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

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.

SUBMITTER: Tavallaii A

PROVIDER: S-EPMC7712806 | biostudies-literature | 2020 Jan-Dec

REPOSITORIES: biostudies-literature

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

Tavallaii Amin A Keykhosravi Ehsan E Rezaee Hamid H Kianbakht Camellia C

Neuro-oncology advances 20200101 1

<h4>Background</h4>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.<h4>Methods</h4>A ...[more]

PMID: 33305270

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Project description:Atypical choroid plexus papilloma (APP) represents a novel intermediate-grade subtype of choroid plexus tumor (CPT), the clinical outcome of which has not been described yet. We present the first analysis of a group of APP patients enrolled in the ongoing CPT-SIOP-2000 study of CPTs. A worldwide registration and a randomized trial for those patients who require chemotherapy started in 2000. For APP, maximal surgical resection was recommended. After surgery, patients who had undergone complete resection were observed, whereas patients with incompletely resected or metastasized APP were treated with six chemotherapy courses (etoposide and vincristine, combined with either carboplatin or cyclophosphamide). Risk-adapted radiotherapy was given only to patients older than 3 years of age. Of the 106 patients with a centrally confirmed CPT histology, 30 had APP, 42 CPP and 34 CPC. APP patients were significantly younger (median = 0.7 years) than patients with CPP or CPC (both medians = 2.3 years). Complete resection was achieved in 68 (64%) patients (79% in CPP, 63% in APP, and 47% in CPC). Metastases were present at diagnosis in 17% of APP patients, 5% of CPP patients, and 21% of CPC patients. All nine APP patients who received postoperative chemotherapy showed an early response after two cycles: two had complete remission, four had partial response, and three had stable disease. In the observation group of 15 patients, one event was seen, and all patients were alive. In the treatment group, one patient with a metastasized tumor and incompletely resected APP died. While APP was defined histologically, median percentages of both the Ki-67/MIB-1 proliferation marker and the p53 tumor suppressor protein increased across the three histological subtypes (from CPP to APP and then CPC), suggesting that the subtypes comprise an ordinal categorization of increasingly severe CPT tumors. This ordering was reiterated by clinical outcome in the 92 patients treated per the study protocol, with 5-year EFS rates of 92% in 39 CPP patients, 83% in 24 APP patients, and 28% in 29 CPC patients. A similar ordering was seen when all 106 patients were evaluated for EFS. APP responded favorably to chemotherapy. The intermediate position of APP between CPP and CPC was supported by the clinical data.

Project description:BackgroundChoroid plexus papilloma represents 1-4% of pediatric brain tumors, mostly located in the ventricular atrium.[1] Intraventricular tumors represent a challenge due to the poor visualization of the surgical field and damage to surrounding structures.[2] Use of tubular retraction reduces cerebrovascular trauma to the surrounding parenchyma by distributing pressure uniformly, allowing less invasive corticotomy, and more stability on surgical corridors that allow the surgeon to use both hands and external visualization devices.[2-5].Case descriptionWe present the case of a 3-year-old boy with progressive headache, vomiting, and loss of control in the left hand for 3 months, with a history of ventricular shunt placement for acute obstructive hydrocephalus. The MRI revealed large lobulated lesion, which was hypointense on T1, hyperintense on T2, marked enhancement on T1 C+ (Gd) within the atrium of the right lateral ventricle, and spectroscopy with a peak of choline. Written consent for the use of photos and videos on this work was obtained from the patient's mother. A high-definition two-dimensional exoscope (VITOM® Karl Storz, Tuttlingen) was used during the surgical approach and throughout tumor removal, which was aided by ViewSite Brain Access System (VBAS®; Vycor Medical Inc.).[3] We performed a transparietal minimally invasive transsulcal parafascicular approach through the Frazier point for direct access to the ventricular atrium. Histological examination confirmed atypical choroid plexus papilloma. Postoperative imaging shows no residual tumor. The postoperative course was satisfactory with improvement of the headache and control of the left hand, leading to discharge home 1 week after surgery.ConclusionThe tubular transparietal minimally invasive approach obviates the need for traditional approaches to the atrium. This technique is safe and effective for the treatment of intraventricular and periventricular lesions, thus making this challenging target in more accessible to neurosurgeons, avoiding structure damage and any associated morbidity or mortality.

Project description:Choroid plexus papilloma (CPP) is a rare benign tumor of the central nervous system that is usually confined to the cerebral ventricles. 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). To the best of our knowledge, identification of these tumors mutations by next generation DNA sequencing (NGS) has not been yet reported. In the present study, NGS analysis of an a-CPP case was performed. Data were analyzed using Advaita Bioinformatics i-VariantGuide and Ion Reporter 5.6 programs. The results from NGS identified 12 novel missense mutations in the following genes: NOTCH1, ATM, STK36, MAGI1, DST, RECQL4, NUMA1, THBS1, MYH11, MALT1, SMARCA4 and CDH20. The PolyPhen score of six variants viz., DST, RECQL4, NUMA1, THBS1, MYHI1 and SMARCA4 were high, which suggested these variants represents pathogenic variants. Two novel insertions that caused frameshift were also found. Furthermore, two novel nonsense mutations and 14 novel intronic variants were identified in this tumor. The novel missense mutation detected in ATM gene was situated in c.5808A>T; p. (Leu1936Phe) in exon 39, and a known ATM mutation was in c.5948A>G; p. (Asn1983Ser). These novel mutations had not been reported in previous database. Subsequently, the quality statistics of these variants, including allele coverage, allele ratio, P-value, Phred quality score, sequencing coverage, PolyPhen score and alleles frequency was performed. For all variants, P-value was highly significant and the Phred quality score was high. In addition, the results from sequencing coverage demonstrated that 97.02% reads were on target and that 97.88% amplicons had at least 500 reads. These findings may serve at determining new strategies to distinguish the types of choroid plexus tumor, and at developing novel targeted therapies. Development of NGS technologies in the Kingdom of Saudi Arabia may be used in molecular pathology laboratories.

Dataset Information

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

Publications

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

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