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Professor Neil A Shepherd

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1 Professor Neil A Shepherd
Special polyp types Professor Neil A Shepherd Gloucester, UK NHSBCSP Pathology Day, London, November 21, 2007

2 Pathology and the NHSBCSP
a polyp is any lesion raised above the level of an epithelial surface suddenly, with BCSP, there are lots more colonoscopies all done by experts with dye-spray of small lesions and EMR we might expect to see the occasional funny and we need to be alert

3 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – the triumvirate of polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

4 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc but some ‘polyps’ likely to be excised in BCSP are not in the list…..

5 68M. Polypoid nodule in rectum.
chromogranin synaptophysin

6 Gloucestershire BCSP, January-October 2007
normal 30 19.87% cancer 24 15.89% low risk polyps 20 13.25% intermediate risk polyps 38 25.17% high risk polyps 22 14.57% abnormal result, not polyps 6 3.97% refer for surgery (polyp) 5 3.31% miscellaneous TOTAL 151 100%

7 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

8 Hyperplastic polyps about 25% of all polyps Issues:
differentiating them from mixed polyps & serrated adenoma when they are large and right-sided and show SSP features when they show epithelial misplacement – inverted hyperplastic polyps

9 Inverted hyperplastic polyps
closely related to right sided SSPs Shepherd, 1991

10 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

11 Inflammatory polyps the most common ‘other’ entity in BCSP
most are single/scanty and not related to CIBD (UC, CD, chronic infection such as schisto) in the age group of the BSCP population, diverticulosis-associated polyps, polypoid mucosal prolapse, post-interventional polyps more common

12 67F. BCSP. Diverticulosis and sigmoid colonic polyp.

13 Polypoid mucosal prolapse
in the BCSP age group, lower rectal/anal most common: inflammatory cloacogenic polyp epithelial (villous) hyperplasia traps the unwary into calling them large villous adenomas may be seen in association with diverticulosis, at stomas, in SUMPS, etc

14 Polypoid mucosal prolapse at the anorectal junction
a word of warning with inflammatory cloacogenic polyp 4 cases of adenomas of the lower rectum causing secondary polypoid mucosal prolapse Parfitt & Shepherd, 2008

15 a b Ki-67 c d p53 Figure 1

16 a b Ki-67 c d p53 Figure 2

17 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

18 Juvenile polyps commonest polyp in childhood
sporadic juvenile polyps do occur in adulthood but very rare in BCSP age group inflammatory polyps can look similar pretty unlikely to see juvenile polyposis….

19 Juvenile polyposis

20 ‘Atypical juvenile polyps’ with dysplasia

21 Juvenile polyposis 1 in 80,000 genetics becoming clearer but polymorphic: SMAD4 implicated in 25% high rates of colorectal cancer: life time risk - ? up to 30% dysplasia in atypical juvenile polyps ? surveillance ? prophylactic colectomy Jass et al 1988, Woodford-Richens et al, 2000

22 Quick quiz question SMAD 4 mutation is implicated in about a quarter of cases of this condition. What does SMAD stand for? Is it: somatic mutation in Arctic drosophila serine-methionine-arginine deletion small mothers against decapentaplegia

23 Quick quiz question SMAD 4 mutation is implicated in about a quarter of cases of this condition. What does SMAD stand for? Is it: somatic mutation in Arctic drosophila serine-methionine-arginine deletion small mothers against decapentaplegia

24 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

25 Peutz-Jeghers polyposis

26 Peutz-Jeghers polyps syndrome about 1 in 100,000
sporadic PJ-like polyps described but very rare ? relationship to ‘inflammatory myoglandular polyp’ aren’t sporadic PJPs most likely to be post-inflammatory or post-mucosal prolapse?

