Adult-onset cancer predisposition syndromes in children and adolescents - to test or not to test?
Clin Cancer Res. 2024 Feb 27. doi: 10.1158/1078-0432.CCR-23-3683. Online ahead of print.ABSTRACTWith the increasing use of comprehensive germline genetic testing of children and adolescents with cancer, it has become evident that pathogenic variants (PVs) in adult-onset cancer predisposition genes (aoCPGs) underlying adult-onset cancer predisposition syndromes (aoCPS) such as Lynch syndrome or hereditary breast and ovarian cancer are enriched and reported in one to two percent of children and adolescents with cancer. However, the causal relationship between PVs in aoCPGs and childhood cancer is still under investigation. T...
Source: Clinical Genitourinary Cancer - February 27, 2024 Category: Cancer & Oncology Authors: Christian P Kratz Philip J Lupo Kristin Zelley Jaclyn Schienda Kim E Nichols Douglas R Stewart David Malkin Garrett M Brodeur Kara Maxwell Sharon E Plon Michael F Walsh Source Type: research
Adult-onset cancer predisposition syndromes in children and adolescents - to test or not to test?
Clin Cancer Res. 2024 Feb 27. doi: 10.1158/1078-0432.CCR-23-3683. Online ahead of print.ABSTRACTWith the increasing use of comprehensive germline genetic testing of children and adolescents with cancer, it has become evident that pathogenic variants (PVs) in adult-onset cancer predisposition genes (aoCPGs) underlying adult-onset cancer predisposition syndromes (aoCPS) such as Lynch syndrome or hereditary breast and ovarian cancer are enriched and reported in one to two percent of children and adolescents with cancer. However, the causal relationship between PVs in aoCPGs and childhood cancer is still under investigation. T...
Source: Clinical Breast Cancer - February 27, 2024 Category: Cancer & Oncology Authors: Christian P Kratz Philip J Lupo Kristin Zelley Jaclyn Schienda Kim E Nichols Douglas R Stewart David Malkin Garrett M Brodeur Kara Maxwell Sharon E Plon Michael F Walsh Source Type: research
Adult-onset cancer predisposition syndromes in children and adolescents - to test or not to test?
Clin Cancer Res. 2024 Feb 27. doi: 10.1158/1078-0432.CCR-23-3683. Online ahead of print.ABSTRACTWith the increasing use of comprehensive germline genetic testing of children and adolescents with cancer, it has become evident that pathogenic variants (PVs) in adult-onset cancer predisposition genes (aoCPGs) underlying adult-onset cancer predisposition syndromes (aoCPS) such as Lynch syndrome or hereditary breast and ovarian cancer are enriched and reported in one to two percent of children and adolescents with cancer. However, the causal relationship between PVs in aoCPGs and childhood cancer is still under investigation. T...
Source: Clinical Cancer Research - February 27, 2024 Category: Cancer & Oncology Authors: Christian P Kratz Philip J Lupo Kristin Zelley Jaclyn Schienda Kim E Nichols Douglas R Stewart David Malkin Garrett M Brodeur Kara Maxwell Sharon E Plon Michael F Walsh Source Type: research
Adult-onset cancer predisposition syndromes in children and adolescents - to test or not to test?
Clin Cancer Res. 2024 Feb 27. doi: 10.1158/1078-0432.CCR-23-3683. Online ahead of print.ABSTRACTWith the increasing use of comprehensive germline genetic testing of children and adolescents with cancer, it has become evident that pathogenic variants (PVs) in adult-onset cancer predisposition genes (aoCPGs) underlying adult-onset cancer predisposition syndromes (aoCPS) such as Lynch syndrome or hereditary breast and ovarian cancer are enriched and reported in one to two percent of children and adolescents with cancer. However, the causal relationship between PVs in aoCPGs and childhood cancer is still under investigation. T...
