scholarly journals Choroid Plexus Papilloma with Dandy Walker Variant: Co-existence or Association- A Case Report

2019 ◽  
Vol 46 (2) ◽  
pp. 61-65
Author(s):  
Mohammad Hossain ◽  
Nazmin Ahmed ◽  
Narendra Shalike ◽  
Md Rokibul Islam ◽  
Soumen Samadder ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Communicating Hydrocephalus ◽  
Intermediate Form ◽  
Genetic Syndromes ◽  
Who Grade ◽  
Choroid Plexus Tumors ◽  
Well Differentiated ◽  
Central Nervous Sytem ◽  
Plexus Papilloma

Choroid plexus tumors are rare intracranial tumors which account for 0.4-0.6% of all brain tumors. Choroid plexus tumors represent a spectrum of neoplasms derived from papillary epithelium of normal choroid plexus, including well-differentiated papilloma (WHO grade I), intermediate form as atypical Choroid Plexus Papilloma (WHO grade II) and highly aggressive choroid plexus carcinomas (WHO grade III). Though rare, it is responsible for the communicating hydrocephalus in children due to overproduction of cerebrospinal fluid. Due to advances in molecular biology and better understanding of the tumorigenesis of choroid plexus papilloma, now it is established that several genetic syndromes and central nervous sytem abnormalities are associated with this tumor. Here, we reported a case of a 10 months old child who presented with sudden deterioration of consciousness level and after thorough evaluation, diagnosed as a case of Choroid Plexus Papilloma with Dandy Walker Variant. Till date, this is the first reported case of the association/ co-existence of such two conditions which needs further evaluation. Bangladesh Med J. 2017 May; 46 (2): 61-65

scholarly journals RARE-26. RETROSPECTIVE ANALYSIS OF PEDIATRIC CHOROID PLEXUS TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii447-iii448
Author(s):  
Yoshiko Nakano ◽  
Atsufumi Kawamura ◽  
Yuko Watanabe ◽  
Ryuta Saito ◽  
Masayuki Kanemori ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Survival Rates ◽  
Choroid Plexus Papilloma ◽  
Clinical Information ◽  
Local Relapse ◽  
Current Status ◽  
Choroid Plexus Tumors ◽  
Li Fraumeni Syndrome ◽  
Plexus Papilloma

Abstract BACKGROUND Choroid plexus tumors (CPT) include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Because of their rarity, limited data are available on the current status of treatment and outcomes for pediatric CPTs. METHOD We retrospectively reviewed clinical information on patients with CPT patients aged between 0 and 30 years at diagnosis and were treated in 8 institutions in Japan. RESULTS Of forty-two cases initially diagnosed as CPT, 18 cases were reviewed by central pathologists. As a result, the diagnosis of CPC or aCPP in five cases were changed to other tumors including AT/RT and astroblastoma. The remaining 37 cases were subjected to analysis. Median age at diagnosis was two years (0 to 25) and the mean follow-up period was seven years. All 26 patients with CPP (n=20) or aCPP (n=6) underwent gross-total resection without adjuvant therapy. Of them 24 patients are alive without recurrence. Four patients of patients with CPC (n=11) died of cancer. Five patients including three patients experienced local relapse, achieved complete remission after resection of tumor plus chemoradiotherapy. All three patients with dissemination of CPC at diagnosis or relapse died of the disease. At least three patients were diagnosed with Li-Fraumeni syndrome: one died of medulloblastoma and one patient developed osteosarcoma. CONCLUSION Compared with the excellent prognosis of CPP, the survival rates for CPC, especially disseminated CPC are unsatisfactory. Our results also underline the importance of considering genetic testing of TP53 for patients with CPC.

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 Choroid Plexus Papilloma in a Dog

1971 ◽  
Vol 8 (1) ◽  
pp. 91-95 ◽  
Author(s):  
H. J. Kurtz ◽  
Griselda F. Hanlon
Keyword(s):  
Choroid Plexus ◽  
Fourth Ventricle ◽  
Choroid Plexus Papilloma ◽  
Clinical Signs ◽  
Single Layer ◽  
Ciliated Epithelium ◽  
The Central Nervous System ◽  
Well Differentiated ◽  
The Right ◽  
Plexus Papilloma

A 6-year-old female Cocker Spaniel with a choroid plexus papilloma had clinical signs of disease of the central nervous system for 1 month. The neoplasm originated from the right choroid plexus of the fourth ventricle and had displaced contiguous parts of the medulla oblongata and cerebellum. It was well differentiated and was composed of papillae arranged in an arboriform pattern. The papillae were lined by a single layer of non ciliated epithelium.

