scholarly journals Rare Brain Tumors with Infrequent Clinical Manifestations: Illustrative Cases

2021 ◽  
Author(s):  
José Luis Navarro-Olvera ◽  
Armando Armas-Salazar ◽  
José Damián Carrillo-Ruiz ◽  
Jesús Q. Beltrán ◽  
Gustavo Parra-Romero ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Brain Tumors ◽  
Choroid Plexus Papilloma ◽  
Clinical Manifestations ◽  
Intracranial Tumors ◽  
Clinical Syndromes ◽  
Rosai Dorfman Disease ◽  
Neuroenteric Cyst ◽  
Histological Characteristics ◽  
Plexus Papilloma

This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.

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

2013 ◽  
Vol 12 (1) ◽  
pp. 13-20 ◽  
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.

scholarly journals Experience of choroid plexus papilloma in children at Mansoura University Hospital

2019 ◽  
pp. 144-149
Author(s):  
Hatem Badr ◽  
Ahmad Zaher ◽  
Mohamed State ◽  
Amr Farid Khalil
Keyword(s):  
Choroid Plexus ◽  
Lateral Ventricle ◽  
Choroid Plexus Papilloma ◽  
Clinical Manifestations ◽  
University Hospital ◽  
Complete Relief ◽  
University Hospitals ◽  
Excellent Outcome ◽  
Csf Leakage ◽  
Plexus Papilloma

Choroid plexus papilloma (CPP) are rare, benign tumours of neuroectodermal origin; they represent 1-3% of central nervous system (CNS) tumours in paediatric patients. Authors present their experience in the management of such lesion in Mansoura University Hospitals.Methods. For children with Choroid plexus papilloma who were treated via microsurgical excision over 4 years from January 2012 to January 2016 in Mansoura University Hospitals, a retrospective analysis was done for age, sex, clinical manifestations, surgical treatment and follow up.Results. Twenty-three paediatrics were treated over 4 years. Age ranged from (7 months - 8 years). There were 13 female and10 males. Tumours were located in the lateral ventricle in all cases. Features of raised intracranial pressure were the predominant presentation. Total microsurgical excision was achieved in all cases. All cases had intraoperative blood loss < 100 ml with mean Haematocrit 28%. Follow up period (7-53 months). Complete relief of all symptoms was reported in all cases with no mortality. One of our patients needs postoperative ventriculoperitoneal shunt for persistent CSF leakage and another one required subdural peritoneal shunt for persistent subdural CSF collection.Conclusion. Total excision of Choroid plexus papilloma is usually the rule with an excellent outcome. Routine external ventricular drainage for at least 3 days is effective in lowering shunt-dependent cases as it allows the release of bloody CSF and small tumour residue. With proper microsurgical technique through superior parietal lobule to access lateral ventricle then tumour coagulation at the same time of irrigation that helps the tumour to shrink and thus, decrease the incidence of bleeding. The tumour vascular pedicle should be resected with the last part of the tumour to avoid pedicle retraction that may lead to ventricular haemorrhage.

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

2021 ◽  
Vol 20 (4) ◽  
pp. 33-41
Author(s):  
A. F. Valiakhmetova ◽  
L. I. Papusha ◽  
A. V. Sanakoeva ◽  
L. V. Shishkina ◽  
O. I. Budanov ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Choroid Plexus ◽  
Tumor Resection ◽  
Choroid Plexus Papilloma ◽  
Radical Resection ◽  
World Health ◽  
Surgical Removal ◽  
Who Grade ◽  
Atypical Choroid Plexus Papilloma ◽  
Plexus Papilloma

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. 

scholarly journals PEDT-02 Clinical usage of NCC Oncopanel/FoundationOne CDx for pediatric/AYA patients with recurrent malignant brain tumors

2020 ◽  
Vol 2 (Supplement_3) ◽  
pp. ii9-ii9
Author(s):  
Ichiyo Shibahara ◽  
Toshihiro Kumabe ◽  
Takuichiro Hide ◽  
Ryota Sasao ◽  
Hikaru Sasaki ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Effective Treatment ◽  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Braf Inhibitor ◽  
Diffuse Astrocytoma ◽  
Anaplastic Meningioma ◽  
Malignant Brain Tumors ◽  
Epithelioid Glioblastoma ◽  
Plexus Papilloma

