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Published in: Diseases of the Colon & Rectum 3/2004

01-03-2004 | Original Contribution

Significance of Incidental Desmoids Identified During Surgery for Familial Adenomatous Polyposis

Authors: J. E. Hartley, M.B.Ch.B., J. M. Church, M.B.Ch.B., S. Gupta, M.D., E. McGannon, B.S.W., V. W. Fazio, M.B., M.S.

Published in: Diseases of the Colon & Rectum | Issue 3/2004

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PURPOSE

The behavior of intra-abdominal desmoids in familial adenomatous polyposis is incompletely understood. Findings range from typical mass lesions to flat sheets, termed the desmoid reaction or desmoid precursor lesion. The latter often are incidental findings of uncertain significance. The study was designed to describe the natural history of incidental intra-abdominal desmoid tumors with particular reference to the desmoid reaction.

METHODS

Patients who underwent laparotomy for familial adenomatous polyposis at the Cleveland Clinic Foundation were identified. The incidence of incidental intra-abdominal desmoid tumors was determined by review of operative records. Intra-abdominal desmoid tumors were classified as mass lesions if three-dimensional or desmoid reaction if two-dimensional. The incidence of clinically apparent intra-abdominal desmoid tumors (typical mass lesions on physical examination or cross-sectional imaging in symptomatic patients) was determined by chart review. The incidence of clinical intra-abdominal desmoid tumors between groups was compared by Fisher’s exact test.

RESULTS

A total of 266 patients (153 females; median age, 26 (range, 9–63) years) underwent abdominal surgery for familial adenomatous polyposis. Incidental intra-abdominal desmoid tumors were identified in 34 patients: 8 at the index surgery and 26 at relaparotomy. These lesions influenced the planned procedure in eight cases (26 percent), including preventing ileoanal pouch in 3 of 19 patients in whom this was intended. The median follow-up from the time of identification of intra-abdominal desmoids was 42 (range, 2–178) months at which point four patients (11 percent) had developed clinical intra-abdominal desmoid tumors. There was no significant difference in incidence of clinical intra-abdominal desmoid tumors between mass and desmoid reaction groups (P = 0.27).

