The Experts below are selected from a list of 1380 Experts worldwide ranked by ideXlab platform
Louis P. Dehner - One of the best experts on this subject based on the ideXlab platform.
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ivado treatment of type ii and type iii pleuropulmonary Blastoma ppb a report from the international ppb registry
Journal of Clinical Oncology, 2014Co-Authors: Leslie Doros, Louis P. Dehner, Gretchen M. Williams, Kris Ann P Schultz, Ashley D Hill, Anne K Harris, Nicolas Andre, Carlos Rodriguezgalindo, Yoav H MessingerAbstract:10060 Background: Pleuropulmonary Blastoma (PPB) is a rare malignancy of the lung presenting in young children. The International PPB Registry (IPBBR) has pathologically confirmed more than 400 cas...
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type i pleuropulmonary Blastoma a report from the international pleuropulmonary Blastoma registry
Journal of Clinical Oncology, 2006Co-Authors: Ashley D Hill, Gretchen M. Williams, Yoav Messinger, Christopher L Moertel, Marsha Finkelstein, Louis P. DehnerAbstract:Purpose Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen chil...
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Type I pleuropulmonary Blastoma: a report from the International Pleuropulmonary Blastoma
2006Co-Authors: John R. Priest, Gretchen M. Williams, Yoav Messinger, Ashley D Hill, Christopher L Moertel, Marsha J. Finkelstein, Louis P. DehnerAbstract:Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n 30) and literature (n 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery chemotherapy group (P .01); overall survival did not differ (P .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. Conclusion Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy. J Clin Oncol 24:4492-4498. © 2006 by American Society of Clinical Oncolog
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pleuropulmonary Blastoma is the pulmonary Blastoma of childhood
Seminars in Diagnostic Pathology, 1994Co-Authors: Louis P. DehnerAbstract:Pulmonary Blastoma was defined by Spencer and others as a neoplasm with histopathological features thought to be reminiscent of Wilms' tumor. Unlike the other embryonal-fetal neoplasms that typically occur in early childhood, the majority of pulmonary Blastomas have been reported in adults. One explanation offered by Spencer for the delayed clinical presentation of pulmonary Blastoma is the continued development of lung parenchyma well past the postnatal period. It has been proposed that the pulmonary Blastoma is a variant of carcinosarcoma, which is seen almost exclusively in adults. The classic pulmonary Blastoma is described as a neoplasm with a mixture of primitive tubular profiles, immature blastema, and a spindle cell stroma. More recently, some have included the well-differentiated adenocarcinoma of fetal type as a pure epithelial expression of pulmonary Blastoma. By contrast, most cases of pulmonary Blastoma in children have been described as having an exclusive mesenchymal composition, either embryonal rhabdomyosarcoma or a complex, mixed blastematous and sarcomatous neoplasm. Some pulmonary Blastomas in children, particularly those with only embryonal rhabdomyosarcoma, have presented as a peripheral multicystic lesion that has been interpreted as a developmental cyst, either cystic adenomatoid malformation or bronchogenic cyst. Other tumors have been described as intrathoracic and anatomically separate from the lung, like some extralobar sequestrations. We have proposed the designation pleuropulmonary Blastoma for these pulmonary or extrapulmonary neoplasms of childhood. We propose that this neoplasm is the rightful pulmonary Blastoma rather than the pulmonary Blastoma of Spencer, which has been the long-time pretender to the title.
Mark T Keating - One of the best experts on this subject based on the ideXlab platform.
