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Presented by Ralph Hruban, M.D. and prepared by Robert E LeBlanc, M.D.
Case 1: This adult patient presented with a large mass extending from the pancreas to the stomach.
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Question 1 of 1
1. Question
Week 512: Case 1
This adult patient presented with a large mass extending from the pancreas to the stomach. A distal pancreatectomy and partial gastrectomy were performed.images/REL_ONE_1A.jpg
images/REL_ONE_1B.jpg
images/REL_ONE_1C.jpgCorrect
Answer: Gastrointestinal stromal tumor
Histology: This spindle cell neoplasm abuts the pancreas. Note the high mitotic rate and the areas of fibrosis and hyaline degeneration indicating pre-operative treatment effect.
Discussion: Because the pancreas is so intimately associated with the duodenum and stomach, gastrointestinal stromal tumors (GISTs) should be high on the differential diagnosis of a spindle cell neoplasm in the area around the pancreas. The expression of cKIT helps establish the diagnosis in this case. Tumor site, size, and mitotic index are all important prognosticators.
Reference(s):
– Natural history of imatinib-naive GISTs: a retrospective analysis of 929 cases with long-term follow-up and development of a survival nomogram based on mitotic index and size as continuous variables.
– Rossi S, Miceli R, Messerini L, Bearzi I, Mazzoleni G, Capella C, Arrigoni G, Sonzogni A, Sidoni A, Toffolatti L, Laurino L, Mariani L, Vinaccia V, Gnocchi C, Gronchi A, Casali PG, Dei Tos AP. Am J Surg Pathol. 2011 Nov;35(11):1646-56.PMID: 21997685Incorrect
Answer: Gastrointestinal stromal tumor
Histology: This spindle cell neoplasm abuts the pancreas. Note the high mitotic rate and the areas of fibrosis and hyaline degeneration indicating pre-operative treatment effect.
Discussion: Because the pancreas is so intimately associated with the duodenum and stomach, gastrointestinal stromal tumors (GISTs) should be high on the differential diagnosis of a spindle cell neoplasm in the area around the pancreas. The expression of cKIT helps establish the diagnosis in this case. Tumor site, size, and mitotic index are all important prognosticators.
Reference(s):
– Natural history of imatinib-naive GISTs: a retrospective analysis of 929 cases with long-term follow-up and development of a survival nomogram based on mitotic index and size as continuous variables.
– Rossi S, Miceli R, Messerini L, Bearzi I, Mazzoleni G, Capella C, Arrigoni G, Sonzogni A, Sidoni A, Toffolatti L, Laurino L, Mariani L, Vinaccia V, Gnocchi C, Gronchi A, Casali PG, Dei Tos AP. Am J Surg Pathol. 2011 Nov;35(11):1646-56.PMID: 21997685
