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Gastrointestinal Polyposis in Childhood: Clinicopathologic and Genetic Features

  • Perspectives in Pediatric Pathology
  • Published:
Pediatric and Developmental Pathology

Abstract

Gastrointestinal polyps and certain extraintestinal lesions in children may herald a hereditary polyposis syndrome, with an increased risk of neoplasia and other health problems for both children and their relatives. The availability of molecular/genetic screening tests has increased early diagnosis of younger members of known polyposis families. This article reviews the gross and microscopic features of polyposis syndromes of childhood and summarizes the molecular/genetic advances in this field. Clinical management is also briefly discussed.

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REFERENCES

  1. Online Mendelian Inheritance in Man, OMIM (TM). Baltimore, MD: McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University, and Bethesda, MD: National Center for Biotechnology Information, National Library of Medicine, 2000; World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  2. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {174900} {1/12/2002}: World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  3. RW Burt RF Jacobi (2003) Polyposis syndromes T Yamada (Eds) Textbook of Gastroenterology 4th edition, Lippincott-Williams and Wilkins Philadelphia 1914–1939

    Google Scholar 

  4. EJ Hoffenberg A Sauaia T Maltzman K Knoll DJ Ahnen (1999) ArticleTitleSymptomatic colonic polyps in childhood: not so benign J. Pediatr. Gastroenterol. Nutr. 28 175–181 Occurrence Handle10.1097/00005176-199902000-00016 Occurrence Handle1:STN:280:DyaK1M7ivFKgsw%3D%3D Occurrence Handle9932851

    Article  CAS  PubMed  Google Scholar 

  5. W Hyer I Beveridge P Domizio R Phillips (2000) ArticleTitleClinical management and genetics of gastrointestinal polyps in children J. Pediatr. Gastroenterol. Nutr. 31 469–479 Occurrence Handle1:STN:280:DC%2BD3MrlvVGnug%3D%3D Occurrence Handle11144430

    CAS  PubMed  Google Scholar 

  6. J Corredor J Wambach J Barnard (2001) ArticleTitleGastrointestinal polyps in children: advances in molecular genetics, diagnosis, and management J. Pediatr. 138 621–628 Occurrence Handle10.1067/mpd.2001.113619 Occurrence Handle1:CAS:528:DC%2BD3MXkt1Oiurw%3D Occurrence Handle11343034

    Article  CAS  PubMed  Google Scholar 

  7. KA Waite C Eng (2002) ArticleTitleProtean PTEN: form and function Am. J. Hum. Genet. 70 829–844 Occurrence Handle10.1086/340026 Occurrence Handle1:CAS:528:DC%2BD38XivFClur8%3D Occurrence Handle11875759

    Article  CAS  PubMed  Google Scholar 

  8. GM Scharf JH Becker NJ Laage (1986) ArticleTitleJuvenile gastrointestinal polyposis or the infantile Cronkhite-Canada syndrome J. Pediatr. Surg. 21 953–954 Occurrence Handle1:STN:280:BiiD1cnhtVI%3D Occurrence Handle3794950

    CAS  PubMed  Google Scholar 

  9. S Nicholls V Smith R Davies C Doig A Thomas V Miller (1995) ArticleTitleDiffuse juvenile non-adenomatous polyposis: a rare cause of severe hypoalbuminaemia in childhood Acta. Paediatr. 84 1447–1448 Occurrence Handle1:STN:280:BymB3M%2FktFM%3D Occurrence Handle8645971

    CAS  PubMed  Google Scholar 

  10. A Watanabe H Nagashima M Motoi K Ogawa (1979) ArticleTitleFamilial juvenile polyposis of the stomach Gastroenterology 77 148–151 Occurrence Handle1:STN:280:CSaC1M3ktF0%3D Occurrence Handle447014

    CAS  PubMed  Google Scholar 

  11. DC Desai V Murday RK Phillips KF Neale P Milla SV Hodgson (1998) ArticleTitleA survey of phenotypic features in juvenile polyposis J. Med. Genet. 35 476–481 Occurrence Handle1:STN:280:DyaK1czgsFCnsA%3D%3D Occurrence Handle9643289

    CAS  PubMed  Google Scholar 

  12. JR Howe JC Ringold JH Hughes RW Summers (1999) ArticleTitleDirect genetic testing for SMAD-4 mutations in patients at risk for juvenile polyposis Surgery 126 162–170 Occurrence Handle10.1067/msy.1999.98750 Occurrence Handle1:STN:280:DyaK1MzosVyhsw%3D%3D Occurrence Handle10455879

    Article  CAS  PubMed  Google Scholar 

  13. W Friedl R Kruse S Uhlhaas et al. (1999) ArticleTitleFrequent 4-bp deletion in exon 9 of the SMAD4/MADH4 gene in familial juvenile polyposis patients Genes Chromosomes Cancer 25 403–406 Occurrence Handle10.1002/(SICI)1098-2264(199908)25:4<403::AID-GCC15>3.3.CO;2-G Occurrence Handle1:CAS:528:DyaK1MXkvVOqtLs%3D Occurrence Handle10398437

