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Presented by Ralph Hruban, M.D. and prepared by Matthew Karafin, M.D.
Case 1: This adult patient presented with a large cystic mass in the pancreas.
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1. Question
Week 501: Case 1
This adult patient presented with a large cystic mass in the pancreas. The mass was resected.images/1alex/102411case1image1.jpg
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images/1alex/102411case1image4.jpgCorrect
Answer: Mixed serous and mucinous cystic neoplasm with an associated invasive adenocarcinoma
Histology: This is an extremely unusual case in which two distinct types of epithelia line the same cystic neoplasm. The primary growth pattern is that of a serous cystadenoma and many of the cysts are lined by coidal glycogen rich cells with uniform round nuclei (image 1). However, other locules are lined by mucinous epithelium, as conformed by PAS-d (image 3), and mucin stains (image 2). The foci of mucinous differentiation are not associated with ovarian stroma (ER and PR performed at Johns Hopkins are negative).
Multiple foci of high grade dysplasia are seen throughout the specimen in the areas with mucinous differentiation, and multiple foci of microinvasive adenocarcinoma are seen (image 2).
Discussion: This is a very unusual case that violates one of the principle “rules” of pancreas pathology- one never sees a combined serous and mucinous neoplasm. In fact, separating serous and mucinous cystic neoplasms was the great advance made by Campagno and Ortell more than 30 years ago (Am J Clin Pathol. 1978). This case has both, and also has several microscopic foci of invasive ductal adenocarcinoma. From the overall growth pattern (the formation of back-to-back microcysts) one might speculate that this is a serous cystic neoplasm that was colonized by a mucin-producing neoplasm, but without molecular analyses there is no way to know if this represents one neoplasm with divergent differentiation, or a collision of two tumors.
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Incorrect
Answer: Mixed serous and mucinous cystic neoplasm with an associated invasive adenocarcinoma
Histology: This is an extremely unusual case in which two distinct types of epithelia line the same cystic neoplasm. The primary growth pattern is that of a serous cystadenoma and many of the cysts are lined by coidal glycogen rich cells with uniform round nuclei (image 1). However, other locules are lined by mucinous epithelium, as conformed by PAS-d (image 3), and mucin stains (image 2). The foci of mucinous differentiation are not associated with ovarian stroma (ER and PR performed at Johns Hopkins are negative).
Multiple foci of high grade dysplasia are seen throughout the specimen in the areas with mucinous differentiation, and multiple foci of microinvasive adenocarcinoma are seen (image 2).
Discussion: This is a very unusual case that violates one of the principle “rules” of pancreas pathology- one never sees a combined serous and mucinous neoplasm. In fact, separating serous and mucinous cystic neoplasms was the great advance made by Campagno and Ortell more than 30 years ago (Am J Clin Pathol. 1978). This case has both, and also has several microscopic foci of invasive ductal adenocarcinoma. From the overall growth pattern (the formation of back-to-back microcysts) one might speculate that this is a serous cystic neoplasm that was colonized by a mucin-producing neoplasm, but without molecular analyses there is no way to know if this represents one neoplasm with divergent differentiation, or a collision of two tumors.
Reference(s):
