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
Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathologic...Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology,genetics,and prognosis.Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels.These tumours are rare,with about 75 cases reported in the literature.Most anastomosing haemangiomas are found incidentally on ultrasound,computed tomography,or magnetic resonance imaging.Common symptoms include abdominal pain,haematuria,and abdominal mass.Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes.The prognosis of anastomosing haemangioma is excellent.Primary renal angiosarcomas are malignant tumours showing endothelial differentiation.To date,76 cases have been described in the literature.Primary renal angiosarcomas are frequently symptomatic.The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas,including abdominal pain,haematuria,and abdominal mass.Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR,TIE1,SNRK,TEK,and FLT1 are upregulated in angiosarcomas.Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment,chemotherapy,radiotherapy,or targeted therapy.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.
文摘Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology,genetics,and prognosis.Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels.These tumours are rare,with about 75 cases reported in the literature.Most anastomosing haemangiomas are found incidentally on ultrasound,computed tomography,or magnetic resonance imaging.Common symptoms include abdominal pain,haematuria,and abdominal mass.Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes.The prognosis of anastomosing haemangioma is excellent.Primary renal angiosarcomas are malignant tumours showing endothelial differentiation.To date,76 cases have been described in the literature.Primary renal angiosarcomas are frequently symptomatic.The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas,including abdominal pain,haematuria,and abdominal mass.Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR,TIE1,SNRK,TEK,and FLT1 are upregulated in angiosarcomas.Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment,chemotherapy,radiotherapy,or targeted therapy.