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heat shock protein 60 is required for blastema formation and maintenance during regeneration
Proceedings of the National Academy of Sciences of the United States of America, 2005Co-Authors: Shinji Makino, Geoffrey G Whitehead, Chingling Lien, Soo Joong Kim, Payal Jhawar, Akane Kono, Yasushi Kawata, Mark T KeatingAbstract:Zebrafish fin regeneration requires the formation and maintenance of blastema cells. Blastema cells are not derived from stem cells but behave as such, because they are slow-cycling and are thought to provide rapidly proliferating daughter cells that drive regenerative outgrowth. The molecular basis of blastema formation is not understood. Here, we show that heat-shock protein 60 (hsp60) is required for blastema formation and maintenance. We used a chemical mutagenesis screen to identify no blastema (nbl), a zebrafish mutant with an early fin regeneration defect. Fin regeneration failed in nbl due to defective blastema formation. nbl also failed to regenerate hearts. Positional cloning and mutational analyses revealed that nbl results from a V324E missense mutation in hsp60. This mutation reduced hsp60 function in binding and refolding denatured proteins. hsp60 expression is increased during formation of blastema cells, and dysfunction leads to mitochondrial defects and apoptosis in these cells. These data indicate that hsp60 is required for the formation and maintenance of regenerating tissue.
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heat shock protein 60 is required for blastema formation and maintenance during regeneration
Proceedings of the National Academy of Sciences of the United States of America, 2005Co-Authors: Shinji Makino, Geoffrey G Whitehead, Chingling Lien, Soo Joong Kim, Payal Jhawar, Akane Kono, Yasushi Kawata, Mark T KeatingAbstract:Zebrafish fin regeneration requires the formation and maintenance of blastema cells. Blastema cells are not derived from stem cells but behave as such, because they are slow-cycling and are thought to provide rapidly proliferating daughter cells that drive regenerative outgrowth. The molecular basis of blastema formation is not understood. Here, we show that heat-shock protein 60 (hsp60) is required for blastema formation and maintenance. We used a chemical mutagenesis screen to identify no blastema (nbl), a zebrafish mutant with an early fin regeneration defect. Fin regeneration failed in nbl due to defective blastema formation. nbl also failed to regenerate hearts. Positional cloning and mutational analyses revealed that nbl results from a V324E missense mutation in hsp60. This mutation reduced hsp60 function in binding and refolding denatured proteins. hsp60 expression is increased during formation of blastema cells, and dysfunction leads to mitochondrial defects and apoptosis in these cells. These data indicate that hsp60 is required for the formation and maintenance of regenerating tissue.
Gemma Johnson - One of the best experts on this subject based on the ideXlab platform.
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cellular heterogeneity and lineage restriction during mouse digit tip regeneration at single cell resolution
Developmental Cell, 2020Co-Authors: Gemma Johnson, Erick J Masias, Jessica A LehoczkyAbstract:Summary Innate regeneration following digit tip amputation is one of the few examples of epimorphic regeneration in mammals. Digit tip regeneration is mediated by the blastema, the same structure invoked during limb regeneration in some lower vertebrates. By genetic lineage analyses, the digit tip blastema has been defined as a population of heterogeneous, lineage-restricted progenitor cells. These previous studies, however, do not comprehensively evaluate blastema heterogeneity or address lineage restriction of closely related cell types. In this report, we present single-cell RNA sequencing of over 38,000 cells from mouse digit tip blastemas and unamputated control digit tips and generate an atlas of the cell types participating in digit tip regeneration. We computationally define differentiation trajectories of vascular, monocytic, and fibroblastic lineages over regeneration, and while our data confirm broad lineage restriction of progenitors, our analysis reveals 67 genes enriched in blastema fibroblasts including a novel regeneration-specific gene, Mest.
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cellular heterogeneity and lineage restriction during mouse digit tip regeneration at single cell resolution
bioRxiv, 2019Co-Authors: Gemma Johnson, Erick J Masias, Jessica A LehoczkyAbstract:Innate regeneration following digit tip amputation is one of the few examples of epimorphic regeneration in mammals. Digit tip regeneration is mediated by the blastema, the same structure invoked during limb regeneration in some lower vertebrates. By genetic lineage analyses in mice, the digit tip blastema has been defined as a population of heterogeneous, lineage restricted progenitor cells. These previous studies, however, do not comprehensively evaluate blastema heterogeneity or address lineage restriction of closely related cell types. In this report we present single cell RNA sequencing of over 38,000 cells from mouse digit tip blastemas and unamputated control digit tips and generate an atlas of the cell types participating in digit tip regeneration. We define the differentiation trajectories of vascular, monocytic, and fibroblastic lineages over regeneration, and while our data confirm broad lineage restriction of progenitors, our analysis reveals an early blastema fibroblast population expressing a novel regeneration-specific gene, Mest.
