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On Adenomas and Hyperplastic Polyposis

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... skeletal, odontogenic & cranio-facial abnormalities Osler-Weber-Rendu ... tumor suppressor gene - acts in TGF- signal ... – PowerPoint PPT presentation

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Title: On Adenomas and Hyperplastic Polyposis


1


On Adenomas and Hyperplastic Polyposis
        
2
?? 52, ???? ??????, ????. ????? ??????????
?????????? ?? ????? ?????? ????? ??????? ???
?????? ?????? - ??? ??, ???? ?????????
?????????? ???? ?????? ????????????, ??????
????? ?Juvenile ????? ?????????, ???? ??????
????? ?????? ??????????? ??? ?? ?????? ???
3
???? PAF - ???? ???????, ??? ?????? ????
???? ??????? ??????? ????????, ???? ????????
?? HP ????? ????? ????? ??? - ??????? ?????
???? ?????? (?????? ???????...) ?????
?????????? ?????? ????? ???? ????? ???? ?????
HB11.5 ???? ???? (?????? 9), ??? ????? ??????
????
4
???????????? 5.06 ?????? - ????? ?? ???? ???
????? ??????? (????? ?-80), ???? lt 5 ??. ?????
3 ???????? ??????? ??????? ????? ?5-6 ??. -
????? ???????? ???? ??? ????????? ????? ?????
???? ??????? ????????? LGD, ????
???????????, ?????? Juvenile -- ????????? ??????
?????????, ??????? ???? ????? ?-15 ?? ????
???????????.
5
??? ?? - ?????????
6
???? ????????? - ?????????
7
?????? ?????
1. "?????, ?????... ?????"?2. ??? ??????
8
??????? ???????????
????? ?30-70 ???????? ?????????
??????????????, ????? ??????????. ???? ?????
?????? ?????? ????????? ?????? ??? ????????
?????. ??? ?? ??? ???? "?????" ???????? ??"?,
???? Serrated adenoma - ????? ?? ?????
????????? ???????, (?? ???? ??? ????? ??????
????????? ?? ????? ??? ?????? ?????(
Sweet K et al, JAMA 12.2005 294
(19)2465-73Ferrandez A et al, Am J Gastro
10.2004 99 (10) 2012-8
9
60 ????? ?? ????????? ??????????
????????? ?????????? gt 20 ??????? ????? ??
???? ???? ???? ????
????? ?????? ?"? ?WHO- ?-2000 (Jass and Burt)
????? ??? ?????? 1. gt 5 ??????? ???????????
?????????? ??????, ??? ????? 2 ????? gt 1
??2. ?? ???? ?? ??????? ??????????? ??????????
??????, ???? ???? ????? I ?? ????? ??
????????? ??????????3. gt 30 ??????? ???????????
?????? ????? ???? ??? (?? ?????? gt 20)
Ferrandez A et al, Am J Gastro 10.2004 99 (10)
2012-8
10
60 ????? ?? ????????? ??????????
??????? ????? ????, ????? ??????? ?-1980
?"?Williams ??? ????? ??? ???????? ?????
(????? ???? 3-13 ???? ?? ????? ???????)
????? ???? ????? ????? ?????, ??????? ????? - ???
????? ??????? (Serrated ?? ??????) ?????
?????? ???? ? 0-60 ?????? ?????
Sweet K et al, JAMA 12.2005 294
(19)2465-73Ferrandez A et al, Am J Gastro
10.2004 99 (10) 2012-8
11
60 ????? ?? ????????? ??????????
?? ?? ??????? ?????? ????????? ????? ???????
???? ??? ?????? -Hereditary Mixed Polyposis
syndrome ??? ???? ????? ?? ???????
???????????, Juvenile ????????, ???? ??
??????? ??????? ?? ?????? AD (15q13-q14)
??????????? ??????? ??? ??? ?? ???????
??????????? ???????? ???? ???????? ????? ??????
??? ???? ?? ????? ?????????? ???? ?????
Sweet K et al, JAMA 12.2005 294
(19)2465-73Ferrandez A et al, Am J Gastro
10.2004 99 (10) 2012-8
12
??????? ????? ?????????? ??????????
??' ?????
??????
????
????
7
Williams GT, Histopathol 1980
0 (???? Y 3-13)
23
81
Sweet K, JAMA 2005
36 (4 ?-11)
10

