheie D. Bilateral atypical choroid plexus papillomas in cerebellopontine angles mimicking neurofibromatosis 2. Acta Neuropathol. 2006 May. 111(5):500-2. [Medline].

Ma YH, Ye K, Zhan RY, Wang LJ. Primary choroid plexus papilloma of the sellar region. J Neurooncol. 2008 May. 88(1):51-5. [Medline].

Imai M, Tominaga J, Matsumae M. Choroid plexus papilloma originating from the cerebrum parenchyma. Surg Neurol Int. 2011. 2:151. [Medline]. [Full Text].

Kocaeli H, Yilmazlar S, Abas F, Aksoy K. Total ossification of choroid plexus papilloma mimicking calcified petrous bone pathology. Pediatr Neurosurg. 2007. 43(1):67-71. [Medline].

Pratheesh R, Moorthy RK, Singh R, Rajshekhar V. Choroid plexus papilloma presenting as a non-contrast-enhancing fourth ventricular mass in a child. Neurol India. 2009 Jul-Aug. 57(4):486-8. [Medline].

Anselem O, Mezzetta L, Grangé GV, et al. Fetal tumors of the choroid plexus: is differential diagnosis between papilloma and carcinoma possible?. Ultrasound Obstet Gynecol. 2011 Jan 10. [Medline].

Paulus W, Brandner S. Choroid Plexus Tumors. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO Classification of Tumours of the Central Nervous System. 4th edition. Lyon, France: International Agency for Research on Cancer; 2007. 81-85.

Jeibmann A, Hasselblatt M, Gerss J, Wrede B, Egensperger R, Beschorner R. Prognostic implications of atypical histologic features in choroid plexus papilloma. J Neuropathol Exp Neurol. 2006 Nov. 65(11):1069-73. [Medline].

Lafay-Cousin L, Mabbott DJ, Halliday W, et al. Use of ifosfamide, carboplatin, and etoposide chemotherapy in choroid plexus carcinoma. J Neurosurg Pediatr. 2010 Jun. 5(6):615-21. [Medline].

Passariello A, Tufano M, Spennato P, Quaglietta L, Verrico A, Migliorati R, et al. The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas. Childs Nerv Syst. 2015 Jul. 31 (7):1079-88. [Medline].

Hallaert GG, Vanhauwaert DJ, Logghe K, et al. Endoscopic coagulation of choroid plexus hyperplasia. J Neurosurg Pediatr. 2012 Feb. 9(2):169-77. [Medline].

Buxton N, Punt J. Choroid plexus papilloma producing symptoms by secretion of cerebrospinal fluid. Pediatr Neurosurg. 1997 Aug. 27(2):108-11. [Medline].

Safaee M, Oh MC, Sughrue ME, Delance AR, Bloch O, Sun M, et al. The relative patient benefit of gross total resection in adult choroid plexus papillomas. J Clin Neurosci. 2013 Jun. 20(6):808-12. [Medline].

Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected?. J Neurooncol. 2002 Apr. 57(2):123-6. [Medline].

Wrede B, Liu P, Ater J. Second surgery and the prognosis of choroid plexus carcinoma–results of a meta-analysis of individual cases. Anticancer Res. 2005 Nov-Dec. 25(6C):4429-33. [Medline].

Tabori U, Shlien A, Baskin B, Levitt S, Ray P, Alon N, et al. TP53 alterations determine clinical subgroups and survival of patients with choroid plexus tumors. J Clin Oncol. 2010 Apr 20. 28(12):1995-2001. [Medline].

Rickert CH, Wiestler OD, Paulus W. Chromosomal imbalances in choroid plexus tumors. Am J Pathol. 2002 Mar. 160(3):1105-13. [Medline].

Kim IY, Niranjan A, Kondziolka D, Flickinger JC, Lunsford LD. Gamma knife radiosurgery for treatment resistant choroid plexus papillomas. J Neurooncol. 2008 Oct. 90(1):105-10. [Medline].

Chow E, Reardon DA, Shah AB. Pediatric choroid plexus neoplasms. Int J Radiat Oncol Biol Phys. 1999 May 1. 44(2):249-54. [Medline].

Gaab MR, Schroeder HW. Neuroendoscopic approach to intraventricular lesions. J Neurosurg. 1998 Mar. 88(3):496-505. [Medline].

Wrede B, Hasselblatt M, Peters O, Thall PF, Kutluk T, Moghrabi A, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009 Dec. 95(3):383-92. [Medline].

