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Choroid plexus carcinoma

Devin Ancona, Dan York, Robert J Higgins, Danika Bannasch, Peter J Dickinson
Choroid plexus tumors (CPTs) occur spontaneously in humans and dogs providing an opportunity for comparative cross species analysis of common tumor mechanisms. Large scale chromosomal copy number alterations are the hallmark of human CPTs and identification of driver genes within these regions is problematic. Copy number alterations in 12 spontaneous dog CPTs were defined using an Illumina 170 K single nucleotide polymorphism array and were characterized by highly recurrent whole chromosomal losses in up to 100% of cases with few chromosome wide gains...
March 14, 2018: Journal of Neuropathology and Experimental Neurology
Tomoaki Sasaki, John Kim, Toshio Moritani, Aristides A Capizzano, Shawn P Sato, Yutaka Sato, Patricia Kirby, Shunta Ishitoya, Akiko Oya, Masahiro Toda, Sayaka Yuzawa, Koji Takahashi
PURPOSE: To explore the utility of the apparent diffusion coefficient (ADC) and tumor volume to predict histological grade and prognosis in patients with choroid plexus tumors. METHODS: ADC and tumor volumes were retrospectively evaluated in 25 patients with choroid plexus papilloma (CPP; WHO grade 1 [n = 13]), atypical CPP (aCPP; grade 2 [n = 8]), or choroid plexus carcinoma (grade 3 [n = 4]) The prognostic roles of ADC and tumor volume were assessed...
March 15, 2018: Neuroradiology
Morgan L Shannon, Ryann M Fame, Kevin F Chau, Neil Dani, Monica L Calicchio, Gwenaelle S Géléoc, Hart G W Lidov, Sanda Alexandrescu, Maria K Lehtinen
Choroid plexus tumors and ciliary body medulloepithelioma are predominantly pediatric neoplasms. Progress in understanding the pathogenesis of these tumors has been hindered by their rarity and lack of models that faithfully recapitulate the disease. Here, we find that endogenous c-Myc is down-regulated in the forebrain neuroepithelium, whose neural plate border domains give rise to anterior choroid plexus and ciliary body. To uncover the consequences of persistent MYC (c-Myc) expression, c-Myc expression was forced in multipotent neural precursors (Nestin-Cre:MYC), which produced a fully penetrant model of choroid plexus carcinoma and ciliary body medulloepithelioma...
March 12, 2018: American Journal of Pathology
Wafik Zaky, Jonathan L Finlay
Choroid plexus (CP) carcinoma is a rare pediatric brain neoplasm. Recent studies have highlighted the potential of genome-wide methylation and gene expression profiling to provide additional layers of information to improve tumor risk-stratification. There is a lack of data regarding the best therapy, and approaches have been heterogeneous. Despite multidisciplinary treatment approaches, the outcome remains guarded and treatments have been based on case series and expert opinions. In this study, we discuss the recent wealth of data regarding CP carcinoma molecular biology and current management...
March 12, 2018: Pediatric Blood & Cancer
Mari Inohana, Ayumi Eguchi, Misato Nakamura, Rei Nagahara, Yosuke Watanabe, Toshinori Yoshida, Makoto Shibutani
We describe a case of choroid plexus carcinoma arising in the cerebrum of a 7-week-old male Sprague-Dawley rat. The tumor mass occupied the right lateral ventricle of the cerebrum. Histological analyses revealed that the epithelial tumor cells had proliferated in tubular, cribriform, papillary and solid growth patterns in the vicinity of the choroid plexus, with slight invasion into the cerebrum parenchyma. We divided the tumor cells into cuboidal, elongated and intermediate cells. Immunohistochemical studies showed that these tumor cells expressed relatively high levels of cytokeratin AE1/AE3, vimentin and glial fibrillary acidic proteins, and low levels of nestin, oligodendrocyte transcription factor and doublecortin proteins...