scholarly journals RARE-18. GENETIC EVALUATION IN PATIENTS WITH CHOROID PLEXUS TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii446-iii446
Author(s):  
Milena Oliveira ◽  
Nasjla Silva ◽  
Andrea Cappellano ◽  
Daniela Almeida ◽  
Sergio Cavalheiro ◽  
...  
Keyword(s):  
Family History ◽  
Choroid Plexus ◽  
P53 Protein ◽  
Positive Family History ◽  
Choroid Plexus Papilloma ◽  
Tp53 Gene ◽  
Genetic Evaluation ◽  
Choroid Plexus Tumors ◽  
Li Fraumeni Syndrome ◽  
Plexus Papilloma

Abstract INTRODUCTION Choroid plexus tumors (CPT) are rare intraventricular neoplasms of epithelial origin. They usually occur in the 2nd year of life, corresponding to 0.4–0.6% of intracranial tumors in this age group. They are sub classified, according to WHO 2016, in choroid plexus carcinoma (CPC), atypical choroid plexus papilloma (ACPP) and choroid plexus papilloma (CPP). Li-Fraumeni syndrome (LFS) is present in 50% of patients with CPC. In Brazil, the TP53 p.R337H mutation affects 0.3% of the population in the South/Southeast. OBJECTIVE Evaluate the incidence of genetic mutations in patients with choroid plexus tumors and therefore the importance of genetic evaluation. PATIENTS AND METHODS Between 1992–2019, 38 patients were diagnosed with CPT in our institution, 23 with CPC. From 2012, 21 patients were referred for genetic evaluation, 16 of which had CPC (2 had previously CPP). Positive family history for neoplasms was present in 87.5%; 37.5% compatible with LFS, 50% of them with mutations. All the patients with positive, but unspecific, family history of neoplasms, had pathogenic mutation. The molecular investigation of the TP53 gene in patients with CPC was performed and positive in 56.2%: R337H (5 patients), R110C, R158H, H179R, R196* (1 patient each). Of those with R337H, p53 protein immunohistochemistry resulted in 90–100% positivity. One of the patients with CPP that evolved to CCP had the H179R mutation. Clinical course was similar among them, and with those without mutations. CONCLUSION These results confirm the need for genetic evaluation in patients with choroid plexus tumors for adequate therapeutic management and long-term follow-up.

Ultrastructural evidence for CSF production by a choroid plexus papilloma

1976 ◽  
Vol 45 (4) ◽  
pp. 409-415 ◽  
Author(s):  
Nitya R. Ghatak ◽  
Joe M. McWhorter
Keyword(s):  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Communicating Hydrocephalus ◽  
Content Type ◽  
Structural Details ◽  
Fluid Production ◽  
Pinocytotic Vesicles ◽  
Fenestrated Endothelium ◽  
Plexus Papilloma ◽  
Tubular Bodies

✓ A choroid plexus papilloma (CPP) removed from a child with communicating hydrocephalus was studied with the electron microscope. In addition to the usual organelles, the neoplastic epithelial cells contained polypoid microvilli, cilia, coated pinocytotic vesicles, and apical tight junctions. The basal surfaces were consistently juxtaposed to large capillaries lined by fenestrated endothelium. The fine structural details of the tumor were essentially the same as those of normal CP and thus appeared ideally suited for an active secretory function. Two additional features in this tumor were tubular bodies in the endothelium and well developed pericytes. The present findings provide strong evidence for cerebrospinal fluid production by CPP, a phenomenon that has long been suggested by clinical observation.

Choroid plexus adenoma in a child: expanding the clinical and pathological spectrum

2018 ◽  
Vol 21 (4) ◽  
pp. 428-433 ◽  
Author(s):  
Nicole Prendergast ◽  
Jeffrey D. Goldstein ◽  
Alexandra D. Beier
Keyword(s):  
Differential Diagnosis ◽  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Choroid Plexus Tumors ◽  
Plexus Papilloma

Primary choroid plexus tumors encompass a variety of tumors, with choroid plexus papilloma and carcinoma being the most common. Also in the differential diagnosis is the rare benign choroid plexus adenoma. As these tumors are infrequently described, the histological profile continues to evolve. The authors present a case with unusual characteristics that will broaden the pathological spectrum for choroid plexus adenomas.

