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Presented by Ralph Hruban, M.D. and prepared by Amy Duffield, M.D., Ph.D.
Case 1: This asymptomatic middle-aged female was noted to have freckles on her lips and buccal mucosa
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Question 1 of 1
1. Question
Week 329: Case 1
This asymptomatic middle-aged female was noted to have freckles on her lips and buccal mucosa. An endoscopic ultrasound was performed revealing a cystic mass in the pancreas./images/1A_10_29_07.jpg
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Answer: Intraductal Papillary Mucinous Neoplasm
Histology: This intraductal neoplasm is composed of tall, finger-like, papillae lined by tall columnar cells with abundant intracellular and extra-cellular mucin production.
Discussion: Freckles on the lips and buccal mucosa are a feature of the Peutz-Jeghers Syndrome, and, indeed, this patient was found to have Peutz-Jeghers. The Peutz-Jeghers Syndrome is caused by inherited (germ line) mutations in the STK11/LKB1 gene on chromosome 19. This gene codes for a serine-threonine kinase. Patients with the Peutz-Jeghers Syndrome have a significantly increased risk of cancer, including a hundred-fold increased risk of pancreatic cancer (see reference 1). This patient underwent endoscopic ultrasound screening because of this cancer risk. Some of these patients, as in the case presented here, the patients develop an intraductal papillary mucinous neoplasm (reference 2).
Reference(s):
– Hearle, N. Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res. 2006 May 15;12(10):3209-15.
– Su, G. Germline and somatic mutations of the STK11/LKB1 Peutz-Jeghers gene in pancreatic and biliary cancers. Am J Pathol. 1999 Jun;154(6):1835-40.Incorrect
Answer: Intraductal Papillary Mucinous Neoplasm
Histology: This intraductal neoplasm is composed of tall, finger-like, papillae lined by tall columnar cells with abundant intracellular and extra-cellular mucin production.
Discussion: Freckles on the lips and buccal mucosa are a feature of the Peutz-Jeghers Syndrome, and, indeed, this patient was found to have Peutz-Jeghers. The Peutz-Jeghers Syndrome is caused by inherited (germ line) mutations in the STK11/LKB1 gene on chromosome 19. This gene codes for a serine-threonine kinase. Patients with the Peutz-Jeghers Syndrome have a significantly increased risk of cancer, including a hundred-fold increased risk of pancreatic cancer (see reference 1). This patient underwent endoscopic ultrasound screening because of this cancer risk. Some of these patients, as in the case presented here, the patients develop an intraductal papillary mucinous neoplasm (reference 2).
Reference(s):
– Hearle, N. Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res. 2006 May 15;12(10):3209-15.
– Su, G. Germline and somatic mutations of the STK11/LKB1 Peutz-Jeghers gene in pancreatic and biliary cancers. Am J Pathol. 1999 Jun;154(6):1835-40.
