Choroid Plexus Carcinoma

Malignant neoplasms originating from the CHOROID PLEXUS.They are neuroectodermal in origin and can range from benign choroid plexus papillomas (CPPs) to malignant choroid carcinomas (CPCs). These rare tumors generally occur in childhood, but have also been reported in adults. Patients typically present with signs and symptoms of increased intracranial pressure including headache, HYDROCEPHALUS; PAPILLEDEMA, nausea, vomiting, cranial nerve deficits, gait impairment, and seizures. Tumor cells show anaplastic features and often invade neighboring brain structures. Cerebrospinal fluid metastases are common. Germline mutations in the TP53 gene have been identified. OMIM: 260500

Networked: 150 relevant articles (2 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Choroid Plexus Papilloma
3.	Ependymoma
4.	Glioma (Gliomas)
5.	Carcinoma (Carcinomatosis)

Experts

1.	Hasselblatt, Martin: 5 articles (05/2020 - 01/2006)
2.	Gajjar, Amar: 4 articles (01/2021 - 07/2009)
3.	Bouffet, Eric: 4 articles (01/2019 - 06/2010)
4.	Gilbertson, Richard J: 4 articles (01/2019 - 01/2016)
5.	Dhall, Girish: 3 articles (05/2020 - 05/2015)
6.	Finlay, Jonathan L: 3 articles (01/2019 - 07/2009)
7.	Roussel, Martine F: 3 articles (01/2019 - 01/2016)
8.	Tabori, Uri: 3 articles (01/2019 - 06/2010)
9.	Wolff, Johannes: 3 articles (11/2017 - 05/2015)
10.	Ivan, Michael E: 3 articles (11/2014 - 01/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Choroid Plexus Carcinoma:

1.	cytidylyl-(3'-5')-cytidine (CpC)IBA 01/01/2024 - "Of 18 CNST diagnoses, choroid plexus carcinoma (CPC) was the diagnosis for which most providers (78%) reported they would offer GT. " 12/30/2022 - "Histopathology was consistent with a disseminated, neuroinvasive choroid plexus carcinoma (CPC) that involved the entire neuroaxis including the meninges of the brain and spinal cord. " 04/01/2019 - "Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. " 01/01/2018 - "Here, we report an extremely rare TP53 missense variant, c.799C > T (p.Arg267Trp), identified in a 2-year-old Saudi proband diagnosed with choroid plexus carcinoma (CPC) and six of his first- and second-degree relatives. " 12/01/2017 - "The optimal treatment of patients with choroid plexus carcinoma (CPC) is unclear. "
2.	Methotrexate (Mexate)FDA LinkGeneric 02/01/2009 - "Methotrexate should not be combined with HDACI in the treatment of choroid plexus carcinoma." 01/01/2005 - "A 4-year-old female with choroid plexus carcinoma developed progressive disturbance of consciousness 2 years after postoperative treatment with radiotherapy, chemotherapy, and focal methotrexate injection into a residual tumor cyst. " 01/01/2003 - "Efficacy of sequential chemotherapy including methotrexate and doxorubicin in an infant with partially resected choroid plexus carcinoma."
3.	Doxorubicin (Adriamycin)FDA LinkGeneric 01/01/2003 - "Efficacy of sequential chemotherapy including methotrexate and doxorubicin in an infant with partially resected choroid plexus carcinoma."
4.	6-chloro-2-(1-piperazinyl)pyrazine (CPP)IBA 11/01/2019 - "According to the World Health Organization, CPP corresponds to a grade I atypical CPP (a-CPP); however, it can become more aggressive and reach grade II, which can rarely undergo malignant transformation into a choroid plexus carcinoma (grade III). " 05/01/2018 - "ADC and tumor volumes were retrospectively evaluated in 25 patients with choroid plexus papilloma (CPP; WHO grade 1 [n = 13]), atypical CPP (aCPP; grade 2 [n = 8]), or choroid plexus carcinoma (grade 3 [n = 4]) The prognostic roles of ADC and tumor volume were assessed. " 01/01/2019 - "Tumor types in order of frequency were choroid plexus papillomas (CPP, 17), primitive neuroectodermal tumor (12), atypical teratoid/rhabdoid tumor (10), high-grade glioma (9), optic glioma (9), ependymoma (8), low-grade glioma (6), pilocytic astrocytoma (6), choroid plexus carcinoma (5), and teratoma (5), with 11 miscellaneous tumors. " 01/06/2021 - "LFS has been associated with Choroid plexus carcinoma (CPC), but rarely with CPP as in our patient. " 12/20/2018 - "The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. "
5.	A-Form DNA (A-DNA)IBA 05/15/2008 - "In this study, we used two genetically engineered mouse carcinoma models, brain choroid plexus carcinoma (CPC) and prostate, to test the effect of inactivating gadd45a, a DNA damage response p53 target gene, on tumor responses to IR. "
6.	TATA-Box Binding Protein (TATA-Binding Protein)IBA 12/10/2022 - "TATA box-binding protein-associated factor 12 (TAF12) has been identified as an oncogene in choroid plexus carcinoma, but its role in glioma is poorly understood because of a lack of previous studies. "
7.	Glial Fibrillary Acidic ProteinIBA 07/01/1989 - "Both were nonreactive for glial fibrillary acidic protein, S-100, and carcinoembryonic antigen (CEA), unlike a previous study which reported that choroid plexus carcinoma, compared to papilloma, was uniquely S-100-negative and CEA-positive. "
8.	Factor XII (Hageman Factor)IBA 12/10/2022 - "TATA box-binding protein-associated factor 12 (TAF12) has been identified as an oncogene in choroid plexus carcinoma, but its role in glioma is poorly understood because of a lack of previous studies. "
9.	Carcinoembryonic AntigenIBA 07/01/1989 - "Both were nonreactive for glial fibrillary acidic protein, S-100, and carcinoembryonic antigen (CEA), unlike a previous study which reported that choroid plexus carcinoma, compared to papilloma, was uniquely S-100-negative and CEA-positive. "
10.	Proteins (Proteins, Gene)FDA Link 10/01/2009 - "Rare intraparenchymal choroid plexus carcinoma resembling atypical teratoid/rhabdoid tumor diagnosed by immunostaining for INI1 protein." 05/01/2005 - "INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma." 07/01/2020 - "Targeted sequencing of a choroid plexus carcinoma and an atypical choroid plexus papilloma, both with moderate-to-strong FGFR3 expression, revealed lack of protein-altering mutations or fusions in FGFR1 or FGFR3, but TP53 was altered in both tumors. " 03/26/2015 - "Freshly isolated mouse ependymoma, glioma and choroid plexus carcinoma cells expressing red fluorescence protein were conditioned to grow at 34 °C. " 01/01/2006 - "Protein expression of genes overexpressed in choroid plexus was evaluated in normal choroid plexus, choroid plexus papilloma, choroid plexus carcinoma, other primary brain tumors, and cerebral metastases. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2003 - "This result deserves further studies to assess the possible curative role of chemotherapy in very young patients suffering from choroid plexus carcinoma." 10/01/2002 - "[A study of usefulness of chemotherapy in long-term survival choroid plexus carcinoma case of childhood]." 03/14/2023 - "TRIPLE INTRAVENTRICULAR CHEMOTHERAPY FOR TREATMENT OF RELAPSED CHOROID PLEXUS CARCINOMA." 02/01/2022 - "Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. " 01/01/2021 - "Triple intraventricular chemotherapy for treatment of relapsed choroid plexus carcinoma."
2.	Radiotherapy 04/21/2021 - "In the absence of a global consensus on choroid plexus carcinoma management, our patient underwent a successful gross total resection and received postoperative radiotherapy. " 09/01/2010 - "The impact of radiotherapy fields in the treatment of patients with choroid plexus carcinoma." 08/01/2001 - "An adult patient with choroid plexus carcinoma who was treated with an incomplete surgical resection and postoperative radiotherapy is reported. " 06/19/1999 - "Radiation therapy and survival in choroid plexus carcinoma." 04/01/2015 - "Response to combined radiotherapy and chemotherapy of a leptomeningeal spread from choroid plexus carcinoma: case report."
3.	Therapeutics 01/01/2021 - "Outcome and molecular analysis of young children with choroid plexus carcinoma treated with non-myeloablative therapy: results from the SJYC07 trial." 12/01/2017 - "Effect of Surgery, Adjuvant Therapy, and Other Prognostic Factors on Choroid Plexus Carcinoma: A Systematic Review and Individual Patient Data Analysis." 01/01/2017 - "Molecular Guided Therapy Provides Sustained Clinical Response in Refractory Choroid Plexus Carcinoma." 04/01/2002 - "Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected?" 03/01/1995 - "[Therapy of choroid plexus carcinoma in childhood. "
4.	Craniotomy 05/23/2021 - "Surgery was performed via left parietal craniotomy, and the lesion was histologically confirmed to be choroid plexus carcinoma. " 10/01/1999 - "CT scan and craniotomy demonstrated an intraventricular papillary tumor with histologic and immunohistochemical features indicative of a choroid plexus carcinoma. "
5.	Craniospinal Irradiation 12/01/2017 - "Here, we report on a child with LFS who developed genomic instability during craniospinal irradiation for metastatic choroid plexus carcinoma (CPC). "