February 16, 2018: Journal of Veterinary Medical Science
Hiroe Ono, Hiromi Sakamoto, Teruhiko Yoshida, Norihisa Saeki
Prostate stem cell antigen (PSCA) is a glycosylphosphatidylinositol (GPI)-anchored cell surface protein and exhibits an organ-dependent expression pattern in cancer. PSCA is upregulated in prostate cancer and downregulated in gastric cancer. PSCA is expressed in a variety of human organs. Although certain studies previously demonstrated its expression in the mammalian and avian brain, its expression in the human brain has not been thoroughly elucidated. Additionally, it was previously reported that PSCA is weakly expressed in the astrocytes of the normal human brain but aberrantly expressed in glioma, suggesting that PSCA is a promising target of glioma therapy and prostate cancer therapy...
March 2018: Oncology Letters
Luisa Vera Muscatello, Giancarlo Avallone, Fabienne Serra, Torsten Seuberlich, Maria Teresa Mandara, Silvia Sisó, Barbara Brunetti, Anna Oevermann
Choroid plexus tumors (CPT) are intraventricular neoplasms accounting for 10% of all primary central nervous system tumors in dogs. They are frequently classified according to the human WHO classification into choroid plexus papilloma (CPP, grade I), atypical CPP (aCPP, grade II), and choroid plexus carcinoma (CPC, grade III). Histological features observed in canine CPT such as increased vascular density (IVD) and glomeruloid microvascular proliferation (GMVP) are not part of the WHO classification. This multi-centric study aimed to investigate tumor-associated vascular hyperplasia in dogs by determining the prevalence of GMVP and IVD in 52 canine CPT and their association with tumor grade...
January 1, 2018: Veterinary Pathology
Jonathan J Mayl, Miguel A Flores, John W Stelzer, Bo Liu, Steven A Messina, John V Murray
Retinal detachment with subsequent silicone oil retinopexy is not uncommon. A known complication of silicone retinopexy is intraventricular migration of the intraocular silicone oil. While the oil itself does not result in direct pathology, misdiagnosis may lead to an unnecessary diagnostic workup and possibly predispose the patient to surgery intervention. Silicone oil typically appears hyperdense on computer tomography (CT) and hyperintense on T1-weighted magnetic resonance (MR). These imaging findings may mimic a mass or blood products...
February 3, 2018: Emergency Radiology
Nicole Prendergast, Jeffrey D Goldstein, Alexandra D Beier
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.
February 2, 2018: Journal of Neurosurgery. Pediatrics
Salsabiel El Nagar, Frederique Zindy, Charlotte Moens, Luc Martin, Damien Plassard, Martine F Roussel, Thomas Lamonerie, Nathalie Billon
Choroid plexus carcinomas (CPCs) are highly malignant brain tumours predominantly found in children and associated to poor prognosis. Improved therapy for these cancers would benefit from the generation of animal models. Here we have created a novel mouse CPC model by expressing a stabilised form of c-Myc (MycT58A) and inactivating Trp53 in the choroid plexus of newborn mice. This induced aberrant proliferation of choroid plexus epithelial cells, leading to aggressive tumour development and death within 150 days...
January 12, 2018: Biochemical and Biophysical Research Communications
Saumya Gupta, Kirti Gupta, Apinderpreet Singh
No abstract text is available yet for this article.
January 11, 2018: Pediatric Neurosurgery
Birgit Nimmervoll, Nidal Boulos, Brandon M Bianski, Jason Dapper, Michael DeCuypere, Anang A Shelat, Sabrina Terranova, Hope Elizabeth Terhune, Amar Gajjar, Yogesh T Patel, Burgess B Freeman, Arzu Onar-Thomas, Clinton F Stewart, Martine F Roussel, R Kiplin Guy, Thomas E Merchant, Christopher Calabrese, Karen D Wright, Richard J Gilbertson
PURPOSE: Curing all children with brain tumors will require an understanding of how each subtype responds to conventional treatments and how best to combine existing and novel therapies.  It is extremely challenging to acquire this knowledge in the clinic alone, especially among patients with rare tumors.  Therefore, we developed a preclinical brain tumor platform to test combinations of conventional and novel therapies in a manner that closely recapitulates clinic trials.  Experimental Design: A multidisciplinary team was established to design and conduct neurosurgical, fractionated radiotherapy and chemotherapy studies, alone or in combination, in accurate mouse models of supratentorial ependymoma (SEP) subtypes and choroid plexus carcinoma (CPC)...
