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Presented by Ralph Hruban, M.D. and prepared by Alex Chang, M.D.
Case 3: This adult patient with a remote history of a nephrectomy was found to have a hypervascular enhancing solid mass in the pancreas.
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1. Question
Week 423: Case 3
This adult patient with a remote history of a nephrectomy was found to have a hypervascular enhancing solid mass in the pancreas.Correct
Answer: Renal cell carcinoma metastatic to the pancreas
Histology: This is a solid cellular neoplasm. Immunolabeling for Pax8 was positive.
Discussion: A variety of neoplasms in the pancreas can have clear cell features. These include serous cystadenomas, pancreatic endocrine neoplasms (especially in patients with von Hippel Lindau syndrome), metastatic renal cell carcinoma, solid-pseudopapillary neoplasm, and, more rarely clear cell change in an adenocarcinoma. This differential can be particularly problematic in patients with von Hippel-Lindau syndrome, as they are predisposed to develop both renal cell carcinoma and pancreatic endocrine neoplasms with clear cell change. A panel of immunostains is often needed to establish the diagnosis. Pancreatic endocrine neoplasms strongly and diffusely express synaptophysin and chromogranin, solid-pseudopapillary neoplasms express CD10 and have an abnormal nuclear pattern of labeling with antibodies to beta-catenin, renal cell carcinomas express Pax8 and RCC, and serous cystic neoplasms are usually easy to diagnose without stains.
Incorrect
Answer: Renal cell carcinoma metastatic to the pancreas
Histology: This is a solid cellular neoplasm. Immunolabeling for Pax8 was positive.
Discussion: A variety of neoplasms in the pancreas can have clear cell features. These include serous cystadenomas, pancreatic endocrine neoplasms (especially in patients with von Hippel Lindau syndrome), metastatic renal cell carcinoma, solid-pseudopapillary neoplasm, and, more rarely clear cell change in an adenocarcinoma. This differential can be particularly problematic in patients with von Hippel-Lindau syndrome, as they are predisposed to develop both renal cell carcinoma and pancreatic endocrine neoplasms with clear cell change. A panel of immunostains is often needed to establish the diagnosis. Pancreatic endocrine neoplasms strongly and diffusely express synaptophysin and chromogranin, solid-pseudopapillary neoplasms express CD10 and have an abnormal nuclear pattern of labeling with antibodies to beta-catenin, renal cell carcinomas express Pax8 and RCC, and serous cystic neoplasms are usually easy to diagnose without stains.