Blastomas,characterized by a mixture of mesenchymal,epithelial,and undifferentiated blastematous components,are rare malignant neoplasms originating from precursor blast cells.This review focuses on digestive system b...Blastomas,characterized by a mixture of mesenchymal,epithelial,and undifferentiated blastematous components,are rare malignant neoplasms originating from precursor blast cells.This review focuses on digestive system blastomas in adult patients,including gastroblastoma,hepatoblastoma,and pancreatoblastoma.Gastroblastoma is a biphasic,epitheliomesenchymal tumor,with only sixteen cases reported to date.In addition to the characteristic histology,metastasisassociated lung adenocarcinoma transcript 1-glioma-associated oncogene homolog 1 gene fusion is typical,although recently novel ewing sarcoma breakpoint region 1-c-terminal binding protein 1 and patched 1-glioma-associated oncogene homolog 2 fusions have been described.Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty.Pancreatoblastoma,primarily a pediatric tumor,displays acinar differentiation and squamoid nests with other lines of differentiation also present,especially neuroendocrine.Diagnostic approaches for these blastomas include a combination of imaging modalities,histopathological examination,and molecular profiling.The treatment generally involves surgical resection,which may be supplemented by chemotherapy or radiotherapy in some cases.Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes,particularly in the setting of metastases.This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.展开更多
BACKGROUND Drug-induced liver injury(DILI)and herbal/dietary supplements(HDS)related liver injury present unique diagnostic challenges.Collaboration between the clinician and the pathologist is required for an accurat...BACKGROUND Drug-induced liver injury(DILI)and herbal/dietary supplements(HDS)related liver injury present unique diagnostic challenges.Collaboration between the clinician and the pathologist is required for an accurate diagnosis and management.AIM To report our experience on the clinical-pathological findings of hepatic injury caused by drugs/HDS.METHODS A retrospective review of clinically proven cases of DILI/HDS who presented to our institution from January 1,2013 to December 31,2017 was performed.Slides were reviewed for histopathological patterns of injury and correlated with the causative agent.Out of 600 patients presenting with unexplained rise in liver enzymes undergoing biopsy,107 were suspected to have DILI/HDS.Of these,53 had a directly linked exposure to drug/herbal supplements.Fifteen patients were excluded for concurrent known liver disease.Thirty-eight patients with clinically proven DILI/HDS were finally included.RESULTS Thirty-eight cases of DILI/HDS with a male:female of 1:1.5 and mean age of 51±3 years were identified.DILI was identified in 84.2%cases while HDS injury in 15.8%.Acute hepatitis(42.1%)was the most common pattern of injury while granulomatous hepatitis(2.6%)was the least common.We found one case of acute-cholestasis due to rivaroxaban and two cases of cholestatic-hepatitis due to rizatriptan and trimethobenzamide-hydrochloride that,to the best of our knowledge,have not been previously reported.One case of steatohepatitis due to trimethoprim-sulfamethoxazole and three unusual cases of cholestatic-hepatitis with bile duct injury and steatosis due to dronedarone,C4-Extreme and hydroxycut,were also seen.Of our cohort,81.6%of the patients fared well with discontinuation of drug and 18.4%underwent transplant;of which 42.9%were deceased.CONCLUSION We describe the clinical findings,histopathological patterns of injury and clinical outcomes caused by drugs.In particular,we report a few previously unreported/rarely observed clinical and histopathological patterns of hepatic injury.展开更多
文摘Blastomas,characterized by a mixture of mesenchymal,epithelial,and undifferentiated blastematous components,are rare malignant neoplasms originating from precursor blast cells.This review focuses on digestive system blastomas in adult patients,including gastroblastoma,hepatoblastoma,and pancreatoblastoma.Gastroblastoma is a biphasic,epitheliomesenchymal tumor,with only sixteen cases reported to date.In addition to the characteristic histology,metastasisassociated lung adenocarcinoma transcript 1-glioma-associated oncogene homolog 1 gene fusion is typical,although recently novel ewing sarcoma breakpoint region 1-c-terminal binding protein 1 and patched 1-glioma-associated oncogene homolog 2 fusions have been described.Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty.Pancreatoblastoma,primarily a pediatric tumor,displays acinar differentiation and squamoid nests with other lines of differentiation also present,especially neuroendocrine.Diagnostic approaches for these blastomas include a combination of imaging modalities,histopathological examination,and molecular profiling.The treatment generally involves surgical resection,which may be supplemented by chemotherapy or radiotherapy in some cases.Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes,particularly in the setting of metastases.This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.
文摘BACKGROUND Drug-induced liver injury(DILI)and herbal/dietary supplements(HDS)related liver injury present unique diagnostic challenges.Collaboration between the clinician and the pathologist is required for an accurate diagnosis and management.AIM To report our experience on the clinical-pathological findings of hepatic injury caused by drugs/HDS.METHODS A retrospective review of clinically proven cases of DILI/HDS who presented to our institution from January 1,2013 to December 31,2017 was performed.Slides were reviewed for histopathological patterns of injury and correlated with the causative agent.Out of 600 patients presenting with unexplained rise in liver enzymes undergoing biopsy,107 were suspected to have DILI/HDS.Of these,53 had a directly linked exposure to drug/herbal supplements.Fifteen patients were excluded for concurrent known liver disease.Thirty-eight patients with clinically proven DILI/HDS were finally included.RESULTS Thirty-eight cases of DILI/HDS with a male:female of 1:1.5 and mean age of 51±3 years were identified.DILI was identified in 84.2%cases while HDS injury in 15.8%.Acute hepatitis(42.1%)was the most common pattern of injury while granulomatous hepatitis(2.6%)was the least common.We found one case of acute-cholestasis due to rivaroxaban and two cases of cholestatic-hepatitis due to rizatriptan and trimethobenzamide-hydrochloride that,to the best of our knowledge,have not been previously reported.One case of steatohepatitis due to trimethoprim-sulfamethoxazole and three unusual cases of cholestatic-hepatitis with bile duct injury and steatosis due to dronedarone,C4-Extreme and hydroxycut,were also seen.Of our cohort,81.6%of the patients fared well with discontinuation of drug and 18.4%underwent transplant;of which 42.9%were deceased.CONCLUSION We describe the clinical findings,histopathological patterns of injury and clinical outcomes caused by drugs.In particular,we report a few previously unreported/rarely observed clinical and histopathological patterns of hepatic injury.