January 4, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Mark R Wick
Several primary pathologic entities in diverse anatomic locations have the potential to simulate metastatic neoplasms histologically. Their misinterpretation as such may result in needless and extensive clinical evaluations that are intended to detect a presumed malignancy at another site. More importantly, mistakes of this type can deprive patients of surgical excisions that could be curative. This presentation considers a review of selected primary lesions that can simulate metastases. They include hemangioblastoma, glioblastoma and meningioma with epithelial metaplasia, choroid plexus carcinomas, primary neuroendocrine carcinomas in unusual locations, special forms of sinonasal and salivary glandular adenocarcinoma, clear-cell thyroid carcinomas, unusual microscopic subtypes of pulmonary adenocarcinoma, epithelioid myomelanocytomas ("sugar tumors"), mesotheliomas, primary thymic carcinomas, endodermal choristomas of the interatrial myocardium, peripheral cholangiocarcinoma, adrenocortical carcinoma, adenocarcinomas of the urinary bladder, mucinous and "rhabdoid" tumors of the ovaries, rete testis adenocarcinomas, interdigitating dendritic-cell sarcoma of lymph nodes, selected sweat gland carcinomas, cutaneous Merkel cell carcinoma, primary dermal and subcutaneous melanoma, mucosal and visceral melanomas, epithelioid sarcoma, clear-cell sarcoma, and adamantinoma of long bones...
March 2018: Seminars in Diagnostic Pathology
Allison F O'Neill, Stephan D Voss, Jyothi P Jagannathan, Junne Kamihara, Callie Nibecker, Elena Itriago-Araujo, Serena Masciari, Erin Parker, Mauricio Barreto, Wendy B London, Judy E Garber, Lisa Diller
BACKGROUND: Li-Fraumeni syndrome (LFS) is an autosomal dominant hereditary cancer syndrome associated with germline mutations in the TP53 gene and a high risk of childhood-onset malignancies. Cancer surveillance is challenging in pediatric mutation carriers given the anatomic spectrum of malignancies and young age of onset. Whole-body magnetic resonance imaging (WB-MRI) may provide an acceptable method for early cancer detection. PROCEDURE: We conducted a prospective feasibility pilot study of pediatric subjects (age < 18 years) with LFS to determine return rates for annual WB-MRI scan...
February 2018: Pediatric Blood & Cancer
Amina Amadou, Maria I Waddington Achatz, Pierre Hainaut
PURPOSE OF REVIEW: Germline pathogenic TP53 mutation may predispose to multiple cancers but penetrance and cancer patterns remain incompletely documented. We have analyzed international agency for research on cancer TP53 database to reevaluate age and variant-dependent tumor patterns. RECENT FINDINGS: Genome-wide studies suggest that germline variants are more frequent than estimated prevalence of Li-Fraumeni syndrome (LFS), suggesting that many carriers of potentially pathogenic mutations may not develop the syndrome...
January 2018: Current Opinion in Oncology
Mariette Renaux-Petel, Françoise Charbonnier, Jean-Christophe Théry, Pierre Fermey, Gwendoline Lienard, Jacqueline Bou, Sophie Coutant, Myriam Vezain, Edwige Kasper, Steeve Fourneaux, Sandrine Manase, Maud Blanluet, Bruno Leheup, Ludovic Mansuy, Jacqueline Champigneulle, Céline Chappé, Michel Longy, Nicolas Sévenet, Brigitte Bressac-de Paillerets, Léa Guerrini-Rousseau, Laurence Brugières, Olivier Caron, Jean-Christophe Sabourin, Isabelle Tournier, Stéphanie Baert-Desurmont, Thierry Frébourg, Gaëlle Bougeard
BACKGROUND: Development of tumours such as adrenocortical carcinomas (ACC), choroid plexus tumours (CPT) or female breast cancers before age 31 or multiple primary cancers belonging to the Li-Fraumeni (LFS) spectrum is, independently of the familial history, highly suggestive of a germline TP53 mutation. The aim of this study was to determine the contribution of de novo and mosaic mutations to LFS. METHODS AND RESULTS: Among 328 unrelated patients harbouring a germline TP53 mutation identified by Sanger sequencing and/or QMPSF, we could show that the mutations had occurred de novo in 40 cases, without detectable parental age effect...
