{"title":"Pediatric Medullary Thyroid Carcinoma","authors":"D. Starenki, Jong-In Park","doi":"10.14205/2309-3021.2015.03.02.1","DOIUrl":"https://doi.org/10.14205/2309-3021.2015.03.02.1","url":null,"abstract":"Medullary thyroid carcinoma (MTC), which originates from thyroid parafollicular C cells, accounts for 3 to 5% of thyroid malignancies. MTC occurs either sporadically or in an inherited autosomal dominant manner. Hereditary MTC occurs as a familial MTC or as a part of multiple endocrine neoplasia (MEN) type 2A and B syndromes. A strong genotype-phenotype correlation has been observed between hereditary MTC and germ-line “gain of function” mutations of the RET proto-oncogene. Most cases of pediatric MTC are hereditary whereas sporadic MTC is rare in children and is usually diagnosed in adults. Therefore, MTC in children is most often diagnosed in the course of a familial genetic investigation. The standard treatment of MTC mainly requires surgery involving total thyroidectomy and central neck node dissection before extrathyroidal extension occurs. To prevent MTC development in hereditary syndromes, prophylactic thyroidectomy is performed in presymptomatic patients. An appropriate age at which the surgery should take place is determined based upon the data from genotyping, serum calcitonin measurements, and ultrasonography. For the treatment of advanced MTC cases, the broad spectrum receptor tyrosine kinase inhibitors vandetanib and cabozantinib, which also inhibit RET, are used although they are not always effective.","PeriodicalId":90229,"journal":{"name":"Journal of pediatric oncology","volume":"23 1","pages":"29 - 37"},"PeriodicalIF":0.0,"publicationDate":"2016-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.14205/2309-3021.2015.03.02.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"66758866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Elizabeth G. Salazar, G. Wertheim, J. Biegel, W. Hwang, S. Tasian, S. Rheingold
{"title":"Mixed Phenotype Acute Leukemia with Low Hypodiploidy in a Pediatric Patient","authors":"Elizabeth G. Salazar, G. Wertheim, J. Biegel, W. Hwang, S. Tasian, S. Rheingold","doi":"10.14205/2309-3021.2015.03.01.4","DOIUrl":"https://doi.org/10.14205/2309-3021.2015.03.01.4","url":null,"abstract":"We describe the case of a 16 year-old female with mixed phenotype acute leukemia B/myeloid, NOS (formerly biphenotypic leukemia) with masked hypodiploidy and somatic TP53 and CDKN2A/B deletions. She achieved morphologic remission with lymphoid-directed multi-agent chemotherapy, but experienced an early medullary relapse 11 months from initial diagnosis. Her case details the unusual finding of hypodiploidy in a patient with ambiguous lineage leukemia and highlights the complexity of therapy selection for these high-risk patients.","PeriodicalId":90229,"journal":{"name":"Journal of pediatric oncology","volume":"3 1","pages":"24 - 28"},"PeriodicalIF":0.0,"publicationDate":"2015-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"66758853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Norbert F Ajeawung, Harish C Joshi, Deepak Kamnasaran
{"title":"Investigation of Targetin, a Microtubule Binding Agent which Regresses the Growth of Pediatric High and Low Grade Gliomas.","authors":"Norbert F Ajeawung, Harish C Joshi, Deepak Kamnasaran","doi":"10.14205/2309-3021.2013.01.01.5","DOIUrl":"https://doi.org/10.14205/2309-3021.2013.01.01.5","url":null,"abstract":"<p><strong>Background: </strong>Pediatric gliomas, the most common solid childhood neoplasm, manifest unique molecular signatures that distinguish them from adult gliomas. Unfortunately, most studies have focused on adult gliomas and extrapolate the findings to treat pediatric gliomas. In this study, we assessed the efficacy of Targetin, a folate conjugated analogue of Noscapine, on the treatment of pediatric low and high grade gliomas.</p><p><strong>Method: </strong>An assortment of standard cancer assays were used with different drug doses and experimental durations.</p><p><strong>Results: </strong>We found that pediatric glioma cells are more susceptible to lower doses of Targetin than parental Noscapine. Targetin functions by disrupting the microtubule network, and can likewise perturb DNA synthesis, delay the cellular transition within the S and G2M cell cycle phases, diminish anchorage independent growth and the migratory/invasiveness of pediatric glioma cells. Moreover, Targetin impairs the expression of several regulators of cancer progression belonging to prominent signalling pathways in pediatric gliomas; including Platelet Derived Growth Factor alpha and some members of the Mitogen Activated Protein Kinase cascade.</p><p><strong>Conclusion: </strong>Targetin has an excellent anti-neoplastic profile and functions to modulate the expression of several genes belonging to key cancer progression pathways in pediatric gliomas. Collectively, findings from this study highlight the usefulness of Targetin for the treatment of pediatric high and low grade gliomas.</p>","PeriodicalId":90229,"journal":{"name":"Journal of pediatric oncology","volume":"1 ","pages":"32-40"},"PeriodicalIF":0.0,"publicationDate":"2013-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c4/e4/nihms519131.PMC3991468.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32276997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Norbert F Ajeawung, Lotta Mononen, Andrea Thorn, Anne-Laure Pin, Harish C Joshi, Jacques Huot, Deepak Kamnasaran
{"title":"<i>In-Vitro</i> and <i>Ex-Vivo</i> Investigations of the Microtubule Binding Drug Targetin on Angiogenesis.","authors":"Norbert F Ajeawung, Lotta Mononen, Andrea Thorn, Anne-Laure Pin, Harish C Joshi, Jacques Huot, Deepak Kamnasaran","doi":"10.14205/2309-3021.2013.01.01.6","DOIUrl":"https://doi.org/10.14205/2309-3021.2013.01.01.6","url":null,"abstract":"<p><strong>Background: </strong>Intervention aimed at disrupting or inhibiting newly formed vascular network is highly desired to attenuate the progression of angiogenesis-dependent diseases. In cancer, this is tightly associated with the generation of VEGF by hypoxia inducible factor-1α following its activation by hypoxia. In light of the multiple cellular roles played by microtubules and their involvement in the processing of the hypoxia inducible factor-1α transcript, modulation of microtubule dynamics is emerging as a logical approach to suppress tumor reliance on angiogenesis. Targetin is a novel noscapinoid that interferes with microtubule dynamicity and inhibits the growth of cell lines from many types of cancers.</p><p><strong>Methods and results: </strong>Utilizing <i>in-vitro</i> and <i>ex-vivo</i> angiogenic models, we discovered the vascular disrupting and anti-angiogenic properties of Targetin. Targetin disrupted pre-assembled capillary-like networks of human endothelial cells by severing cell-cell junctions, inhibiting endothelial cell proliferation and metabolic activity in the presence and absence of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF). Furthermore, we show that Targetin significantly inhibits the formation of neovasculature network sprouting from rat aortic explants stimulated with proangiogenic stimuli, namely VEGF or bFGF.</p><p><strong>Conclusion: </strong>We conclude that Targetin is a potential clinically promising anti-angiogenic agent for the treatment of many diseases including cancers.</p>","PeriodicalId":90229,"journal":{"name":"Journal of pediatric oncology","volume":"1 ","pages":"41-47"},"PeriodicalIF":0.0,"publicationDate":"2013-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/51/8a/nihms519134.PMC3991473.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32276998","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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