Koos B, Paulsson J, Jarvius M, Sanchez BC, Wrede B, Mertsch S. Platelet-derived growth factor receptor expression and activation in choroid plexus tumors. Am J Pathol. 2009 Oct. 175(4):1631-7. [Medline].

Nupponen NN, Paulsson J, Jeibmann A, Wrede B, Tanner M, Wolff JE. Platelet-derived growth factor receptor expression and amplification in choroid plexus carcinomas. Mod Pathol. 2008 Mar. 21(3):265-70. [Medline].

Berger C, Thiesse P, Lellouch-Tubiana A. Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery. 1998 Mar. 42(3):470-5. [Medline].

Beskonakli E, Cayli S, Bostanci U. Choroid plexus papillomas of the posterior fossa: extraventricular extension, intraventricular and primary extraventricular location. Report of four cases. J Neurosurg Sci. 1998 Mar. 42(1):37-40. [Medline].

Chen R, Cohen LG, Hallett M. Nervous system reorganization following injury. Neuroscience. 2002. 111(4):761-73. [Medline].

Chevrie-Muller C, Simon AM. [The standardization of a set of verbal aptitude tests in the 5 to 8 year-old child]. Rev Neuropsychiatr Infant. 1975 Aug-Sep. 23(8-9):521-36. [Medline].

Graham DI, Lantos PL. Tumors of the choroid plexus. In: Graham DI, Lantos PL, eds. Greenfield’s Neuropathology. Vol 2. 7th ed. Arnold, a member of the Hodder Headline Group,. 2002. 834-839:

Huang H, Reis R, Yonekawa Y. Identification in human brain tumors of DNA sequences specific for SV40 large T antigen. Brain Pathol. 1999 Jan. 9(1):33-42. [Medline].

Krieger MD, Panigrahy A, McComb JG, Nelson MD, Liu X, Gonzalez-Gomez I. Differentiation of choroid plexus tumors by advanced magnetic resonance spectroscopy. Neurosurg Focus. 2005 Jun 15. 18(6A):E4. [Medline].

Krutilkova V, Trkova M, Fleitz J, Gregor V, Novotna K, Krepelova A. Identification of five new families strengthens the link between childhood choroid plexus carcinoma and germline TP53 mutations. Eur J Cancer. 2005 Jul. 41(11):1597-603. [Medline].

Kubo S, Ogino S, Fukushima T. Immunocytochemical detection of insulin-like growth factor II (IGF-II) in choroid plexus papilloma: a possible marker for differential diagnosis. Clinical Neuropathology. 1999. 18(2):74-79. [Medline].

Meyers SP, Khademian ZP, Chuang SH. Choroid plexus carcinomas in children: MRI features and patient outcomes. Neuroradiology. 2004 Sep. 46(9):770-80. [Medline].

Okamoto H, Mineta T, Ueda S. Detection of JC virus DNA sequences in brain tumors in pediatric patients. J Neurosurg. 2005 Apr. 102(3 Suppl):294-8. [Medline].

Picht T, Jansons J, van Baalen A, Harder A, Pietilae TA. Infant with unusually large choroid plexus papilloma undergoing emergency surgery. Case report with special emphasis on the surgical strategy. Pediatr Neurosurg. 2006. 42(2):116-21. [Medline].

Sarkar C, Sharma MC, Gaikwad S. Choroid plexus papilloma: a clinicopathological study of 23 cases. Surgical Neurology. 1999. 52(1):37-39. [Medline].

Wolff JE, Sajedi M, Brant R. Choroid plexus tumours. Br J Cancer. 2002 Nov 4. 87(10):1086-91. [Medline]. [Full Text].

Zakrzewska M, Wojcik I, Zakrzewski K, Polis L, Grajkowska W, Roszkowski M. Mutational analysis of hSNF5/INI1 and TP53 genes in choroid plexus carcinomas. Cancer Genet Cytogenet. 2005 Jan 15. 156(2):179-82. [Medline].

Cheryl Ann Palmer, MD Professor of Pathology, Director of Neuropathology, Director of Pathology Residency Program, Department of Pathology, Huntsman Cancer Institute, University of Utah School of Medicine

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Society for Neuro-Oncology, International Society of Neuropathology

Disclosure: Nothing to disclose.

James Robinson Hackney, MD Neuropathology Fellow, Department of Pathology, University of Alabama at Birmingham School of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting.

The authors wish to acknowledge the contributions of Daniel Keith Harrison, MD, to prior versions of this article.

Choroid Plexus Papilloma

Research & References of Choroid Plexus Papilloma|A&C Accounting And Tax Services
Source

Leave a Reply