Gretchen M. Williams - One of the best experts on this subject based on the ideXlab platform.
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pleuropulmonary Blastoma a report on 350 central pathology confirmed pleuropulmonary Blastoma cases by the international pleuropulmonary Blastoma registry
Cancer, 2015Co-Authors: Yoav H Messinger, Gretchen M. Williams, Kris Ann P Schultz, Douglas R Stewart, Anne K Harris, Jiandong Yang, Leslie Doros, Philip S Rosenberg, Ashley D HillAbstract:Background Pleuropulmonary Blastoma (PPB) has 3 subtypes on a tumor progression pathway ranging from type I (cystic) to type II (cystic/solid) and type III (completely solid). A germline mutation in DICER1 is the genetic cause in the majority of PPB cases.
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ivado treatment of type ii and type iii pleuropulmonary Blastoma ppb a report from the international ppb registry
Journal of Clinical Oncology, 2014Co-Authors: Leslie Doros, Louis P. Dehner, Gretchen M. Williams, Kris Ann P Schultz, Ashley D Hill, Anne K Harris, Nicolas Andre, Carlos Rodriguezgalindo, Yoav H MessingerAbstract:10060 Background: Pleuropulmonary Blastoma (PPB) is a rare malignancy of the lung presenting in young children. The International PPB Registry (IPBBR) has pathologically confirmed more than 400 cas...
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dicer1 mutations in childhood cystic nephroma and its relationship to dicer1 renal sarcoma
Modern Pathology, 2014Co-Authors: Leslie Doros, Gretchen M. Williams, Kris Ann P Schultz, Yoav H Messinger, Jiandong Yang, Christopher T Rossi, Amanda Field, Mariana M Cajaiba, Elizabeth J Perlman, Helen P CathroAbstract:The pathogenesis of cystic nephroma of the kidney has interested pathologists for over 50 years. Emerging from its initial designation as a type of unilateral multilocular cyst, cystic nephroma has been considered as either a developmental abnormality or a neoplasm or both. Many have viewed cystic nephroma as the benign end of the pathologic spectrum with cystic partially differentiated nephroBlastoma and Wilms tumor, whereas others have considered it a mixed epithelial and stromal tumor. We hypothesize that cystic nephroma, like the pleuropulmonary Blastoma in the lung, represents a spectrum of abnormal renal organogenesis with risk for malignant transformation. Here we studied DICER1 mutations in a cohort of 20 cystic nephromas and 6 cystic partially differentiated nephroBlastomas, selected independently of a familial association with pleuropulmonary Blastoma and describe four cases of sarcoma arising in cystic nephroma, which have a similarity to the solid areas of type II or III pleuropulmonary Blastoma. The genetic analyses presented here confirm that DICER1 mutations are the major genetic event in the development of cystic nephroma. Further, cystic nephroma and pleuropulmonary Blastoma have similar DICER1 loss of function and 'hotspot' missense mutation rates, which involve specific amino acids in the RNase IIIb domain. We propose an alternative pathway with the genetic pathogenesis of cystic nephroma and DICER1-renal sarcoma paralleling that of type I to type II/III malignant progression of pleuropulmonary Blastoma.
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type i pleuropulmonary Blastoma a report from the international pleuropulmonary Blastoma registry
Journal of Clinical Oncology, 2006Co-Authors: Ashley D Hill, Gretchen M. Williams, Yoav Messinger, Christopher L Moertel, Marsha Finkelstein, Louis P. DehnerAbstract:Purpose Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen chil...
