BACKGROUND Adolescent/adult pancreatoblastoma(PB)is an uncommon malignant pancreatic tumor.The paucity of data stemming from the rarity of this disease leads to minimal generalized guidelines regarding its diagnosis a...BACKGROUND Adolescent/adult pancreatoblastoma(PB)is an uncommon malignant pancreatic tumor.The paucity of data stemming from the rarity of this disease leads to minimal generalized guidelines regarding its diagnosis and treatment.There is a limited number of case reports in the literature and there has been no recent analysis of the literature to consolidate their common features.The purpose of the featured study is to review the available cases of adolescent/adult PB and analyze the common genetic features,histologic features,treatment regimens,tumor sizes,tumor locations,and areas of metastasis to advance ongoing research and better understand and treat this rare condition.AIM To present a patient case and systematically review all available cases in the literature to consolidate the common physical,genetic,and histologic features of PB.METHODS This is a systematic review of the literature with a case study.A total of 89 patient cases were discovered in the literature database for adolescent/adult PB,all of which were reviewed and are included in our research.Patients aged 16-18 were considered adolescent and patients aged greater than 18 were considered adult.Adolescents and adults were grouped together for the purpose of this study.The patient from the case report was seen in a community hospital setting.RESULTS The 89 cases analyzed from the literature were found in 51 references(our case report included),which were consolidated into the six categories mentioned above.A plurality of references reports PB in the head of the pancreas,4.0-10.0 cm in size,and with the most common site of metastasis to the liver.Histology studies most commonly included acinar groups,squamous corpuscles/nests,cytokeratin,chromogranin,trypsin,chymotrypsin,and synaptophysin.Genetic studies most commonly included adenomatous polyposis coli,B-cell lymphoma/leukemia 10,catenin beta 1,and Wnt/beta-catenin mutations.The mainstay of treatment was surgery with chemotherapy typically including cisplatin,carboplatin,doxorubicin,5 fluorouracil,mitomycin,bleomycin,gemcitabine,and vindesine.Radiation was also often used.CONCLUSION Common pancreatoblastoma features include acinar groups,chromogranin,chymotrypsin,squamous corpuscles,synaptophysin and trypsin on histology and adenomatous polyposis coli,B-cell lymphoma/leukemia 10,catenin beta 1,and Wnt/beta-catenin genetic mutations.展开更多
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
Pediatric pancreatic tumors,though rare,pose significant diagnostic and manage-ment challenges.The recent,22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into...Pediatric pancreatic tumors,though rare,pose significant diagnostic and manage-ment challenges.The recent,22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into this uncommon enti-ty,revealing striking geographical variations and questioning current treatment paradigms.This editorial commentary analyzes the study's key findings,inclu-ding the predominance of solid pseudopapillary neoplasms and their younger age of onset,which contrast sharply with Western data.It explores the implications for clinical practice and research,emphasizing the need for population-specific approaches to diagnosis and treatment.The revealed limited institutional expe-rience and surgical management patterns prompt a reevaluation of optimal care delivery for these complex cases,suggesting benefits of centralizing healthcare services.Furthermore,the commentary advocates for international collaborative studies to elucidate the genetic,environmental,and lifestyle factors influencing the development and progression of pediatric pancreatic tumors across diverse populations.It also outlines future directions,calling for advancements in precision medicine and innovative care delivery models to improve global patient outcomes.Unraveling Makita et al's findings within the broader landscape of pediatric oncology can stimulate further research and clinical advancements in managing pancreatic and other rare tumors in children.展开更多
基金Supported by the Honor Health Foundation,Michael and Mary Ellen Francisthe Seena Magowitz Foundation.
文摘BACKGROUND Adolescent/adult pancreatoblastoma(PB)is an uncommon malignant pancreatic tumor.The paucity of data stemming from the rarity of this disease leads to minimal generalized guidelines regarding its diagnosis and treatment.There is a limited number of case reports in the literature and there has been no recent analysis of the literature to consolidate their common features.The purpose of the featured study is to review the available cases of adolescent/adult PB and analyze the common genetic features,histologic features,treatment regimens,tumor sizes,tumor locations,and areas of metastasis to advance ongoing research and better understand and treat this rare condition.AIM To present a patient case and systematically review all available cases in the literature to consolidate the common physical,genetic,and histologic features of PB.METHODS This is a systematic review of the literature with a case study.A total of 89 patient cases were discovered in the literature database for adolescent/adult PB,all of which were reviewed and are included in our research.Patients aged 16-18 were considered adolescent and patients aged greater than 18 were considered adult.Adolescents and adults were grouped together for the purpose of this study.The patient from the case report was seen in a community hospital setting.RESULTS The 89 cases analyzed from the literature were found in 51 references(our case report included),which were consolidated into the six categories mentioned above.A plurality of references reports PB in the head of the pancreas,4.0-10.0 cm in size,and with the most common site of metastasis to the liver.Histology studies most commonly included acinar groups,squamous corpuscles/nests,cytokeratin,chromogranin,trypsin,chymotrypsin,and synaptophysin.Genetic studies most commonly included adenomatous polyposis coli,B-cell lymphoma/leukemia 10,catenin beta 1,and Wnt/beta-catenin mutations.The mainstay of treatment was surgery with chemotherapy typically including cisplatin,carboplatin,doxorubicin,5 fluorouracil,mitomycin,bleomycin,gemcitabine,and vindesine.Radiation was also often used.CONCLUSION Common pancreatoblastoma features include acinar groups,chromogranin,chymotrypsin,squamous corpuscles,synaptophysin and trypsin on histology and adenomatous polyposis coli,B-cell lymphoma/leukemia 10,catenin beta 1,and Wnt/beta-catenin genetic mutations.
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Pediatric pancreatic tumors,though rare,pose significant diagnostic and manage-ment challenges.The recent,22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into this uncommon enti-ty,revealing striking geographical variations and questioning current treatment paradigms.This editorial commentary analyzes the study's key findings,inclu-ding the predominance of solid pseudopapillary neoplasms and their younger age of onset,which contrast sharply with Western data.It explores the implications for clinical practice and research,emphasizing the need for population-specific approaches to diagnosis and treatment.The revealed limited institutional expe-rience and surgical management patterns prompt a reevaluation of optimal care delivery for these complex cases,suggesting benefits of centralizing healthcare services.Furthermore,the commentary advocates for international collaborative studies to elucidate the genetic,environmental,and lifestyle factors influencing the development and progression of pediatric pancreatic tumors across diverse populations.It also outlines future directions,calling for advancements in precision medicine and innovative care delivery models to improve global patient outcomes.Unraveling Makita et al's findings within the broader landscape of pediatric oncology can stimulate further research and clinical advancements in managing pancreatic and other rare tumors in children.
