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Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Choroid-plexus papillomas are rare intra-ventricular tumors derived from the choroid-plexus epithelium. They are predominantly found in children. The malignant choroid-plexus carcinoma is even less common. Treatment is surgical, and the outcome is highly dependent on the completeness of resection. Radiotherapy is reserved for incompletely removed tumors and malignant lesions. CSF spread can occur even in low-grade lesions.
Anatomical and Biological Imaging of Pediatric Brain Tumor
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Rob A. Dineen, Shivaram Avula, Andrew C. Peet, Giovanni Morana, Monika Warmuth-Metz
Choroid plexus carcinoma (Figure 5.26) is a WHO grade III tumor most commonly seen in children under 2 years of age. These tumors are typically intraventricular and appear as a heterogeneous mass with lobulated or irregular margin.166 The lesions are predominantly isointense to gray matter on T1- and T2-weighted imaging and may show cystic areas and surrounding edema. Enhancement following administraction of GBCA is variable, ranging from absent, heterogeneous, to marked and uniform. Differentiation from choroid plexus papilloma is not always possible, but choroid plexus carcinomas are typically larger than papillomas162 and have lower ADC (Figure 5.26f)16 and myo-inositol. Metastases may be present in around a fifth of patients at presentation (Figure 5.26e).164
Central nervous system
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
Are the following statements regarding choroid plexus papilloma true or false? Peak age is between six and 10 years.The commonest location is within the third ventricle.It may transform into choroid plexus carcinoma.Calcification is rare.The ventricles typically appear asymmetrically dilated.
Norrie disease with a spontaneously shrinking choroid plexus abnormality: a case report
Published in Ophthalmic Genetics, 2021
Subhi Talal Younes, James Mason Shiflett, Kristin Weaver, Andrew Smith, Betty Herrington, Charlotte Taylor, Kartik Reddy
As a whole, choroid plexus masses are rare in children, accounting for 1% or less of intracranial tumors in children. The most common choroid plexus lesions in children include choroid plexus cysts, choroid plexus papilloma, or choroid plexus carcinoma. Choroid plexus cysts are believed to arise from fluid trapping within the developing neuroepithelium (12). Estimated to be present in one to 2% of the population, such cysts require no intervention. Choroid plexus papilloma and carcinoma are neoplasms; as such, they require treatment. The former can be cured with surgery alone (13). The latter is typically a manifestation of an underlying cancer predisposition syndrome, most commonly, Li Fraumeni (14). These aggressive tumors require resection and adjuvant therapy, usually craniospinal irradiation or intensive chemotherapy regimens (15,16). Unfortunately, survival for choroid plexus carcinoma remains poor. For reasons explained above, none of these entities appeared to be the diagnosis in this patient.
Omics-driven drug repurposing as a source of innovative therapies in rare cancers
Published in Expert Opinion on Orphan Drugs, 2018
Choroid plexus carcinoma is a rare, aggressive pediatric brain tumor often associated with Li–Fraumeni syndrome or somatic TP53 mutation. There are no standard curative therapies for relapsed disease and the prognosis is almost universally grim. A 23-month-old child with refractory metastatic disease was enrolled on a precision oncology trial (NCT01802567) and genomic analysis revealed an array of actionable pathways including mTOR, PDGFRB, FGF2, HDAC3, and HDAC8 [13]. Four drugs were selected to target these pathways: sunitinib, thalidomide, sirolimus (a non-cancer drug), and vorinostat. Of note, this is an all oral cocktail of drugs – in line with the wishes of the child’s family. Clinical response showed significant regression in all lesions during 36 months of treatment, and no further progression in the 12 months following cessation of treatment.
Choroid Plexus Carcinoma with Hyaline Globules: An Uncommon Histological Finding
Published in Fetal and Pediatric Pathology, 2022
Shilpy Jha, Suvendu Purkait, Chinmaya Dash, Chinzah Lalsangzuala
The tumor cells were immunopositivity for pan-cytokeratin, while epithelial membrane antigen (EMA), alpha-fetoprotein (AFP), and SALL-4 were negative. Immunostain positivity for INI-1 and ATRX was retained. The Mib-1 labeling index was 4–5% in the areas of most mitotic activity. The hyaline globules were immunopositive for AFP (Fig. 1(F)). All other immunostains were negative in the globules. A diagnosis of choroid plexus carcinoma with hyaline globules (WHO grade III) was rendered.