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Type I pleuropulmonary Blastoma: a report from the International Pleuropulmonary Blastoma
2006Co-Authors: John R. Priest, Gretchen M. Williams, Yoav Messinger, Ashley D Hill, Christopher L Moertel, Marsha J. Finkelstein, Louis P. DehnerAbstract:Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n 30) and literature (n 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery chemotherapy group (P .01); overall survival did not differ (P .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. Conclusion Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy. J Clin Oncol 24:4492-4498. © 2006 by American Society of Clinical Oncolog
Ashley D Hill - One of the best experts on this subject based on the ideXlab platform.
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pleuropulmonary Blastoma a report on 350 central pathology confirmed pleuropulmonary Blastoma cases by the international pleuropulmonary Blastoma registry
Cancer, 2015Co-Authors: Yoav H Messinger, Gretchen M. Williams, Kris Ann P Schultz, Douglas R Stewart, Anne K Harris, Jiandong Yang, Leslie Doros, Philip S Rosenberg, Ashley D HillAbstract:Background Pleuropulmonary Blastoma (PPB) has 3 subtypes on a tumor progression pathway ranging from type I (cystic) to type II (cystic/solid) and type III (completely solid). A germline mutation in DICER1 is the genetic cause in the majority of PPB cases.
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ivado treatment of type ii and type iii pleuropulmonary Blastoma ppb a report from the international ppb registry
Journal of Clinical Oncology, 2014Co-Authors: Leslie Doros, Louis P. Dehner, Gretchen M. Williams, Kris Ann P Schultz, Ashley D Hill, Anne K Harris, Nicolas Andre, Carlos Rodriguezgalindo, Yoav H MessingerAbstract:10060 Background: Pleuropulmonary Blastoma (PPB) is a rare malignancy of the lung presenting in young children. The International PPB Registry (IPBBR) has pathologically confirmed more than 400 cas...
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type i pleuropulmonary Blastoma a report from the international pleuropulmonary Blastoma registry
Journal of Clinical Oncology, 2006Co-Authors: Ashley D Hill, Gretchen M. Williams, Yoav Messinger, Christopher L Moertel, Marsha Finkelstein, Louis P. DehnerAbstract:Purpose Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen chil...
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Type I pleuropulmonary Blastoma: a report from the International Pleuropulmonary Blastoma
2006Co-Authors: John R. Priest, Gretchen M. Williams, Yoav Messinger, Ashley D Hill, Christopher L Moertel, Marsha J. Finkelstein, Louis P. DehnerAbstract:Type I pleuropulmonary Blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results Thirty-eight type I PPB cases were identified: Registry (n 30) and literature (n 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery chemotherapy group (P .01); overall survival did not differ (P .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. Conclusion Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy. J Clin Oncol 24:4492-4498. © 2006 by American Society of Clinical Oncolog
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uscap specialty conference case 1 type i pleuropulmonary Blastoma
Pediatric and Developmental Pathology, 2005Co-Authors: Ashley D HillAbstract:Pleuropulmonary Blastoma (PPB) was defined in 1988 by Manivel et al. in a series describing 11 intrathoracic pulmonary neoplasms in young children. The PPB is a unique peripheral pulmonary or pleural-based tumor of childhood that is characterized in its earliest form as a bland-appearing multiloculated cyst with small foci of tumor cells and in later forms as mixed and predominantly primitive, overtly malignant neoplasms. Prior to the introduction of the PPB as a distinct entity, this tumor had been reported in the literature as pulmonary Blastoma, sarcoma arising in mesenchymal cystic hamartoma, embryonal sarcoma, malignant mesenchymoma, primary pulmonary rhabdomyosarcoma and rhabdomyosarcoma arising in congenital adenomatoid malformation or bronchogenic cyst. Over the past 15 years, PPB has come to be recognized in centers around the world. With the establishment of the Pleuropulmonary Blastoma Registry by Jack Priest, MD, and colleagues, there has been improved understanding of this rare pediatric neoplasm.