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Presented by Ralph Hruban, M.D. and prepared by Doreen Nguyen, M.D.
Case 1: This 40 some year old patient with a family history of cancer, was found to have a mass in the pancreas.
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Question 1 of 1
1. Question
Week 562: Case 1
This 40 some year old patient with a family history of cancer, was found to have a mass in the pancreas (images 4 and 5) and in their kidney (images 1-3). Both lesions were biopsied.images/D Nguyen/8-12-13/case 1/kidney_4x_450 pixels.jpg
images/D Nguyen/8-12-13/case 1/kidney_10x_450 pixels.jpg
images/D Nguyen/8-12-13/case 1/kidney_20x_450 pixels.jpg
images/D Nguyen/8-12-13/case 1/pancreas_4x_450 pixels.jpg
images/D Nguyen/8-12-13/case 1/pancreas_10x_450 pixels.jpgCorrect
Answer: Renal cell carcinoma (kidney) and serous cystadenoma (pancreas)
Histology: Both lesions have a clear morphology and are richly vascular. The lesion in the pancreas forms small cysts and the lesional cells are cuboidal, with optically clear cytoplasm and uniform round nuclei.
Discussion: The biopsies in this patient reveal a renal cell carcinoma in the kidney, and a serous cystadenoma of the pancreas. The combination of these two lesions should suggest the possibility of von Hippel Lindau (VHL) Syndrome, and, indeed, this patient was found to have VHL. VHL is caused by a germline mutation in the VHL gene on chromosome 3p, and the syndrome is characterized by hemangioblastomas of the brain and retina, clear cell renal cell carcinoma, pheochromocytoma, serous cystic neoplasms and well-differentiated neuroendocrine tumors of the pancreas, epidydimal papillary cystadenomas and endolymphatic sac tumors.
Incorrect
Answer: Renal cell carcinoma (kidney) and serous cystadenoma (pancreas)
Histology: Both lesions have a clear morphology and are richly vascular. The lesion in the pancreas forms small cysts and the lesional cells are cuboidal, with optically clear cytoplasm and uniform round nuclei.
Discussion: The biopsies in this patient reveal a renal cell carcinoma in the kidney, and a serous cystadenoma of the pancreas. The combination of these two lesions should suggest the possibility of von Hippel Lindau (VHL) Syndrome, and, indeed, this patient was found to have VHL. VHL is caused by a germline mutation in the VHL gene on chromosome 3p, and the syndrome is characterized by hemangioblastomas of the brain and retina, clear cell renal cell carcinoma, pheochromocytoma, serous cystic neoplasms and well-differentiated neuroendocrine tumors of the pancreas, epidydimal papillary cystadenomas and endolymphatic sac tumors.