    Article  CAS  PubMed  Google Scholar 

  14. XP Zhou K Woodford-Richens R Lehtonen et al. (2001) ArticleTitleGermline mutations in BMPR1A/ALK3 cause a subset of cases of juvenile polyposis syndrome and of Cowden and Bannayan-Riley-Ruvalcaba syndromes Am. J. Hum. Genet. 69 704–711 Occurrence Handle10.1086/323703 Occurrence Handle11536076

    Article  PubMed  Google Scholar 

  15. S Roth P Sistonen R Salovaara et al. (1999) ArticleTitleSMAD genes in juvenile polyposis Genes Chromosomes Cancer 26 54–61 Occurrence Handle10.1002/(SICI)1098-2264(199909)26:1<54::AID-GCC8>3.0.CO;2-D Occurrence Handle1:CAS:528:DyaK1MXlsFymtb4%3D Occurrence Handle10441006

    Article  CAS  PubMed  Google Scholar 

  16. JR Howe JL Bair MG Sayed et al. (2001) ArticleTitleGermline mutations of the gene encoding bone morphogenetic protein receptor 1A in juvenile polyposis Nat. Genet. 28 184–187 Occurrence Handle10.1038/88919 Occurrence Handle1:CAS:528:DC%2BD3MXktFOrtb0%3D Occurrence Handle11381269

    Article  CAS  PubMed  Google Scholar 

  17. RF Jacoby S Schlack CE Cole M Skarbek C Harris LF Meisner (1997) ArticleTitleA juvenile polyposis tumor suppressor locus at 10q22 is deleted from nonepithelial cells in the lamina propria Gastroenterology 112 1398–1403 Occurrence Handle1:CAS:528:DyaK2sXislyhtbY%3D Occurrence Handle9098028

    CAS  PubMed  Google Scholar 

  18. K Woodford-Richens J Williamson S Bevan et al. (2000) ArticleTitleAllelic loss at SMAD4 in polyps from juvenile polyposis patients and use of fluorescence in situ hybridization to demonstrate clonal origin of the epithelium Cancer Res. 60 2477–2482 Occurrence Handle1:CAS:528:DC%2BD3cXjtV2qtL4%3D Occurrence Handle10811127

    CAS  PubMed  Google Scholar 

  19. DJ Marsh PL Dahia Z Zheng et al. (1997) ArticleTitleGermline mutations in PTEN are present in Bannayan-Zonana syndrome Nat. Genet. 16 333–334 Occurrence Handle1:STN:280:ByiA1critlc%3D Occurrence Handle9241266

    CAS  PubMed  Google Scholar 

  20. BA Leggett LR Thomas N Knight S Healey G Chenevix-Trench J Searle (1993) ArticleTitleExclusion of APC and MCC as the gene defect in one family with familial juvenile polyposis Gastroenterology 105 1313–1316 Occurrence Handle1:STN:280:ByuD2c%2FkvVc%3D Occurrence Handle8224634

    CAS  PubMed  Google Scholar 

  21. TT Wu B Rezai A Rashid et al. (1997) ArticleTitleGenetic alterations and epithelial dysplasia in juvenile polyposis syndrome and sporadic juvenile polyps Am. J. Pathol. 150 939–947 Occurrence Handle1:STN:280:ByiB3MvksFY%3D Occurrence Handle9060832

    CAS  PubMed  Google Scholar 

  22. KL Cox RC Jr Frates A Wong G Gandhi (1980) ArticleTitleHereditary generalized juvenile polyposis associated with pulmonary arteriovenous malformation Gastroenterology 78 1566–1570 Occurrence Handle1:STN:280:Bi%2BC1c%2FitVA%3D Occurrence Handle7372073

    CAS  PubMed  Google Scholar 

  23. Conte WJ, Rotter JL, Schwartz AG, Congleton JE (1982) “Hereditary generalized juvenile polyposis, arteriovenous malformations and colonic carcinoma (abstract)” Clin. Res. 30: 93A

  24. AL Baert Daele M Casteels-Van J Broeckx L Wijndaele G Wilms E Eggermont (1983) ArticleTitleGeneralized juvenile polyposis with pulmonary arteriovenous malformations and hypertrophic osteoarthropathy AJR. Am. J. Roentgenol. 141 661–662 Occurrence Handle1:STN:280:BiyA3crjs1c%3D Occurrence Handle6604412

    CAS  PubMed  Google Scholar 

  25. EL Simpson MK Dalinka (1985) ArticleTitleAssociation of hypertrophic osteoarthropathy with gastrointestinal polyposis AJR. Am. J. Roentgenol. 144 983–984 Occurrence Handle1:STN:280:BiqC2s7mtFA%3D Occurrence Handle3872584

