Choroid Plexus Papilloma Workup

  • Author: Cheryl Ann Palmer, MD; Chief Editor: Allen R Wyler, MD   more...
 
Updated: Mar 8, 2012
 

Laboratory Studies

No particular laboratory studies are required for the evaluation of choroid plexus tumors. Any preoperative lab study needed to prepare the patient for surgery is indicated.

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Imaging Studies

Imaging studies are required in patients presenting with presumed mass effect.

In very young children, CT scanning is the procedure of choice. CT scan demonstrates a homogeneously hypodense to slightly hyperdense enhancing mass with cystic areas. This mass may be sizable and may be associated with hydrocephalus. Punctate calcifications, observed in 20% of tumors, are more indicative of a papilloma, whereas global calcification throughout the mass is more indicative of carcinoma. A choroid plexus carcinoma is generally associated with edema or invasion into the surrounding parenchyma, which may be observed as an area of enhancement. Areas suggestive of necrosis by imaging studies are generally not a feature of CPP but may be seen in choroid plexus carcinomas. Typically, the changes on CT scan are observed in the lateral ventricles of children and in the fourth ventricle of adults, as depicted in the image below, corresponding to the typical location of this tumor.

Imaging appearance of a fourth ventricular choroidImaging appearance of a fourth ventricular choroid plexus papilloma (CPP).

In older children and adults, MRI is indicated. The appearance of a CPP on MRI (with and without contrast) is similar to that of a CT scan and shows intermediate-to-strong intensity on both T1- and T2-weighted images. The malignant choroid plexus carcinoma appears more heterogeneous than the papilloma and often shows adjacent parenchymal invasion or surrounding edema.

Prenatal ultrasound and prenatal/antenatal MRI have also been used for diagnosis.[13]

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Diagnostic Procedures

While intraventricular lesions are readily identified on imaging studies, tumor biopsy is still warranted. Biopsy may facilitate differentiation of a papilloma from an aggressive carcinoma that may require a different surgical approach. Biopsy also helps diagnose tumors not of choroid plexus origin. Biopsy is most practically accomplished intraoperatively with the help of frozen section neuropathology consultation. However, differentiating normal choroid plexus from CPP can be very difficult.

Monitoring and normalizing excessive CSF pressure in young children is suggested prior to surgery. This is generally accomplished in the very young patient by repeated lumbar punctures and in the older patient by ventricular shunt.

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Histologic Findings

Histologically, well-differentiated CPPs are difficult to distinguish from normal choroid plexus. Both have papillae with fibrovascular cores and are lined by a single layer of cuboidal-to-columnar epithelium. Normal choroid plexus epithelial cells tend to have a hobnail shape on the ventricular side, whereas the epithelium of CPPs is more flattened, as depicted in the image below. Well-differentiated choroid plexus papillomas are designated as WHO Grade I lesions. Occasional examples of brain invasion in otherwise benign CPPs have been described, but parenchymal invasion more often heralds anaplasia.

Histologic appearance of a choroid plexus papillomHistologic appearance of a choroid plexus papilloma (CPP) stained with hematoxylin and eosin.

Atypical choroid plexus papillomas are now recognized as Grade II lesions by the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (2007).[14] They are defined as choroid plexus papillomas with increased mitotic activity. The study cited by the WHO states that a mitotic index of two or more mitoses per 10 randomly selected high power microscopic fields can establish the diagnosis.[15] These tumors often have a more solid appearance with blurring of papillary architecture, increased cellularity, nuclear pleomorphism and tumor necrosis, but these criteria are not required for the diagnosis.

Choroid plexus carcinomas, WHO Grade III, as depicted in the image below, are characterized by frank signs of malignancy such as nuclear pleomorphism and hyperchromasia, increased mitotic activity (usually greater than 5 mitoses per 10 high power fields), the occurrence of tumor giant cells and tumor necrosis. Frank invasion of the underlying brain parenchyma is also seen in high-grade lesions, however, the significance of brain invasion in an otherwise low-grade lesion is unclear.

Histologic appearance of a choroid plexus carcinomHistologic appearance of a choroid plexus carcinoma stained with hematoxylin and eosin.

The histological differential diagnosis includes choroid plexus hyperplasia, papillary ependymoma, metastatic carcinoma (in older patients), and rare entities such as medulloepithelioma or germ cell tumor, in which a papillary pattern may predominate. Distinction of these entities can be successfully accomplished with the judicious use of immunohistological markers and careful attention to the clinical and imaging features.[16]

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Staging

As with most CNS neoplasms, confinement to the intracranial cavity is usual. If myelopathic symptoms are present, consideration of dissemination into the spinal canal should warrant spinal cord neuroimaging studies.

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Contributor Information and Disclosures
Author

Cheryl Ann Palmer, MD  Professor of Pathology and Neurology, Director, Neuropathology Fellowship Program, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Medical Association of the State of Alabama, Society for Neuro-Oncology, and Southern Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Scott C Dulebohn, MD  Neurological Surgeon, Appalachian Neurosurgical

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: Medscape Salary Employment

Allen R Wyler, MD  Former Medical Director, Northstar Neuroscience, Inc

Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD  Former Medical Director, Northstar Neuroscience, Inc

Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

Additional Contributors

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

References

  1. [Best Evidence] 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. Oct 11 2011;[Medline].

  2. 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].

  3. 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. Mar 2009;92(1):87-98. [Medline].

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

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

  6. 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. Nov 15 1999;86(10):2124-32. [Medline].

  7. 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. Mar 15 2009;69(6):2219-23. [Medline].

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

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

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

  11. 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].

  12. 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. Jul-Aug 2009;57(4):486-8. [Medline].

  13. 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. Jan 10 2011;[Medline].

  14. Paulus W, Brandner S. Choroid Plexus Tumors. In: 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.

  15. 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. Nov 2006;65(11):1069-73. [Medline].

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

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

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

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

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

  21. 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. Nov-Dec 2005;25(6C):4429-33. [Medline].

  22. 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. Apr 20 2010;28(12):1995-2001. [Medline].

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

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

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

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

  27. 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. Dec 2009;95(3):383-92. [Medline].

  28. 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. Oct 2009;175(4):1631-7. [Medline].

  29. 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. Mar 2008;21(3):265-70. [Medline].

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

  31. 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. Mar 1998;42(1):37-40. [Medline].

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

  33. 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. Aug-Sep 1975;23(8-9):521-36. [Medline].

  34. 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.

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

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

  37. 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. Jun 15 2005;18(6A):E4. [Medline].

  38. 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. Jul 2005;41(11):1597-603. [Medline].

  39. 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].

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

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

  42. 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].

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

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

  45. 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. Jan 15 2005;156(2):179-82. [Medline].

 
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Imaging appearance of a fourth ventricular choroid plexus papilloma (CPP).
Histologic appearance of a choroid plexus papilloma (CPP) stained with hematoxylin and eosin.
Histologic appearance of a choroid plexus carcinoma stained with hematoxylin and eosin.
 
 
 
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