Abstract Backgrounds: Analyses of somatic mutations in malignant brain tumors have been used to make effective treatment strategies. NCC Oncopanel and FoundationOne CDx are custom targeted next-generation sequencing (NGS) panels. The cost for this analysis is 560,000 yen covered by National Health Insurance in Japan since June 2019. These methods can be applied for the solid cancers with no established therapies and relapsed after the standard therapies. Following these inclusion criteria, most malignant brain tumors, especially recurrent malignant brain tumors in pediatric/AYA generations, can be included. Object: To report the results of our initial experiences. Methods: In the last one year, we utilized these NGS panels for five patients with recurrent malignant brain tumors in this generations: 2 epithelioid glioblastomas; 1 anaplastic meningioma; 1 diffuse astrocytoma (gliomatosis cerebri); 1 atypical choroid plexus papilloma. Results: Final recommended treatments are as follows: BRAF/MEK inhibitors, bevacizumab, or anti-PD-1 antibody for one epithelioid glioblastoma; MEK inhibitor for another epithelioid glioblastoma previously treated by BRAF inhibitor and bevacizumab; ERK1/2 inhibitors for anaplastic meningioma. The diffuse astrocytoma had IDHR132H mutation. There was no clinical trial using IDH inhibitor for recurrent diffuse astrocytoma; thus, the final recommendation for this case was rechallenge of temozolomide. To date, only one NGS for a choroid plexus papilloma has been reported (Arch Pathol Lab Med, 2017). Our case had multiple actionable gene alterations, including TERT mutation and amplification of various genes. Unfortunately, there was no druggable gene alteration among them. Conclusions: Insurance-covered cancer gene panel tests could represent effective treatment options for some malignant brain tumors in pediatric/AYA generations. If the relapse is local and can be treated by repeat resections, we think the surgery is the first-line choice. But, in another situation, information from NGS panels should be obtained positively. Efforts to increase the utility of off-label use of drugs are encouraged.

Clinical Manifestations and Radiological Characteristics on Rosai-Dorfman Disease: A Retrospective Observational Study

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Blood Vessels ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Ct Images ◽  
High Signal ◽  
Radiological Features ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
Heterogeneous Enhancement ◽  
The Masses

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.

MR Findings of Choroid Plexus Papilloma: Case Report

1994 ◽  
Vol 30 (4) ◽  
pp. 643
Author(s):  
Joo Hyeong Oh ◽  
Tae Hoon Kim ◽  
Woo Suk Choi
Keyword(s):  
Case Report ◽  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Plexus Papilloma

scholarly journals BIOM-62 SENSITIVE DETECTION AND DISCRIMINATION OF INTRACRANIAL TUMORS BY BLOOD

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii15-ii15
Author(s):  
Farshad Nassiri ◽  
Ankur Chakravarthy ◽  
Shengrui Feng ◽  
Roxana Shen ◽  
Romina Nejad ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Brain Tumors ◽  
Model Performance ◽  
High Sensitivity ◽  
Circulating Tumor Dna ◽  
Low Grade ◽  
Learning Approaches ◽  
Intracranial Tumors ◽  
Cell Of Origin ◽  
Tumor Dna

Abstract BACKGROUND The diagnosis of intracranial tumors relies on tissue specimens obtained by invasive surgery. Non-invasive diagnostic approaches, particularly for patients with brain tumours, provide an opportunity to avoid surgery and mitigate unnecessary risk to patients. We reasoned that DNA methylation profiles of circulating tumor DNA in blood can be used as a clinically useful biomarker for patients with brain tumors, given the specificity of DNA methylation profiles for cell-of-origin. METHODS We generated methylation profiles on the plasma of 608 patients with cancer (219 intracranial, 388 extracranial) and 60 healthy controls using a cell-free methylated DNA immunoprecipitation combined with deep sequencing (cfMeDIP-seq) approach. Using machine-learning approaches we generated and evaluated models to distinguish brain tumors from extracranial cancers that may metastasize to the brain, as well as additional models to discriminate common brain tumors included in the differential diagnosis of solitary extra-axial and intra-axial tumors. RESULTS We observed high sensitivity and discriminative capacity for our models to distinguish gliomas from other cancerous and healthy patients (AUC=0.99, 95%CI 0.96–1), with similar performance in IDH mutant and wildtype gliomas as well as in lower- and high-grade gliomas. Excluding non-malignant contributors to plasma methylation did not change model performance (AUC=0.982, 95%CI 0.93–1). Models generated to discriminate intracranial tumors from each other also demonstrated high accuracy for common extra-axial tumors (AUCmeningioma=0.89, 95%CI 0.80–0.97; AUChemangiopericytoma=0.95, 95%CI 0.73–1) as well as intra-axial tumors ranging from low-grade indolent glial-neuronal tumors (AUC 0.93, 95%CI 0.80 – 1) to diffuse intra-axial gliomas with distinct molecular composition (AUCIDH-mutant glioma = 0.82, 95%CI 0.66 -0.98; AUCIDH-wildtype-glioma = 0.71, 95%CI 0.53 – 0.9). Plasma cfMeDIP-seq signals originated from corresponding tumor tissue DNA methylation signals (r=0.37, p&lt; 2.2e-16). CONCLUSIONS These results demonstrate the potential for cfMeDIP-seq profiles to not only detect circulating tumor DNA, but to accurately discriminate common intracranial tumors that share cell-of-origin lineages.

Spinal deposits of a benign choroid plexus papilloma

2008 ◽  
Vol 15 (6) ◽  
pp. 708-712 ◽  
Author(s):  
H. Akil ◽  
N.J. Coupe ◽  
J. Singh
Keyword(s):  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Plexus Papilloma
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