Rubio CA, Endoscopy 2006
70
13
Hyman NH, Dis Col Rec 2004
54
14
79
Lage P, Am J Gastro 2004
43
15
73
Ferrandez A, Am J Gastro 2004
7 (???? 0.3-13, AV3Y)
12
92
Leggett BA, Am J Surg Pathol 2001
58
Place RJ, J Am Coll Surg 1999
6

50
7

Rozen P, Am J Gastro 2003(1 pedigree, mixed
polyposis)
0 (CRC in family members)
13
??????? ????? ?????????? ??????????
  • ????? ??????? ????? ?????-??? (overestimation) ??
    ?????? ?????????
  • 1. ???? ??????, ?????? ????? ????? ????? ??????
    ??????????? ?????? ?????? ?? ??????? ??????
    ?? ????? ??? ?????? ???????.
  • 2. ??? ???? ????? ???? ????? ????? ?? ?????
    ???????? ?????????? ????? ???? ??????
    ????????????? (???? ??? ????? ??????) ????
    ?????? ?????.
  • .3 ????? ??????? ???? ??????? ????? ????? ???
    ?????.

14
?????? ????? ?????? ?????????? ??????????
???? ????? ?????????? ?????? 1-3 ???? ??"?
???? 20
??? ??????? ????? ?????? ?????? ????? ??????
???? ?? ??? ????? ??????? ?????. ?????
????????? ???? ????? ?? ?????.
???? ???? ??? ??? ?????? ??????? ?????
??????? ?????? ?? ?????? ????? ??? ?? ????
?????-?????? ????? ???? ???? ???? ??????.
Lage P et al, Am J Gastro 9.2004 99 (9) 1779-84
15
?????? (?????)
1. ?????! 2. ??? - ????? ??? ?? ????, ?????
????? ?? ???? ????
16
Familial Syndromes with Juvenile Polyps
????????? ?????? (Juvenile Polyposis)
  • Juvenile Polyposis (JPS)
  • Peutz-Jeghers (PJ)
  • Hereditary mixed polyposis
  • Basal cell nevous
  • Gorlin
  • Osler-Weber- Rendu
  • Bannayan- Riley- Ruvalcaba(BRR)
  • Cowden

17
??????? ??????? ??? ??????? ???? Juvenile
  • Bannayan- Riley- Ruvalcaba (BRRs) Juvenile
    polyposis, macrocephaly, cognitive motor
    dysfunction, subcutaneous and visceral
    lipomatosis, hemangiomas, genital
    pigmentation.
  • Cowden Juvenile polyposis, multiple other
    hamartomas, macrocephaly, cancers of breast,
    thyroid, uterus skin, papillomas,
    trichilemmomas.
  • Basal cell nevous Juvenile polyposis, multiple
    basal cell carcinomas
  • Gorlin Juvenile polyposis, multiple basal cell
    carcinomas, intracranial calcifications,
    skeletal, odontogenic cranio-facial
    abnormalities
  • Osler-Weber-Rendu

18
????????? ?????? (Juvenile Polyposis) ???????
Autosomal dominant
SMAD4/ DPC4 (18q21.1) (small mothers against
decapentaplegic /deleted in pancreatic
carcinoma) - tumor suppressor gene - acts in
TGF-? signal transduction
PTEN (10q22-23) (phosphatase tensin
homologue) - specific phosphatase, acts as
tumor suppressor
19
  • ????????? ??????- ????? ?????? (????? ???
    ???????????? ?????)
  • ????? 5-10 ??????? ???? ??????- Juvenile ???? ???
  • ????? ??????? ???? ?? ????? ?? ????? ??????
  • ?????? ?? ???? ?????? ?? ????????? ?????? ??
    ???? ?? ??????? ???? ??

( Vasen HFA, J Clin Oncol, 11/2000)
20
???????/??????? ?????????? ?????? (Juvenile
Polyposis)
???????
?? ????? ??????
????
??? ??
  • ????? ???? ????? ????? ????? ???????? ??????
    ?????? 55-27 (???? ?? 38-13 ????' ?????
    ?????? 21-14)

21
  • ????????? ?????? - ????? ??????
  • ???? ???? ??????, ????? ??????? (??????)
  • ????? ????? ??????????? ?? ??' ????? ??????
    ??????? ????? ??? ?2-3 ???? (???? ?????-25 ??
    ????? ???? ?? ?? ?????????)
  • ????????? ?????? - ?????
  • ??????????? ?? ????? ??????? ?????
  • ????? ??? ?? - ????????/??????? ?????????? ?????
    ?????? ?????? ?????????? ?? ?????????? ????
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