Source: Clinical Genitourinary Cancer - February 27, 2024 Category: Cancer & Oncology Authors: Christian P Kratz Philip J Lupo Kristin Zelley Jaclyn Schienda Kim E Nichols Douglas R Stewart David Malkin Garrett M Brodeur Kara Maxwell Sharon E Plon Michael F Walsh Source Type: research
Current status of head and neck sarcomas in Japan in 2016 –2019: an analysis using the national cancer registry
ConclusionsHead and neck sarcomas occurred rarely, but most frequently in the nasal cavity and paranasal sinuses in Japan. Poor outcomes were observed for sarcoma patients than for non-sarcoma head and neck cancer patients. (Source: International Journal of Clinical Oncology)
Source: International Journal of Clinical Oncology - February 27, 2024 Category: Cancer & Oncology Source Type: research
KDM3B inhibitors disrupt the oncogenic activity of PAX3-FOXO1 in fusion-positive rhabdomyosarcoma
Nat Commun. 2024 Feb 24;15(1):1703. doi: 10.1038/s41467-024-45902-y.ABSTRACTFusion-positive rhabdomyosarcoma (FP-RMS) is an aggressive pediatric sarcoma driven primarily by the PAX3-FOXO1 fusion oncogene, for which therapies targeting PAX3-FOXO1 are lacking. Here, we screen 62,643 compounds using an engineered cell line that monitors PAX3-FOXO1 transcriptional activity identifying a hitherto uncharacterized compound, P3FI-63. RNA-seq, ATAC-seq, and docking analyses implicate histone lysine demethylases (KDMs) as its targets. Enzymatic assays confirm the inhibition of multiple KDMs with the highest selectivity for KDM3B. St...
Source: Cell Research - February 24, 2024 Category: Cytology Authors: Yong Yean Kim Berkley E Gryder Ranuka Sinniah Megan L Peach Jack F Shern Abdalla Abdelmaksoud Silvia Pomella Girma M Woldemichael Benjamin Z Stanton David Milewski Joseph J Barchi John S Schneekloth Raj Chari Joshua T Kowalczyk Shilpa R Shenoy Jason R Eva Source Type: research
KDM3B inhibitors disrupt the oncogenic activity of PAX3-FOXO1 in fusion-positive rhabdomyosarcoma
Nat Commun. 2024 Feb 24;15(1):1703. doi: 10.1038/s41467-024-45902-y.ABSTRACTFusion-positive rhabdomyosarcoma (FP-RMS) is an aggressive pediatric sarcoma driven primarily by the PAX3-FOXO1 fusion oncogene, for which therapies targeting PAX3-FOXO1 are lacking. Here, we screen 62,643 compounds using an engineered cell line that monitors PAX3-FOXO1 transcriptional activity identifying a hitherto uncharacterized compound, P3FI-63. RNA-seq, ATAC-seq, and docking analyses implicate histone lysine demethylases (KDMs) as its targets. Enzymatic assays confirm the inhibition of multiple KDMs with the highest selectivity for KDM3B. St...
Source: Biomed Res - February 24, 2024 Category: Research Authors: Yong Yean Kim Berkley E Gryder Ranuka Sinniah Megan L Peach Jack F Shern Abdalla Abdelmaksoud Silvia Pomella Girma M Woldemichael Benjamin Z Stanton David Milewski Joseph J Barchi John S Schneekloth Raj Chari Joshua T Kowalczyk Shilpa R Shenoy Jason R Eva Source Type: research
Management of Diaphragm Tumors
Diaphragm tumors are very rare, with secondary tumors being more common than primary tumors. The most common benign primary tumors include lipomas and cysts, and malignant primary tumors include rhabdomyosarcoma and leiomyosarcoma. Endometriosis is the most common benign secondary tumor, followed by malignant tumors with localized spread of disease. In addition, widely metastatic disease has been described. Benign lipomas and cysts can be managed conservatively, but more complex or concerning disease typically requires resection. The diaphragm can often be repaired primarily, though any large defect or tension would indica...
Source: Thoracic Surgery Clinics - February 24, 2024 Category: Cardiovascular & Thoracic Surgery Authors: Marta Engelking, Madhuri Rao Source Type: research
Evaluation of TRIM63 RNA in situ hybridization (RNA-ISH) as a potential biomarker for alveolar soft-part sarcoma (ASPS)
Med Oncol. 2024 Feb 23;41(3):76. doi: 10.1007/s12032-024-02305-9.ABSTRACTAlveolar soft-part sarcoma (ASPS) is a rare soft tissue tumor with a broad morphologic differential diagnosis. While histology and immunohistochemistry can be suggestive, diagnosis often requires exclusion of other entities followed by confirmatory molecular analysis for its characteristic ASPSCR1-TFE3 fusion. Current stain-based biomarkers (such as immunohistochemistry for cathepsin K and TFE3) show relatively high sensitivity but may lack specificity, often showing staining in multiple other entities under diagnostic consideration. Given the discove...
