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Presented by Ralph Hruban, M.D. and prepared by Joseph J. Maleszewski, M.D.
Case 3: This patient presented with vague abdominal pain.
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1. Question
Week 385: Case 3
This patient presented with vague abdominal pain. CT revealed a mass in the tail of the pancreas. The mass was resected.images/jjm031709/case3a.jpg
images/jjm031709/case3b.jpg
images/jjm031709/case3c.jpg
images/jjm031709/case3d.jpgCorrect
Answer: Well-differentiated pancreatic endocrine neoplasm
Histology: This neoplasm is composed of polygonal cells with abundant clear, almost foamy, cytoplasm. The nuclei are round and uniform, and have “salt and pepper” chromatin. Immunolabeling for synaptophysin was strongly positive, while renal cell markers (RCCa and Pax8) were negative.
Discussion: A number of neoplasms in the pancreas can have clear cells. These include the serous cystadenoma, renal cell carcinoma metastatic to the pancreas, and well-differentiated endocrine neoplasms with clear cell change. Serous cystic neoplasms are usually fairly obvious, with their cuboidal glycogen-rich cells and uniform round nuclei with evenly dispersed chromatin. The distinction between a metastatic renal cell carcinoma and well-differentiated endocrine neoplasm with clear cell change can be significantly more challenging, particularly in patients with the von Hippel-Lindau syndrome. In many instances a panel of immunostains is needed to distinguish among these entities. RCCa and Pax-8 will label renal cell carcinoma metatstaic to the pancreas, while synaptophysin and chromogranin will label well-differentiated pancreatic endocrine neoplasms. A PAS stain with and without diastase can help identify the abundant glycogen in the serous cystic neoplasm.
Reference(s):
– Hoang MP, Hruban RH, Albores-Saavedra J. Clear cell endocrine pancreatic tumor mimicking renal cell carcinoma: a distinctive neoplasm of von Hippel-Lindau disease. Am J Surg Pathol. 2001 May;25(5):602-9.Incorrect
Answer: Well-differentiated pancreatic endocrine neoplasm
Histology: This neoplasm is composed of polygonal cells with abundant clear, almost foamy, cytoplasm. The nuclei are round and uniform, and have “salt and pepper” chromatin. Immunolabeling for synaptophysin was strongly positive, while renal cell markers (RCCa and Pax8) were negative.
Discussion: A number of neoplasms in the pancreas can have clear cells. These include the serous cystadenoma, renal cell carcinoma metastatic to the pancreas, and well-differentiated endocrine neoplasms with clear cell change. Serous cystic neoplasms are usually fairly obvious, with their cuboidal glycogen-rich cells and uniform round nuclei with evenly dispersed chromatin. The distinction between a metastatic renal cell carcinoma and well-differentiated endocrine neoplasm with clear cell change can be significantly more challenging, particularly in patients with the von Hippel-Lindau syndrome. In many instances a panel of immunostains is needed to distinguish among these entities. RCCa and Pax-8 will label renal cell carcinoma metatstaic to the pancreas, while synaptophysin and chromogranin will label well-differentiated pancreatic endocrine neoplasms. A PAS stain with and without diastase can help identify the abundant glycogen in the serous cystic neoplasm.
Reference(s):
– Hoang MP, Hruban RH, Albores-Saavedra J. Clear cell endocrine pancreatic tumor mimicking renal cell carcinoma: a distinctive neoplasm of von Hippel-Lindau disease. Am J Surg Pathol. 2001 May;25(5):602-9.
