Choroid plexus papilloma and atypical choroid plexus papilloma in children: treatment results

Choroid plexus tumors (CPTs) are rare intraventricular tumors, accounting 2–5% of brain tumors in children. Choroid plexus papilloma (CPP) classified by the World Health Organization (WHO) on the basis of histological criteria as benign tumor (WHO grade I), CPPs accounts for 10–20% of brain tumors during the first year of life, the median age of patients with CPP at the time of diagnosis – 3.5 years. Atypical choroid plexus papilloma (APP) is an intermediate (WHO grade II) tumor which was officially included in the WHO classification in 2007. Median age 0,63 years. In this work, for the first time in the Russian literature, we present the outcomes of CPP and APP therapy in a large cohort of patients. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. In our study from January 1, 2009 to December 31, 2019, 83 CPP and 37 APP were histologically verified. For all patients, surgical removal of the tumor was the first stage of treatment. In 79 (95.2%) patients with CPP and in 32 (86.5%) children with APP, radical resection was performed at the first stage. In 73.5% of children with CPP and 62.2% of children with APP there was no intra- and postoperative complications. In both CPP and APP, the 5-year EFS in patients with complete resection was statistically significantly higher compared to subtotal tumor resection (100% and 33 ± 27% in CPP, 94 ± 4% and 25 ± 22% for APP) (p < 0.001). Chemoradiation therapy was given to 1 patient with CPP, 4 patients with APP with tumor recurrence/progression.

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Immunohistochemical Analysis of Cerebral Intraparenchymal Choroid Plexus Tumor: Case Report

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0037-1615284 ◽

2018 ◽

Vol 80 (01) ◽

pp. 053-057

Author(s):

Yutaka Sawamura ◽

Shinya Tanaka ◽

Takamitsu Fujimaki ◽

Shigehisa Hirose ◽

Toshimitsu Aida ◽

...

Keyword(s):

Choroid Plexus ◽

Tumor Resection ◽

Choroid Plexus Papilloma ◽

Brain Magnetic Resonance Imaging ◽

Unknown Origin ◽

Immunohistochemical Analysis ◽

Cystic Mass ◽

Choroid Plexus Tumor ◽

Atypical Choroid Plexus Papilloma ◽

Plexus Papilloma

Background It is very rare for a choroid plexus tumor to occur intraparenchymally in the absence of a relation to the choroid plexus. Clinical Presentation A case of cerebral intraparenchymal choroid plexus tumor in a 30-year-old woman presenting with left hemiparesis is described. Brain magnetic resonance imaging depicted a large cystic mass in the right frontal lobe. Tumor resection was performed by right frontal craniotomy. No connection with the choroid plexus was observed during the operation. Histologically, the tumor exhibited a glandular structure with a papillary pattern suggesting a neoplasm of epithelial origin. Immunohistochemical analyses revealed the tumor as an atypical choroid plexus papilloma. Conclusion Immunohistochemical findings, especially regarding Kir7.1, are very important for the differential diagnosis of cerebral intraparenchymal choroid plexus tumors from metastatic tumors. The present case reveals that an atypical choroid plexus papilloma can occur intraparenchymally without an association with the choroid plexus. Intraparenchymal atypical choroid plexus papillomas may have previously been diagnosed incorrectly as metastatic adenocarcinomas of unknown origin.

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Role of available adjuvant therapies following surgical resection of atypical choroid plexus papilloma—a systematic review and pooled analysis

Neuro-Oncology Advances ◽

10.1093/noajnl/vdaa139 ◽

2020 ◽

Vol 2 (1) ◽

Author(s):

Amin Tavallaii ◽

Ehsan Keykhosravi ◽

Hamid Rezaee ◽

Camellia Kianbakht

Keyword(s):

