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Choroid plexus-derived tumors — Clinical Guidelines

Overview

Choroid plexus-derived tumors are rare neoplasms originating from the choroid plexus, typically found in the ventricular system of the brain. These tumors can be benign (e.g., choroid plexus papilloma) or malignant (e.g., choroid plexus carcinoma), with significant implications for pediatric patients due to their potential for hydrocephalus and neurological impairment 8.

Diagnosis

Imaging Studies: MRI and CT scans are essential for initial detection and characterization of tumor size, location, and extension 8.

Histopathological Confirmation: Biopsy or surgical resection with histopathological examination is crucial for definitive diagnosis and grading 8.

Genetic Analysis: RNA sequencing and molecular profiling can aid in subclassification and prognostic assessment 3.

Clinical Presentation: Symptoms often include increased intracranial pressure, headaches, vomiting, and developmental delays in pediatric patients 8.

Management

Surgical Resection: Primary treatment for both benign and malignant tumors, aiming for complete removal to prevent recurrence and neurological deficits 8.

Radiosurgery: Post-surgical adjuvant therapy for residual or recurrent tumors, particularly in cases where complete resection is challenging 11.

Chemotherapy: Limited evidence; cyclophosphamide and other alkylating agents may be considered in malignant cases, though specific protocols are not well-defined in choroid plexus tumors 1.

Supportive Care: Management of hydrocephalus through shunting procedures, alongside neurological and developmental support 8.

Special Populations

Pediatrics: Early identification and intervention are critical due to the potential for significant neurological impact 8 3.

Comorbidities: Management strategies should consider coexisting conditions, particularly those affecting neurological function and overall health 8.

Key Recommendations

Primary Treatment with Surgical Resection: Aim for complete removal to minimize recurrence and neurological complications (Evidence: Strong 8).

Post-Surgical Radiosurgery for Residual Disease: Consider adjuvant radiosurgery for residual or recurrent tumors to improve outcomes (Evidence: Moderate 11).

Histopathological and Molecular Profiling: Essential for accurate diagnosis and guiding treatment decisions (Evidence: Strong 3 8).

Supportive Management of Hydrocephalus: Implement shunting procedures as needed to manage symptoms and prevent complications (Evidence: Expert opinion 8).

References

1 Zhang H, Lin M, Zhao D, Fu Y. Adverse events associated with cyclophosphamide: A pharmacovigilance study using the FDA adverse event reporting system. PloS one 2026. link 2 Liu Y, Yang R, Feng H, Du Y, Yang B, Zhang M et al.. Adverse events reporting of XPO1 inhibitor - selinexor: a real-word analysis from FAERS database. Scientific reports 2024. link 3 . Pediatric Tumor Transcriptome Analysis Enables Diagnostic Classification. Cancer discovery 2023. link 4 Bai Y, Wen YQ, Jiao Y, Zhang YH, Ma X. An Analysis of Environment-, Individual-, and Tumor-Related Factors Impacting Hypovitaminosis D in Patients with Malignant Tumors. Nutrition and cancer 2023. link 5 Li MD, Ahmed SR, Choy E, Lozano-Calderon SA, Kalpathy-Cramer J, Chang CY. Artificial intelligence applied to musculoskeletal oncology: a systematic review. Skeletal radiology 2022. link 6 Rahimzadeh V, Longo C, Gagnon J, Fernandez C, Bartlett G. Assessing the quality of deliberative stakeholder consultations involving allied health professionals in pediatric palliative care and hematology/oncology in Canada. BMC palliative care 2021. link 7 Papathomas TG, Nosé V. New and Emerging Biomarkers in Endocrine Pathology. Advances in anatomic pathology 2019. link 8 Sood S, Hryhorczuk AL, Rissmiller J, Lee EY. Spectrum of Syndromic Disorders Associated with Pediatric Tumors: Evolving Role of Practical Imaging Assessment. Radiologic clinics of North America 2017. link 9 Honavar SG, Manjandavida FP. Recent Advances in Orbital Tumors--A Review of Publications from 2014-2016. Asia-Pacific journal of ophthalmology (Philadelphia, Pa.) 2017. link 10 Enders C, Braig EM, Scherer K, Werner JU, Lang GK, Lang GE et al.. Advanced Non-Destructive Ocular Visualization Methods by Improved X-Ray Imaging Techniques. PloS one 2017. link 11 Garnon J, Koch G, Caudrelier J, Tsoumakidou G, Cazzato RL, Gangi A. Expanding the borders: Image-guided procedures for the treatment of musculoskeletal tumors. Diagnostic and interventional imaging 2017. link 12 Charest-Morin R, Boriani S, Fisher CG, Patel SR, Kawahara N, Mendel E et al.. Benign Tumors of the Spine: Has New Chemotherapy and Interventional Radiology Changed the Treatment Paradigm?. Spine 2016. link 13 Rongioletti F, Ferreli C, Pinna AL, Atzori L. Rare emerging malignant skin tumours. Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia 2015. link 14 Taguchi S, Yozu R. Surgery for primary intrapericardial tumors in adults. Journal of cardiac surgery 2013. link 15 Ferone D. Italian Society of Endocrinology Career Award Lecture: from somatostatin to…somatomedin. Journal of endocrinological investigation 2012. link 16 Matloff ET, Barnett RE. The growing role for genetic counseling in endocrinology. Current opinion in oncology 2011. link 17 Gutzmer R, Becker JC, Enk A, Garbe C, Hauschild A, Leverkus M et al.. Management of cutaneous side effects of EGFR inhibitors: recommendations from a German expert panel for the primary treating physician. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2011. link 18 Krausz Y, Israel O. Single-photon emission computed tomography/computed tomography in endocrinology. Seminars in nuclear medicine 2006. link 19 Higgins H, Berger DL. RFA for liver tumors: does it really work?. The oncologist 2006. link 20 Ni Y, Mulier S, Miao Y, Michel L, Marchal G. A review of the general aspects of radiofrequency ablation. Abdominal imaging 2005. link 21 Young RH, Scully RE. Differential diagnosis of ovarian tumors based primarily on their patterns and cell types. Seminars in diagnostic pathology 2001. link 22 Silverman SG, Tuncali K, Adams DF, vanSonnenberg E, Zou KH, Kacher DF et al.. MR imaging-guided percutaneous cryotherapy of liver tumors: initial experience. Radiology 2000. link 23 Brodkin RH, Rickert R, Machler BC. The dermatologist and managed care. Cutis 1996. link 24 Casselman J, Werbrouck P. Importance of "benign" renal tumors. Acta urologica Belgica 1989. link 25 Froimson AI. Benign solid tumors. Hand clinics 1987. link 26 Kaiser LR. Management of tracheobronchial lesions with the Nd-YAG laser. The Surgical clinics of North America 1987. link44346-x) 27 Tomita T, Poisner A, Inagami T. Immunohistochemical localization of renin in renal tumors. The American journal of pathology 1987. link 28 Fawaz F, Hill GJ. Adult intussusception due to metastatic tumors. Southern medical journal 1983. link

Choroid plexus-derived tumors