Answer 1: A. Adenocarcinoma, pancreas.
Answer 2: D. All of the above.
Comment: Peutz-Jeghers syndrome (PJS) is an autosomal dominant disease in which most cases (estimated from 66% to 99%) contain a mutation of STK11/LKB1, a tumor suppressor gene. PJS is typified by gastrointestinal symptoms including obstruction, abdominal pain, rectal bleeding, multiple polyps within the jejunum and colon, and abnormal mucocutaneous pigmentation. Of interest, the intestinal polyps are typically hamartomas. However, these patients also, when compared to the general population, have a 15-fold increased risk of developing intestinal cancers, of which 36% are pancreatic. About 90% to 95% of human pancreatic carcinomas are ductal. Human pancreatic adenocarcinomas have a high mutation rate of the oncogene K-ras and also either overexpression or mutation of the tumor suppressor p53 gene. Of interest, overexpression has been attributed to survival difference but the association is still controversial.
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