Systematic Review ◽

Adjuvant Therapy ◽

Choroid Plexus ◽

Outcome Measures ◽

Tumor Resection ◽

Choroid Plexus Papilloma ◽

Pooled Analysis ◽

Adjuvant Therapies ◽

Atypical Choroid Plexus Papilloma ◽

Plexus Papilloma

Abstract Background Atypical choroid plexus papilloma is a recently introduced entity with intermediate pathological characteristics. These tumors are relatively rare and the optimal management of these tumors is a matter of debate. Therefore, we performed a systematic review and pooled analysis about the effects of adjuvant therapies on outcome measures of these patients. We also compared these effects on totally and partially resected tumors and pediatric and adult populations. Methods A systematic search of 3 databases based on inclusion/exclusion criteria was performed. Data extraction was separately performed by 2 authors, and the summarized data were presented in the form of tables. Pooled estimates of different outcome measures were calculated for each adjuvant therapy and presented separately for studies with pediatric, adult, or mixed populations. Results A review of 14 included studies consisting of 144 patients revealed the effect of adjuvant treatment on reduction of tumor recurrence, metastasis, and reoperation rates and increasing survival rates in patients with subtotal tumor resection. This advantage was not seen in the case of gross total tumor resection. Almost all outcome measures were more favorable in the pediatric population. Conclusions It can be concluded that whenever gross total resection is not feasible, the implementation of adjuvant therapy can improve the outcome and prognosis. In other cases, it should be decided on an individual basis. Also, more aggressive behavior and higher rates of recurrence and mortality in the adult population suggest the consideration of more aggressive adjuvant treatments for adult patients.

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Grade II atypical choroid plexus papilloma with normal karyotype

Child s Nervous System ◽

10.1007/s00381-009-0938-z ◽

2009 ◽

Vol 25 (12) ◽

pp. 1623-1626 ◽

Cited By ~ 3

Author(s):

María Sol Brassesco ◽

Elvis Terci Valera ◽

Aline Paixão Becker ◽

Ricardo Santos Oliveira ◽

Carlos Alberto Scrideli ◽

...

Keyword(s):

Choroid Plexus ◽

Choroid Plexus Papilloma ◽

Normal Karyotype ◽

Atypical Choroid Plexus Papilloma ◽

Grade Ii ◽

Plexus Papilloma

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Ossified choroid plexus papilloma of the fourth ventricle: elucidation of the mechanism of osteogenesis in benign brain tumors

Journal of Neurosurgery Pediatrics ◽

10.3171/2013.3.peds12400 ◽

2013 ◽

Vol 12 (1) ◽

pp. 13-20 ◽

Cited By ~ 3

Author(s):

Sunil Manjila ◽

Erin Miller ◽

Amad Awadallah ◽

Shunichi Murakami ◽

Mark L. Cohen ◽

...

Keyword(s):

Brain Tumors ◽

Bone Formation ◽

Choroid Plexus ◽

Fourth Ventricle ◽

Choroid Plexus Papilloma ◽

Immunohistochemical Analysis ◽

Pluripotent Cell ◽

Bone Trabeculae ◽

Plexus Papilloma ◽

Mature Bone

True ossification within benign brain tumors is rare, and the molecular mechanism for this process is poorly understood. The authors report a case of ossified choroid plexus papilloma (CPP) and analyze it to help elucidate the underlying molecular basis of osteogenesis in benign brain tumors. A 21-year-old man presented with headache and depression that progressed over years. Computed tomography, MRI, and angiography demonstrated a large heavily calcified fourth ventricular tumor with a vascular blush and no hydrocephalus. The tumor was resected and was found to be an ossified CPP. Immunohistochemical staining for VEGF, Sox2, BMP-2, osterix, osteopontin, and osteocalcin was performed in an attempt to elucidate the mechanism of bone formation. The tumor was extensively ossified with mature bone trabeculae. Immunostaining for VEGF was positive. Additional staining showed the presence of osteocalcin in this ossified tumor but not in samples of nonossified CPPs collected from other patients. Staining for osterix and osteopontin was equivocally positive in the ossified CPP but also in the nonossified CPPs examined. The presence of osteocalcin in the ossified CPP demonstrates that there is true bone formation rather than simple calcification. Its appearance within cells around the trabeculae suggests the presence of osteoblasts. The presence of osterix suggests that a pluripotent cell, or one that is already partially differentiated, may be differentiated into an osteoblast through this pathway. This represents the first systematic immunohistochemical analysis of osteogenesis within choroid plexus tumors.

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