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
Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential...Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.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.
文摘Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.
