eMedicine Specialties > Neurosurgery > Neoplasm

Choroid Plexus Papilloma: Follow-up

Author: Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, 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
Coauthor(s): Daniel Keith Harrison, MD, Neuropathology Fellow, Department of Pathology, University of Alabama at Birmingham
Contributor Information and Disclosures

Updated: Jun 5, 2008

Outcome and Prognosis

The prognosis for both CPPs and carcinomas is determined by the completeness of lesion removal at surgery. Gross total resection of intraventricular CPPs nearly always effects a cure. Multivariate analysis of 124 patients showed increased mitotic activity (2 or more per 10 high-power fields) to be the sole histologic feature associated with recurrence, increasing the likelihood of recurrence at five years to almost 5 fold.5 Analysis of 75 pediatric cases of choroid plexus carcinomas by Fitzpatrick and colleagues (2002) also reveals the benefit of gross total tumor resection: 84% of patients with gross total resections were alive compared with 18% of patients with subtotal resections at approximately 2 years.7 Subtotally resected papillomas or carcinomas require adjuvant therapy such as chemotherapy or craniospinal irradiation. Meta-analysis data suggests that patients with incompletely resected choroid plexus carcinomas have a better prognosis with a second resection than without.8 Patients with either a subtotal resection alone or with extensive parenchymal invasion have the worst prognosis.

Complications resulting in neurological or psychological problems may also influence outcome. In some series, this number may be as high as 50%.9

As noted above, numerous chromosomal imbalances have been discovered in choroid plexus tumors. Although most of these genetic aberrations did not affect survival, significantly longer survival times were noted in patients with choroid plexus carcinomas associated with +9p and -10q.10

Future and Controversies

Increasingly widespread use of endoscopic surgery may alter the future therapy of choroid plexus neoplasms. In Gaab and Schroeder's 1998 series, many types of intraventricular lesions could be totally resected through the endoscope, with fewer and less severe complications.11

The evaluation of atypical papillomas, or of more widespread, benign-appearing papillomas, may be aided by evaluation of the proliferation index or by presence or absence of various tumor markers. Patients with CPPs with a higher proliferation index or the presence of certain markers have been shown to have worse outcomes, generally observed as tumor recurrence.

A difficult decision centers on the degree of therapy, especially in very young children. Tumor surgery adjacent to functionally important areas in the brain requires caution. Nevertheless, the infant brain is able to accommodate insults to functional areas, often without permanent deficits. Malignant transformation has been reported in pediatric patients with subtotal resection of choroid plexus papilloma (CPP).9 Because of the poor prognosis associated with malignant transformation and the ability of the infant brain to compensate, many widely agree that complete surgical resection of CPPs should be the goal, regardless of tumor size, location or clinical condition of the infant.

 


More on Choroid Plexus Papilloma

Overview: Choroid Plexus Papilloma
Workup: Choroid Plexus Papilloma
Treatment: Choroid Plexus Papilloma
Follow-up: Choroid Plexus Papilloma
Multimedia: Choroid Plexus Papilloma
References
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References

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Further Reading

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Keywords

choroid plexus tumors, choroid plexus neoplasm, CPP, choroid plexus carcinoma, intracranial neoplasms, von Hippel-Lindau syndrome, Li-Fraumeni cancer syndrome, Aicardi syndrome, simian vacuolating virus No. 40, SV40, hydrocephalus, hydrocephaly, macrocephalus, macrocephaly, increased intracranial pressure

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Contributor Information and Disclosures

Author

Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, 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)

Daniel Keith Harrison, MD, Neuropathology Fellow, Department of Pathology, University of Alabama at Birmingham
Daniel Keith Harrison, MD is a member of the following medical societies: American Society for Clinical Pathologists and College of American Pathologists
Disclosure: Nothing to disclose.

Medical Editor

Scott C Dulebohn, MD, Assistant Professor, Department of Surgery, Division of Neurosurgery, University of Minnesota College of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing 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.

CME Editor

Herbert H Engelhard III, MD, PhD, Director, UIC Neuro-Oncology Program, Chief, Division of Neuro-Oncology, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago
Herbert H Engelhard III, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, American Society for Cell Biology, American Society of Clinical Oncology, Chicago Medical Society, Congress of Neurological Surgeons, Illinois State Medical Society, Society for Neuro-Oncology, and Society for Neuroscience
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.

 
 
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