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Presented by Dr. Elizabeth Thompson and prepared by Dr. Monica Butcher
This a 67 year old man with a large mass arising from the anterior aspect of the pancreas with associated bulky regional lymphadenopathy and evidence of liver metastases.
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1. Question
This a 67 year old man with a large mass arising from the anterior aspect of the pancreas with associated bulky regional lymphadenopathy and evidence of liver metastases.
Diagnosis:
Correct
Answer: C
Histologic description: H&E sections from the biopsy show a neoplasm with predominantly tubular-type architecture infiltrating through fibrous tissue. In some areas the tubules appear more like acini and in others look more glandular. No definite mucin is identified on H&E but many of the tubules contain eosinophilic material/concretions. Prominent nucleoli are identified in some nuclei. Immunostains demonstrate that the tumor cells are positive for cytokeratin AE1/3, BCL10, and chymotrypsin and negative for synaptophysin, TTF-1, CDX2, CK7, CK20, and NKX3.1. The morphology and immunoprofile show that this is an acinar cell carcinoma of the pancreas (ACC).
Differential Diagnosis: The differential diagnosis is predominantly with other solid cellular neoplasms of the pancreas, including neuroendocrine neoplasms and solid-pseudopapillary neoplasms. However, acinar cell carcinomas with a more tubular appearance due to extensive acini formation can also mimic adenocarcinomas. As metastases to the pancreas are fairly rare, the main diagnostic consideration would be a primary pancreatic ductal adenocarcinoma, which would be expected to show intracellular mucin, label for CK7 and would be negative for exocrine enzymes and surrogates (BCL10, trypsin, chymotrypsin). Dpc4/Smad4 is lost in 60% of pancreatic ductal adenocarcinomas, but caution should be used in interpreting this IHC marker when ACC is on the differential as a small portion of ACC have been reported to also show Dpc4/Smad4 loss. Given the acinar formation and prominent nucleoli, metastatic prostatic adenocarcinoma could mimic ACC, but expression of NKX3.1, PSA, and P501s and lack of BCL10, trypsin and chymotrypsin labeling can distinguish these entities. Neuroendocrine neoplasms can be identified by their expression of synaptophysin and chromogranin and lack of labeling for BCL10, trypsin and chymotrypsin. However, ACC can also express neuroendocrine markers and neuroendocrine labeling alone cannot exclude an ACC. ACC with over 30% of cells showing neuroendocrine labeling can be considered mixed acinar-neuroendocrine neoplasms, however these tumors show similar genetic profile and clinical behavior to ACC and should best be considered as a sub-type of ACC.
Incorrect
Answer: C
Histologic description: H&E sections from the biopsy show a neoplasm with predominantly tubular-type architecture infiltrating through fibrous tissue. In some areas the tubules appear more like acini and in others look more glandular. No definite mucin is identified on H&E but many of the tubules contain eosinophilic material/concretions. Prominent nucleoli are identified in some nuclei. Immunostains demonstrate that the tumor cells are positive for cytokeratin AE1/3, BCL10, and chymotrypsin and negative for synaptophysin, TTF-1, CDX2, CK7, CK20, and NKX3.1. The morphology and immunoprofile show that this is an acinar cell carcinoma of the pancreas (ACC).
Differential Diagnosis: The differential diagnosis is predominantly with other solid cellular neoplasms of the pancreas, including neuroendocrine neoplasms and solid-pseudopapillary neoplasms. However, acinar cell carcinomas with a more tubular appearance due to extensive acini formation can also mimic adenocarcinomas. As metastases to the pancreas are fairly rare, the main diagnostic consideration would be a primary pancreatic ductal adenocarcinoma, which would be expected to show intracellular mucin, label for CK7 and would be negative for exocrine enzymes and surrogates (BCL10, trypsin, chymotrypsin). Dpc4/Smad4 is lost in 60% of pancreatic ductal adenocarcinomas, but caution should be used in interpreting this IHC marker when ACC is on the differential as a small portion of ACC have been reported to also show Dpc4/Smad4 loss. Given the acinar formation and prominent nucleoli, metastatic prostatic adenocarcinoma could mimic ACC, but expression of NKX3.1, PSA, and P501s and lack of BCL10, trypsin and chymotrypsin labeling can distinguish these entities. Neuroendocrine neoplasms can be identified by their expression of synaptophysin and chromogranin and lack of labeling for BCL10, trypsin and chymotrypsin. However, ACC can also express neuroendocrine markers and neuroendocrine labeling alone cannot exclude an ACC. ACC with over 30% of cells showing neuroendocrine labeling can be considered mixed acinar-neuroendocrine neoplasms, however these tumors show similar genetic profile and clinical behavior to ACC and should best be considered as a sub-type of ACC.