Source: Ann Oncol - February 23, 2024 Category: Cancer & Oncology Authors: Alexander S Taylor Rahul Mannan Liron Pantanowitz Arul M Chinnaiyan Saravana M Dhanasekaran Steven Hrycaj Xuhong Cao May P Chan David Lucas Xiao-Ming Wang Rohit Mehra Source Type: research
Expression of neuroendocrine markers in sinonasal rhabdomyosarcoma of an adult patient: A potential diagnostic pitfall
Indian J Pathol Microbiol. 2023 Nov 9. doi: 10.4103/ijpm.ijpm_722_21. Online ahead of print.NO ABSTRACTPMID:38394430 | DOI:10.4103/ijpm.ijpm_722_21 (Source: Indian Journal of Pathology and Microbiology)
Source: Indian Journal of Pathology and Microbiology - February 23, 2024 Category: Pathology Authors: Sandhya Biswal Zaid Shaikh Dillip K Samal Chappity Preetam Pritinanda Mishra Amit K Adhya Source Type: research
Evaluation of TRIM63 RNA in situ hybridization (RNA-ISH) as a potential biomarker for alveolar soft-part sarcoma (ASPS)
Med Oncol. 2024 Feb 23;41(3):76. doi: 10.1007/s12032-024-02305-9.ABSTRACTAlveolar soft-part sarcoma (ASPS) is a rare soft tissue tumor with a broad morphologic differential diagnosis. While histology and immunohistochemistry can be suggestive, diagnosis often requires exclusion of other entities followed by confirmatory molecular analysis for its characteristic ASPSCR1-TFE3 fusion. Current stain-based biomarkers (such as immunohistochemistry for cathepsin K and TFE3) show relatively high sensitivity but may lack specificity, often showing staining in multiple other entities under diagnostic consideration. Given the discove...
Source: Ann Oncol - February 23, 2024 Category: Cancer & Oncology Authors: Alexander S Taylor Rahul Mannan Liron Pantanowitz Arul M Chinnaiyan Saravana M Dhanasekaran Steven Hrycaj Xuhong Cao May P Chan David Lucas Xiao-Ming Wang Rohit Mehra Source Type: research
Expression of neuroendocrine markers in sinonasal rhabdomyosarcoma of an adult patient: A potential diagnostic pitfall
Indian J Pathol Microbiol. 2023 Nov 9. doi: 10.4103/ijpm.ijpm_722_21. Online ahead of print.NO ABSTRACTPMID:38394430 | DOI:10.4103/ijpm.ijpm_722_21 (Source: Indian Journal of Pathology and Microbiology)
Source: Indian Journal of Pathology and Microbiology - February 23, 2024 Category: Pathology Authors: Sandhya Biswal Zaid Shaikh Dillip K Samal Chappity Preetam Pritinanda Mishra Amit K Adhya Source Type: research
Expression of neuroendocrine markers in sinonasal rhabdomyosarcoma of an adult patient: A potential diagnostic pitfall
Indian J Pathol Microbiol. 2023 Nov 9. doi: 10.4103/ijpm.ijpm_722_21. Online ahead of print.NO ABSTRACTPMID:38394430 | DOI:10.4103/ijpm.ijpm_722_21 (Source: Indian Journal of Pathology and Microbiology)
Source: Indian Journal of Pathology and Microbiology - February 23, 2024 Category: Pathology Authors: Sandhya Biswal Zaid Shaikh Dillip K Samal Chappity Preetam Pritinanda Mishra Amit K Adhya Source Type: research
Expression of neuroendocrine markers in sinonasal rhabdomyosarcoma of an adult patient: A potential diagnostic pitfall
Indian J Pathol Microbiol. 2023 Nov 9. doi: 10.4103/ijpm.ijpm_722_21. Online ahead of print.NO ABSTRACTPMID:38394430 | DOI:10.4103/ijpm.ijpm_722_21 (Source: Indian Journal of Pathology and Microbiology)
Source: Indian Journal of Pathology and Microbiology - February 23, 2024 Category: Pathology Authors: Sandhya Biswal Zaid Shaikh Dillip K Samal Chappity Preetam Pritinanda Mishra Amit K Adhya Source Type: research
