Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known...Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known about its clinical and pathologic features.This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis,treatment,and follow-up.Although pancreatoblastoma commonly occurs in childhood,there have now been more than 70 adult pancreatoblastomas described in the literature.There is a slight male predominance.There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic.The most common presenting symptom is abdominal pain.Grossly,the tumours are often large and well-circumscribed.Microscopically,pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests.These tumours are positive for trypsin,chymotrypsin,lipase,and BCL10.Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas.Adult pancreatoblastomas are aggressive tumours with frequent local invasion,recurrence,and distant metastasis.Treatment consists of surgical resection.Chemotherapy and radiotherapy may have a role in the treatment of recurrent,residual,unresectable,and metastatic disease.It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas,solid pseudopapillary neoplasms,and pancreatic neuroendocrine neoplasms.展开更多
Background:Pediatric pancreatoblastoma is an extremely rare malignant tumor,posing diagnostic and treatment difficulties for pediatric surgeons.Using the Surveillance,Epidemiology,and End Results(SEER)database,we pres...Background:Pediatric pancreatoblastoma is an extremely rare malignant tumor,posing diagnostic and treatment difficulties for pediatric surgeons.Using the Surveillance,Epidemiology,and End Results(SEER)database,we present an up-to-date report of the epidemiology,clinicopathological features,survival rates,and prognosis of pancreatoblastoma in pediatric patients.Methods:All pediatric patients diagnosed with pancreatoblastoma between 1975 and 2018 were identified in the SEER regis-tries(SEER 8 registries and SEER 17 registries).We conducted a survival analysis to assess overall survival and 1-and 5-year late mortality rates.Descriptive statistics and log-rank test were performed.Results:A total of 22 children and adolescents with pancreatoblastoma were identified.In this cohort,12 of 22 were male(54.55%),14 were White(63.64%),and 11 were diagnosed between the ages of 1 and 4 years(50.0%).Among the 22 patients,11(50.0%)had distant metastases,whereas 7(31.82%)had localized,and 4(18.18%)had a regional disease.A total of 5 children and adolescents died during the study period,with cumulative survival rates of 14 of 17(82.35%)and 10 of 11(90.95%)among 1-and 5-year survivors,respectively.Cancer-directed surgery was significantly associated with an increased life expectancy(log-rank test,P=.018).Conclusion:Pediatric pancreatoblastoma is a rare entity.Cases that underwent surgery had a greater likelihood of overall survival and reduced late mortality.展开更多
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
文摘Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known about its clinical and pathologic features.This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis,treatment,and follow-up.Although pancreatoblastoma commonly occurs in childhood,there have now been more than 70 adult pancreatoblastomas described in the literature.There is a slight male predominance.There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic.The most common presenting symptom is abdominal pain.Grossly,the tumours are often large and well-circumscribed.Microscopically,pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests.These tumours are positive for trypsin,chymotrypsin,lipase,and BCL10.Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas.Adult pancreatoblastomas are aggressive tumours with frequent local invasion,recurrence,and distant metastasis.Treatment consists of surgical resection.Chemotherapy and radiotherapy may have a role in the treatment of recurrent,residual,unresectable,and metastatic disease.It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas,solid pseudopapillary neoplasms,and pancreatic neuroendocrine neoplasms.
基金Dr Peiyi Li’s research is in part supported by the National Natural Science Foundation of China(Grant No.72207174)Dr Yujia Kong’s research is in part supported by the National Nature Science Foundation of China(Grant No.20003560)+1 种基金Natural Science Foundation of Shandong Province(Grant No.ZR2020MH340)Undergraduate Education Reform Research Project of Shandong Province(Grant No.M2021174).
文摘Background:Pediatric pancreatoblastoma is an extremely rare malignant tumor,posing diagnostic and treatment difficulties for pediatric surgeons.Using the Surveillance,Epidemiology,and End Results(SEER)database,we present an up-to-date report of the epidemiology,clinicopathological features,survival rates,and prognosis of pancreatoblastoma in pediatric patients.Methods:All pediatric patients diagnosed with pancreatoblastoma between 1975 and 2018 were identified in the SEER regis-tries(SEER 8 registries and SEER 17 registries).We conducted a survival analysis to assess overall survival and 1-and 5-year late mortality rates.Descriptive statistics and log-rank test were performed.Results:A total of 22 children and adolescents with pancreatoblastoma were identified.In this cohort,12 of 22 were male(54.55%),14 were White(63.64%),and 11 were diagnosed between the ages of 1 and 4 years(50.0%).Among the 22 patients,11(50.0%)had distant metastases,whereas 7(31.82%)had localized,and 4(18.18%)had a regional disease.A total of 5 children and adolescents died during the study period,with cumulative survival rates of 14 of 17(82.35%)and 10 of 11(90.95%)among 1-and 5-year survivors,respectively.Cancer-directed surgery was significantly associated with an increased life expectancy(log-rank test,P=.018).Conclusion:Pediatric pancreatoblastoma is a rare entity.Cases that underwent surgery had a greater likelihood of overall survival and reduced late mortality.
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.