October 25, 2017: Journal of Medical Genetics
Joanna Trubicka, Iwona Filipek, Iwanowski Piotr, Małgorzata Rydzanicz, Wiesława Grajkowska, Dorota Piekutowska-Abramczuk, Krystyna Chrzanowska, Agnieszka Karkucińska-Więckowska, Katarzyna Iwanicka-Pronicka, Maciej Pronicki, Maria Łastowska, Rafał Płoski, Bożenna Dembowska-Bagińska
Choroid plexus tumors (CPT) constitute 2%-5% of all pediatric brain tumors and include high grade choroid plexus carcinoma (CPC). About 40% of CPC patients harbor germline TP53 mutations, associated with diminished survival rates. However, the number of TP53 carriers might be underestimated due to suboptimal ability of Sanger sequencing to identify mosaicism. We describe an 18-month-old boy with ultra-rare, bilateral disseminated CPC and negative family history of cancer. Next generation sequencing (NGS) revealed constitutional mosaicism of de novo TP53 mutation, which was barely detectable by Sanger sequencing...
October 2017: Cancer Genetics
Albert Cornelius, Jessica Foley, Jeffrey Bond, Abhinav B Nagulapally, Julie Steinbrecher, William P D Hendricks, Maria Rich, Sangeeta Yendrembam, Genevieve Bergendahl, Jeffrey M Trent, Giselle S Sholler
Choroid plexus carcinomas (CPCs) are rare, aggressive pediatric brain tumors with no established curative therapy for relapsed disease, and poor survival rates. TP53 Mutation or dysfunction correlates with poor or no survival outcome in CPCs. Here, we report the case of a 4 month-old female who presented with disseminated CPC. After initial response to tumor resection and adjuvant-chemotherapy, the tumor recurred and metastasized with no response to aggressive relapse therapy suggesting genetic predisposition...
2017: Frontiers in Pharmacology
Supriya Mallick, Rony Benson, Wineeta Melgandi, Goura K Rath
PURPOSE: The optimal treatment of patients with choroid plexus carcinoma (CPC) is unclear. We conducted a systematic review and meta-analysis of individual patient information to determine the effect of surgery, adjuvant therapy, and other prognostic factors for CPC. METHODS AND MATERIALS: A comprehensive search of the PubMed and Google Scholar databases was performed using the following MeSH terms to find all possible reports on CPC: choroid plexus tumor; choroid plexus carcinoma; choroid plexus carcinoma AND treatment; and choroid plexus carcinoma AND survival...
December 1, 2017: International Journal of Radiation Oncology, Biology, Physics
Matthew McEvoy, Nathan Robison, Peter Manley, Torunn Yock, Kristine Konopka, Robert E Brown, Johannes Wolff, Adam L Green
The management of choroid plexus carcinoma (CPC) is challenging and multifaceted. Here, we discuss a 3-year-old girl with CPC and Li-Fraumeni syndrome who achieved full remission after surgery and chemotherapy, with radiation therapy spared. At recurrence, we used a novel, standard-dose cytotoxic chemotherapy regimen, focal proton radiation therapy, and targeted agents based on morphoproteomic analysis to achieve long-term survival. We highlight the rationale for our therapy at recurrence, as well as the risk-benefit analyses necessary in decision making for these patients...
November 2017: Journal of Pediatric Hematology/oncology
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