27 Do sporadic Peutz-Jeghers polyps exist?
119 polyps in 38 patients most patients with PJ-like polyps were actually shown to have PJS (scanty colonic polyps in the syndrome) 8 left: three small bowel ones with PJ features; 5 colonic with suggestive features of PJPs (top GI pathologists could not tell for certain: mucosal prolapse could not be excluded). 40% in BCSP screening age group if they exist, sporadic colorectal PJ polyps are very rare Burkhart et al. Do sporadic Peutz-Jeghers polyps exist? Experience from a large teaching hospital. AJSP, August 2007

28 A classification of polyps of the colorectum
adenomas hyperplastic polyps mixed polyps inflammatory polyps juvenile polyps Peutz-Jeghers polyps stromal polyps syndromic polyps – polyposes, Cowden’s, Cronkhite-Canada, etc Morson & Dawson, 2003

29 Stromal polyps GIST leiomyoma of muscularis mucosae
gastrointestinal schwannoma neurofibroma ganglioneuroma epithelioid mucosal nerve sheath tumour intestinal perineurioma fibroblastic polyp inflammatory fibroid polyp that’s enough stromal polyps (Ed)

30 Small polypoid rectal nodules

31 Gastrointestinal schwannoma
33 cases: 4 oesophagus, 24 stomach, 2 colon and 3 rectum mainly within muscularis propria and/or subserosa none encapsulated but all well circumscribed 32/33 cuff of lymphocytes only 12 originally called schwannoma S100 and vimentin +ve CD117, CD34, ASMA, desmin –ve nestin 80% positive; GFAP 64% positive all showed some nuclear pleomorphism: ? longevity: no mitoses all benign Hou et al, 2006 (Histopathology)

32

33 Colonic polypoid mucosal lesions with mucosal entrapment and epithelial serration
A feature only rarely seen in GISTs

34 Colorectal intramucosal tumours with epithelial ‘entrapment’
Name First recognised S100 CD 34 EMA Epithelial serration Clinical features neuro-fibroma The year dot + ve -ve - ve occasionally Most solitary NF ganglio-neuroma probably down to Carney… +ve not described sporadic or NF-1, MEN 2b benign fibroblastic polyp of colon 2004 3/13 none intestinal peri-neurioma 2005 5/9 epithelioid NS tumour +/-ve 0/6

35 CD34 positive: ‘neural’ markers negative
Benign fibroblastic polyp of colon: Eslami-Varzaneh F, Washington K, Robert ME, Kashgarian M, Goldblum JR, Jain D. AJSP 2004; 28: CD34 positive: ‘neural’ markers negative

36 EMA positive: CD 117, CD 34, S100 negative
Intestinal perineurioma: Hornick JL, Fletcher CDM. AJSP 2005; 29: EMA positive: CD 117, CD 34, S100 negative

37 S100 & CD 34 positive: EMA & CD 117 negative
Mucosal epithelioid nerve sheath tumour: Lewin MR, Dilworth HP, Alfa AK, Epstein JI, Montgomery E. AJSP 2005; 29: S 100 CD 34 S100 & CD 34 positive: EMA & CD 117 negative

38 Colorectal intramucosal tumours with epithelial ‘entrapment’ and surface serration
extraordinary ‘new’ descriptions of three entities – smallish spindle cell polypoid tumours with epithelial entrapment and surface epithelial serration ? recent recognition due to legislation to allow Medicare customers to undergo screening colonoscopy (2001) lesions are mainly of schwann cell/perineural cell origin differentiation by immunohistochemistry although specificity of these entities is a little doubtful.. Zamecnik & Chlumska, 2006 Groisman et al, 2006 all are benign and should not be confused with more sinister lesions such as GISTs and others

39 Take home messages: special polyp types
inflammatory polyp/polypoid mucosal prolapse most common after adenomas and HPs remember other lesions can be polypoid – leiomyoma of mm and rectal carcinoid – clinical, endoscopic and imaging correlation important the ‘hamartomatous’ polyps and syndromes are very rare (but not unheard of) in the BCSP age group serrated change can be a secondary phenomenon (polypoid mucosal prolapse, fibroblastic polyps, etc)


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