CONCLUSIONS

Incidental intra-abdominal desmoid tumors are a common finding at relaparotomy in patients with familial adenomatous polyposis. These lesions influence planned surgery in a minority of cases. Desmoid reaction may have little bearing on the subsequent development of clinically significant intra-abdominal desmoid tumors.
Literature
1.
go back to reference Anthony, T, Rodriguez-Bigas, MA, Weber, T, Petrelli, NJ 1996Desmoid tumorsJ Am Coll Surg18236977 Anthony, T, Rodriguez-Bigas, MA, Weber, T, Petrelli, NJ 1996Desmoid tumorsJ Am Coll Surg18236977
2.
go back to reference Reitamo, JJ, Scheinin, TM, Hayry, P 1986The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of desmoid tumorAm J Surg1512307 Reitamo, JJ, Scheinin, TM, Hayry, P 1986The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of desmoid tumorAm J Surg1512307
3.
go back to reference Jones, IT, Fazio, VW, Weakley, FL, Jagelman, DG, Lavery, IC, McGannon, E 1986Desmoid tumours in familial polyposis coliAnn Surg204947 Jones, IT, Fazio, VW, Weakley, FL, Jagelman, DG, Lavery, IC, McGannon, E 1986Desmoid tumours in familial polyposis coliAnn Surg204947
4.
go back to reference Lotfi, AM, Dozois, RR, Gordon, H, et al. 1989Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatmentInt J Colorectal Dis4306 Lotfi, AM, Dozois, RR, Gordon, H,  et al. 1989Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatmentInt J Colorectal Dis4306
5.
go back to reference Penna, C, Kartheuser, A, Parc, R, et al. 1993Secondary proctectomy and ileal pouch-anal anastomosis after ileorectal anastomosis for familial adenomatous polyposisBr J Surg8016213 Penna, C, Kartheuser, A, Parc, R,  et al. 1993Secondary proctectomy and ileal pouch-anal anastomosis after ileorectal anastomosis for familial adenomatous polyposisBr J Surg8016213
6.
go back to reference Gurbuz, AK, Giardello, FM, Peterson, GM, et al. 1994Desmoid tumours in familial adenomatous polyposisGut3537781 Gurbuz, AK, Giardello, FM, Peterson, GM,  et al. 1994Desmoid tumours in familial adenomatous polyposisGut3537781
7.
go back to reference Rodriguez-Bigas, MA, Mahoney, MC, Karakousis, CP, Petrelli, NJ 1994Desmoid tumors in patients with familial adenomatous polyposisCancer7412704 Rodriguez-Bigas, MA, Mahoney, MC, Karakousis, CP, Petrelli, NJ 1994Desmoid tumors in patients with familial adenomatous polyposisCancer7412704
8.
go back to reference Heiskanen, L, Jarvinen, HJ 1996Occurrence of desmoid tumours in familial polyposis and results of treatmentInt J Colorect Dis1115762 Heiskanen, L, Jarvinen, HJ 1996Occurrence of desmoid tumours in familial polyposis and results of treatmentInt J Colorect Dis1115762
9.
go back to reference Clark, SK, Neale, KF, Landgrebe, JC, Phillips, RK 1999Desmoid tumours complicating familial adenomatous polyposisBr J Surg8611859 Clark, SK, Neale, KF, Landgrebe, JC, Phillips, RK 1999Desmoid tumours complicating familial adenomatous polyposisBr J Surg8611859
10.
go back to reference Caspari, R, Olschwang, S, Friedl, W, et al. 1995Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444Hum Mol Genet433740 Caspari, R, Olschwang, S, Friedl, W,  et al. 1995Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444Hum Mol Genet433740
11.
go back to reference Eccles, DM, Lujit, R, Breukel, C, et al. 1996Hereditary desmoid disease due to a frameshift mutation at codon 1924 of the APC geneAm J Hum Genet591193201 Eccles, DM, Lujit, R, Breukel, C,  et al. 1996Hereditary desmoid disease due to a frameshift mutation at codon 1924 of the APC geneAm J Hum Genet591193201
12.
go back to reference Scott, RJ, Froggatt, NJ, Trembath, RC, Evans, DG, Hodgson, SV, Maher, ER 1996Familial infiltrative fibromatosis (desmoid tumours) (MIM 135290) caused by a recurrent 3′ APC gene mutationHum Mol Genet519214 Scott, RJ, Froggatt, NJ, Trembath, RC, Evans, DG, Hodgson, SV, Maher, ER 1996Familial infiltrative fibromatosis (desmoid tumours) (MIM 135290) caused by a recurrent 3′ APC gene mutationHum Mol Genet519214
13.
go back to reference Arvanitis, ML, Jagelman, DG, Fazio, VW, Lavery, IC, McGannon, E 1990Mortality in patients with familial polyposisDiseases Colon Rectum3363942 Arvanitis, ML, Jagelman, DG, Fazio, VW, Lavery, IC, McGannon, E 1990Mortality in patients with familial polyposisDiseases Colon Rectum3363942
