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 upon outcome measures of these patients. We also compared these effects upon totally and partially resected tumors and pediatric and adult populations.
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“…Furthermore, particularly in the context of LFS, second malignancies remain a concern. A recent literature review was inconclusive with respect to specific indications for chemotherapy and radiotherapy [13]. Establishing a treatment algorithm as guidance was a major objective of the CPT-SIOP-2000 study, and the algorithm developed was widely followed in the international pediatric neuro-oncology community.…”
Section: Discussionmentioning
confidence: 99%
“…Furthermore, particularly in the context of LFS, second malignancies remain a concern. A recent literature review was inconclusive with respect to specific indications for chemotherapy and radiotherapy [ 13 ].…”
Section: Discussionmentioning
confidence: 99%
“…Treatment recommendations for CPT include multidisciplinary approaches, with maximal surgical resection for all CPT [8][9][10][11][12][13][14], followed by chemotherapy [11,[15][16][17][18][19][20][21] and radiotherapy [22][23][24] for high-risk CPT. The prognosis of CPC remains dismal when tumor resection is the only treatment modality, and the role, sequence, and intensity of primary chemotherapy remain debatable [13,19,21].…”
Introduction
Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established.
Methods
CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled.
Results
For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators.
Conclusions
Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.
“…Furthermore, particularly in the context of LFS, second malignancies remain a concern. A recent literature review was inconclusive with respect to specific indications for chemotherapy and radiotherapy [13]. Establishing a treatment algorithm as guidance was a major objective of the CPT-SIOP-2000 study, and the algorithm developed was widely followed in the international pediatric neuro-oncology community.…”
Section: Discussionmentioning
confidence: 99%
“…Furthermore, particularly in the context of LFS, second malignancies remain a concern. A recent literature review was inconclusive with respect to specific indications for chemotherapy and radiotherapy [ 13 ].…”
Section: Discussionmentioning
confidence: 99%
“…Treatment recommendations for CPT include multidisciplinary approaches, with maximal surgical resection for all CPT [8][9][10][11][12][13][14], followed by chemotherapy [11,[15][16][17][18][19][20][21] and radiotherapy [22][23][24] for high-risk CPT. The prognosis of CPC remains dismal when tumor resection is the only treatment modality, and the role, sequence, and intensity of primary chemotherapy remain debatable [13,19,21].…”
Introduction
Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established.
Methods
CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled.
Results
For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators.
Conclusions
Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.
“…Chemotherapy, particularly in cases of CPC, has been reported to increase overall survival. [ 18 ] Although data regarding specific dosing regimens is largely lacking, etoposide, vincristine, cisplatin, cyclophosphamide, carboplatin, or ifosfamide are most commonly utilized. In a randomized trial, Wolff et al .…”
Background:
Choroid plexus papillomas (CPPs) are rare neoplasms arising from choroid plexus epithelium representing <1% of all intracranial tumors. Symptoms vary based on location and regional mass effect; however, hydrocephalus is common due to cerebrospinal fluid flow obstruction and/or overproduction. Distant site metastasis or de novo formation in extraventricular sites is rare.
Case Description:
A 57-year-old female with a history of a 4th ventricular CPP status post resection in 2004 and 2018 with subsequent gamma knife therapy in 2019 presented with increased thirst and urination. Since her initial surgery, she has experienced significant gait imbalance, diplopia, dysphagia, and right-sided hemiparesis and hemisensory loss. Magnetic resonance imaging revealed a new 1.5 × 1.8 cm suprasellar lesion. She underwent a left supraorbital craniotomy for tumor resection, with pathology revealing metastatic World Health Organization grade II CPP.
Conclusion:
Extraventricular manifestation of CPP is rare. De novo or metastatic involvement of the sella has seldom been reported. Treatment should target gross total surgical resection. Adjuvant chemotherapy and radiation may be useful in higher-grade lesions.
“…Previously published survival rates for perinatal atypical choroid plexus papilloma is 67%, which is intermediate between 81% for choroid plexus papilloma and 45% for choroid plexus carcinoma [ 2 ]. If patients survive the neonatal period, the treatment regimen for this tumor typically involves surgical resection, and when indicated and feasible, may also include adjuvant or neoadjuvant chemotherapy, and/or preoperative embolization, to reduce tumor size and vascularity [ 13 , 14 ]. Radiation is not typically used in the neonatal population due to long-term deleterious effects on developing tissue [ 2 ].…”
We present a rare prenatal diagnosis of bilateral choroid plexus papillomas by obstetrical ultrasound and fetal MRI at 20 weeks 6 days gestation. The fetus demonstrated bilateral enlarged, echogenic choroid plexus with increased Doppler flow suggestive of vascularized choroid tissue. Same-day fetal MRI demonstrated that the choroid plexus appeared enlarged bilaterally without definite hemorrhage. The combined features on ultrasound and MRI suggested bilateral choroid plexus papillomas with increased cerebrospinal fluid production, leading to ventriculomegaly and enlarged extra-axial spaces. The diagnosis was confirmed by postnatal pathology, which demonstrated WHO grade II atypical choroid plexus papillomas.
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