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  #1  
Old 02-26-2011
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Arrow Test your knowledge #22! (Pathology)

A twelve-year-old male presents with perirectal bleeding. Physical examination reveals multiple small brown lentigines about his mouth as well as blueish macules on his gums and inside his lips. The boy’s mother reports that the latter “runs in the family”. Which hamartomatous polyposis syndrome is likely, and what is a worrisome correlated risk?
  1. Cowden syndrome; increased frequency of malignant tumours
  2. Cronkhite-Canada syndrome; intussusception, prolapse, malignancy of polyps
  3. Juvenile polyposis; increased risk of colonic adenocarcinoma
  4. Peutz-Jeghers syndrome; increased risk of pancreatic, breast, lung, and adnexal tumours
  5. Tuberous sclerosis; development of neoplasias in any organ
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  #2  
Old 02-26-2011
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Correct answer is D.

Peutz-Jeghers syndrome, autosomal-dominant disease with multiple non-malignant/non-premalignant hamartomas in the GI tract, including small intestine. Hyperpigmented lesions in mouth/lips are classic in PJS. There's increase risk of colon cancer and other malignancies. Also associated with Serine-Threonine Kinase II defect.

Keep the good work!
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  #3  
Old 02-26-2011
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I think answer is D.

None of the others have characteristic mucocutaneous lesions as their symptom. Not sure though, but I'll stick to answer D
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  #4  
Old 02-26-2011
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Default i go wid ans D..

,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,
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Old 02-26-2011
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Add my D as well.
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Old 02-26-2011
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I have no idea!!!
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Peutz-Jeghers syndrome; increased risk of pancreatic, breast, lung, and adnexal tumours
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Correct Answer Correct!

Answer: D. Peutz-Jeghers syndrome, increased risk of pancreatic, breast, lung, and adnexal tumours. As jorgemorales noted, these lesions are classic for PJS, and might be the clue that gives you the question quickly!
Test your knowledge #22! (Pathology)-169972-182006-124.jpg
click image to enlarge
  • Blue-to-dark-brown macules on buccal mucosa are a typical finding of P-J, and the median age of presentation is 11 years.
  • All of the answers are syndromes of hamartomatous polyposis.
  • All of the disease/sequelae pairings are correct
  • All of the answers are autosomal-dominant, except for Cronkhite-Canada, which is not inherited
  • Laugier-Hunziker syndrome presents with many of the same dermatological findings as P-J, but without polyps; the pathology of L-H is limited to hyperpigmentation.
  • Familial adenomatous polyposis, while not typically presenting with blue mucosal macules, is more common and might be considered.
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  #9  
Old 02-26-2011
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Quote:
Originally Posted by Mondoshawan View Post
Answer: D. Peutz-Jeghers syndrome, increased risk of pancreatic, breast, lung, and adnexal tumours. As jorgemorales noted, these lesions are classic for PJS, and might be the clue that gives you the question quickly!
Attachment 1066
click image to enlarge
  • Blue-to-dark-brown macules on buccal mucosa are a typical finding of P-J, and the median age of presentation is 11 years.
  • All of the answers are syndromes of hamartomatous polyposis.
  • All of the disease/sequelae pairings are correct
  • All of the answers are autosomal-dominant, except for Cronkhite-Canada, which is not inherited
  • Laugier-Hunziker syndrome presents with many of the same dermatological findings as P-J, but without polyps; the pathology of L-H is limited to hyperpigmentation.
  • Familial adenomatous polyposis, while not typically presenting with blue mucosal macules, is more common and might be considered.
I just had a related question, is it okay to generalize and say that Polyp's will be benign, and never have malignant potential?

I remember someone stating something like that in otorhinolaryngology where they had nasal polyp's.
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Quote:
Originally Posted by patelMD View Post
I just had a related question, is it okay to generalize and say that Polyp's will be benign, and never have malignant potential?

I remember someone stating something like that in otorhinolaryngology where they had nasal polyp's.
I think that we cannot make that assumption. Maybe in practice nasal and small bowel polyps are rarely malignant, but colonic polyps can certainly be premalignant (viz. FAP above, or the polyp found on a colonoscopy that must always get sent to pathology). Also, we are in the weird and rarified context of Step 1, where the incidence of rare diseases is 1 in every 3 questions!
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Quote:
Originally Posted by patelMD View Post
I just had a related question, is it okay to generalize and say that Polyp's will be benign, and never have malignant potential?

I remember someone stating something like that in otorhinolaryngology where they had nasal polyp's.
Adding to what Mondoshawan said, we were discussing specifically hamartomatous polyps and not adenomatous polyps. To my understanding, hamartomatous polyps are normal tissue outgrowings without INCREASED potential for malignancy. This does not discard the possibility of malignant transformation, but the likelyhood would be similar to that of any other normal tissue. As someone said before, in medicine 2 plus 2 is not always 4...
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Old 02-29-2012
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this is a great question!... just wondering why they didn't add the risk for CRC in the peutz jeghers option, which is the correct one. It has a 66% chance of progressing to CRC.
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Quote:
Originally Posted by LatinGeorge View Post
this is a great question!... just wondering why they didn't add the risk for CRC in the peutz jeghers option, which is the correct one. It has a 66% chance of progressing to CRC.
I left it out because I was thinking of the things that differentiated the syndromes, but you're right about increased incidence of GI and other cancers! This from Emedicine:
Quote:
Cancer is the main consequence of Peutz-Jeghers syndrome (PJS) as patients age. The major sites of cancer occurrence, in order of relative risk (RR) over the general population, are as follows:
  • Small intestine - RR 520
  • Stomach - RR 96
  • Pancreas - RR 132
  • Colon - RR 84
  • Esophagus - RR 57
  • Ovary - RR 27
  • Lung - RR 17
  • Uterus - RR 16
  • Breast - RR 15.2
In addition, other reproductive site cancers have been associated with PJS, including adenoma malignum of the cervix, Sertoli cell tumors, and sex cord tumors with annular tubules.

The cumulative risk of developing any cancers associated with PJS in patients aged 15-64 years is 93%. The cumulative risks of developing particular cancers from ages 15-64 years are as follows:
  • Esophagus - 0.5%
  • Stomach - 29%
  • Small intestine - 13%
  • Colon - 39%
  • Pancreas - 36%
  • Lung - 15%
  • Testes - 9%
  • Breast - 54%
  • Uterus - 9%
  • Ovary - 21%
  • Cervix - 10%
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Agree man, if you read it from FA for example, it only makes you think of CRC, its good to know the other risks associated, but the most important clue will always be the hyperpigmentation + cancer risk.
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Quote:
Originally Posted by Mondoshawan View Post
Answer: D. Peutz-Jeghers syndrome, increased risk of pancreatic, breast, lung, and adnexal tumours. As jorgemorales noted, these lesions are classic for PJS, and might be the clue that gives you the question quickly!
Attachment 1066
click image to enlarge
  • Blue-to-dark-brown macules on buccal mucosa are a typical finding of P-J, and the median age of presentation is 11 years.
  • All of the answers are syndromes of hamartomatous polyposis.
  • All of the disease/sequelae pairings are correct
  • All of the answers are autosomal-dominant, except for Cronkhite-Canada, which is not inherited
  • Laugier-Hunziker syndrome presents with many of the same dermatological findings as P-J, but without polyps; the pathology of L-H is limited to hyperpigmentation.
  • Familial adenomatous polyposis, while not typically presenting with blue mucosal macules, is more common and might be considered.
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Old 02-29-2012
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colon polyps + oral hyperpigmentation/ulcers = peutz-jegher.
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