14.
go back to reference Clark, SK, Phillips, RK 1996Desmoids in familial adenomatous polyposisBr J Surg831494504 Clark, SK, Phillips, RK 1996Desmoids in familial adenomatous polyposisBr J Surg831494504
15.
go back to reference Clark, SK, Johnson Smith, TG, Katz, DE, Reznek, RH, Phillips, RK 1998Identification and progression of a desmoid precursor lesion in patients with familial adenomatous polyposisBr J Surg859703 Clark, SK, Johnson Smith, TG, Katz, DE, Reznek, RH, Phillips, RK 1998Identification and progression of a desmoid precursor lesion in patients with familial adenomatous polyposisBr J Surg859703
16.
go back to reference Church, JM 1995Desmoid tumours in patients with familial adenomatous polyposisSemin Colon Rectal Surg62932 Church, JM 1995Desmoid tumours in patients with familial adenomatous polyposisSemin Colon Rectal Surg62932
17.
go back to reference Soravia, C, Berk, T, McLeod, RS, Cohen, Z 2000Desmoid disease in patients with familial adenomatous polyposisDis Colon Rectum433639 Soravia, C, Berk, T, McLeod, RS, Cohen, Z 2000Desmoid disease in patients with familial adenomatous polyposisDis Colon Rectum433639
18.
go back to reference Brooks, AP, Reznek, RH, Nugent, K, Farmer, KC, Thomson, JP, Phillips, RK 1994CT appearances of desmoid tumours in familial polyposis: further observationsClin Radiol496017 Brooks, AP, Reznek, RH, Nugent, K, Farmer, KC, Thomson, JP, Phillips, RK 1994CT appearances of desmoid tumours in familial polyposis: further observationsClin Radiol496017
1.
go back to reference Middleton, SB, Frayling, IF, Phillips, RK 2000Desmoids in familial adenomatous polyposis are monoclonal proliferationsBr J Cancer8282732 Middleton, SB, Frayling, IF, Phillips, RK 2000Desmoids in familial adenomatous polyposis are monoclonal proliferationsBr J Cancer8282732
2.
go back to reference Scates, DK, Clark, SK, Phillips, RK, Venitt, S 1998Lack of telomerase in desmoids occurring sporadically and in association with familial adenomatous polyposisBr J Surg859659 Scates, DK, Clark, SK, Phillips, RK, Venitt, S 1998Lack of telomerase in desmoids occurring sporadically and in association with familial adenomatous polyposisBr J Surg859659
3.
go back to reference Middleton, SB, Pack, K, Phillips, RK 2000Telomere length in familial adenomatous polyposis-associated desmoidsDis Colon Rectum43143540 Middleton, SB, Pack, K, Phillips, RK 2000Telomere length in familial adenomatous polyposis-associated desmoidsDis Colon Rectum43143540
4.
go back to reference Lamlum, H, Ilyas, M, Rowan, A, et al. 1999The type of somatic mutation at APC in FAP is determined by the site of the germline mutation: a new facet to Knudson’s ‘two-hit’ hypothesisNat Med510715 Lamlum, H, Ilyas, M, Rowan, A,  et al. 1999The type of somatic mutation at APC in FAP is determined by the site of the germline mutation: a new facet to Knudson’s ‘two-hit’ hypothesisNat Med510715
5.
go back to reference Clark SK. Studies in desmoid disease in familial adenomatous polyposis. Awarded MD. University of Cambridge 1998. Clark SK. Studies in desmoid disease in familial adenomatous polyposis. Awarded MD. University of Cambridge 1998.
6.
go back to reference Middleton, SB, Clark, SK, Matravers, P, Katz, D, Reznek, R, Phillips, RK 2003Stepwise progression of familial adenomatous polyposis-associated desmoid precursor lesions demonstrated by a novel CT scoring systemDis Colon Rectum464815 Middleton, SB, Clark, SK, Matravers, P, Katz, D, Reznek, R, Phillips, RK 2003Stepwise progression of familial adenomatous polyposis-associated desmoid precursor lesions demonstrated by a novel CT scoring systemDis Colon Rectum464815
Metadata
Title
Significance of Incidental Desmoids Identified During Surgery for Familial Adenomatous Polyposis
Authors
J. E. Hartley, M.B.Ch.B.
J. M. Church, M.B.Ch.B.
S. Gupta, M.D.
E. McGannon, B.S.W.
V. W. Fazio, M.B., M.S.
Publication date
01-03-2004
Publisher
Springer-Verlag
Published in
Diseases of the Colon & Rectum / Issue 3/2004
Print ISSN: 0012-3706
Electronic ISSN: 1530-0358
DOI
https://doi.org/10.1007/s10350-003-0063-0

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