Choroid plexus papilloma in a free ranging eared dove (Zenaida auriculata)

2021 ◽  
Vol 14 (3) ◽  
pp. 188-190
Author(s):  
Pedro Navas-Suárez ◽  
◽  
Ticiana Ervedosa ◽  
Celso di Loreto ◽  
Rodrigo Ressio ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Single Layer ◽  
Domestic Animals ◽  
Choroid Plexus Carcinoma ◽  
Wild Animals ◽  
Neoplastic Cells ◽  
Well Differentiated ◽  
Free Ranging ◽  
Plexus Papilloma

Choroid plexus tumors (CPT) are rare neoplasms and histologically classified as choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP), and choroid plexus carcinoma (CPC). These neoplasms have been described in humans, domestic animals (canine, feline, equine, caprine) and some wild animals (cetaceans and Psittacidae birds). To our best knowledge, herein we report the first CCP in a free-ranging eared dove (Columbiformes; Zenaida auriculata, de Murs 1847). Histologically, the ventricle was dilated, with a papillary proliferation (arboriform pattern) in topography of CP. The neoplasm was well-differentiated, composed by a single layer of cuboidal cells, anchored in a delicate fibrovascular stroma. The neoplastic cells exhibited moderate stroma, with well-defined borders and round nuclei, with vesicular chromatin and inconspicuous nucleoli. Mitotic activity was low (<1 mitosis per 10 high-power fields). Immunohistochemistry for cytokeratin markers (AE1/AE3 antibody) were implemented, however, both neoplastic cells and normal epithelial tissues do not show immunoreactivity.

Choroid plexus papilloma in a girl with hypomelanosis of Ito

2012 ◽  
Vol 10 (3) ◽  
pp. 182-185 ◽  
Author(s):  
Ryoma Morigaki ◽  
Kyong-Hon Pooh ◽  
Kenji Shouno ◽  
Hidekazu Taniguchi ◽  
Shouichi Endo ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
De Novo ◽  
Choroid Plexus Papilloma ◽  
Psychomotor Retardation ◽  
Chromosomal Mosaicism ◽  
Who Grade ◽  
Hypomelanosis Of Ito ◽  
Lines Of Blaschko ◽  
The Right ◽  
Plexus Papilloma

The authors report a case of choroid plexus papilloma in a girl with hypomelanosis of Ito, and they review the literature in brief. Hypomelanosis of Ito is a rare neurocutaneous syndrome characterized by cutaneous hypopigmented whorls, streaks, and patches along lines of Blaschko. Most patients exhibit CNS manifestations, including psychomotor retardation, seizures, hypotonia, and ataxia. A 6-year-old girl with hypomelanosis of Ito was referred to the authors' hospital with bilateral tumors in the lateral ventricles. The right lateral ventricle tumor was surgically removed. Immunohistochemical investigations revealed the tumor to be a choroid plexus papilloma (WHO Grade I). A chromosomal investigation revealed that the tumor tissue demonstrated a large loss of heterozygosity at chromosome 10. The case reported here serves as a reminder that de novo brain tumors may arise in patients with chromosomal mosaicism.

Single-stage bilateral choroid plexectomy for choroid plexus papilloma in a patient presenting with high cerebrospinal fluid output

2010 ◽  
Vol 5 (4) ◽  
pp. 342-345 ◽  
Author(s):  
Shahid M. Nimjee ◽  
Ciaran J. Powers ◽  
Roger E. McLendon ◽  
Gerald A. Grant ◽  
Herbert E. Fuchs
Keyword(s):  
Cerebrospinal Fluid ◽  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Single Stage ◽  
Histopathological Analysis ◽  
Communicating Hydrocephalus ◽  
Csf Shunting ◽  
Balanced Translocations ◽  
Csf Production ◽  
Plexus Papilloma

Cerebrospinal fluid overproduction resulting in communicating hydrocephalus is observed in patients who have choroid plexus papilloma or choroid plexus carcinoma. Less often, patients with these conditions have diffuse villous hyperplasia. Prior studies report CSF production greater than 3 L per day in these patients. These patients are treated with CSF shunting or by either unilateral choroid plexectomy or staged bilateral choroid plexectomy. The authors present a patient who had a number of congenital anomalies and a karyotype that revealed balanced translocations, 5 to 7 and 9 to 11. She presented with hydrocephalus and had CSF production of 5 L per day, greater output than ever previously reported. She was treated with a single-stage bilateral choroid plexectomy. Histopathological analysis revealed a bilateral choroid plexus papilloma. Postoperatively, the patient responded well clinically and showed radiographic improvement of her hydrocephalus. Bilateral choroid plexus papilloma has been reported in the literature as a cause for neonatal and congenital hydrocephalus. It can result in high CSF output and can be successfully treated with a single-stage bilateral choroid plexectomy. Further studies are ongoing to identify genes involved in embryogenesis of the choroid plexus.

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
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