    CAS  PubMed  Google Scholar 

  26. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {175050} {6/20/1997}: World Wide Web URL: http//www.ncbi.nlm.nih.gov/omim/

  27. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {1534800} {4/19/2001}: World Wide Web URL: http//www.ncbi.nlm.nih.gov/omim/

  28. A Lowichik FV White CF Timmons et al. (2000) ArticleTitleBannayan-Riley-Ruvalcaba syndrome: spectrum of intestinal pathology including juvenile polyps Pediatr. Dev. Pathol. 3 155–161 Occurrence Handle10.1007/s100240050019 Occurrence Handle1:STN:280:DC%2BD3c7ksVajuw%3D%3D Occurrence Handle10679034

    Article  CAS  PubMed  Google Scholar 

  29. DJ Marsh JB Kum KI Lunetta et al. (1999) ArticleTitlePTEN mutation spectrum and genotype-phenotype correlations in Bannayan-Riley-Ruvalcaba syndrome suggest a single entity with Cowden syndrome Hum. Mol. Genet. 8 1461–1472 Occurrence Handle10.1093/hmg/8.8.1461 Occurrence Handle1:CAS:528:DyaK1MXltFagtLg%3D Occurrence Handle10400993

    Article  CAS  PubMed  Google Scholar 

  30. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {158350} {4/26/2002}: World Wide Web URL: http//www.ncbi.nlm.nih.gov/omim/

  31. AM Hanssen JP Fryns (1995) ArticleTitleCowden syndrome J. Med. Genet. 32 117–119 Occurrence Handle1:STN:280:ByqB1c%2FivFI%3D Occurrence Handle7760320

    CAS  PubMed  Google Scholar 

  32. AM Hanssen H Werquin E Suys JP Fryns (1993) ArticleTitleCowden syndrome: report of a large family with macrocephaly and increased severity of signs in subsequent generations Clin. Genet. 44 281–286 Occurrence Handle1:STN:280:ByuC2sjnsFU%3D Occurrence Handle8131297

    CAS  PubMed  Google Scholar 

  33. JM Carethers FB Furnari AF Zigman et al. (1998) ArticleTitleAbsence of PTEN/MMAC1 germ-line mutations in sporadic Bannayan-Riley-Ruvalcaba syndrome Cancer Res. 58 2724–2726 Occurrence Handle1:CAS:528:DyaK1cXksFCqtr4%3D Occurrence Handle9661881

    CAS  PubMed  Google Scholar 

  34. EM Arch BK Goodman RA Van Wesep et al. (1997) ArticleTitleDeletion of PTEN in a patient with Bannayan-Riley-Ruvalcaba syndrome suggests allelism with Cowden disease Am. J. Med.Genet. 71 489–493 Occurrence Handle10.1002/(SICI)1096-8628(19970905)71:4<489::AID-AJMG24>3.3.CO;2-I Occurrence Handle1:STN:280:ByiH3s3lslM%3D Occurrence Handle9286463

    Article  CAS  PubMed  Google Scholar 

  35. DJ Marsh V Coulon KL Lunetta et al. (1998) ArticleTitleMutation spectrum and genotype-phenotype analyses in Cowden disease and Bannayan-Zonana syndrome, two hamartoma syndromes with germline PTEN mutation Hum. Mol. Genet. 7 507–515 Occurrence Handle10.1093/hmg/7.3.507 Occurrence Handle1:CAS:528:DyaK1cXitFWku74%3D Occurrence Handle9467011

    Article  CAS  PubMed  Google Scholar 

  36. FM Giardiello SR Hamilton (1997) ArticleTitleHereditary mixed polyposis syndrome: a zebra or a horse dressed in pinstripes Gastroenterology 112 643–645 Occurrence Handle1:STN:280:ByiC2snnslM%3D Occurrence Handle9024316

    CAS  PubMed  Google Scholar 

  37. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {601228} {6/11/1997}: World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  38. SC Whitelaw VA Murday IP Tomlinson et al. (1997) ArticleTitleClinical and molecular features of the hereditary mixed polyposis syndrome Gastroenterology 112 327–334 Occurrence Handle1:STN:280:ByiC2snnsVM%3D Occurrence Handle9024286

    CAS  PubMed  Google Scholar 

  39. CR Sachatello WO Jr Griffen (1975) ArticleTitleHereditary polypoid diseases of the gastrointestinal tract: a working classification Am. J. Surg. 129 198–203 Occurrence Handle1:STN:280:CSqC38nit1w%3D Occurrence Handle1091175

    CAS  PubMed  Google Scholar 

  40. CM Coffin LP Dehner (1996) ArticleTitleWhat is a juvenile polyp? An analysis based on 21 patients with solitary and multiple polyps Arch. Pathol. Lab. Med. 120 1032–1038 Occurrence Handle1:STN:280:DC%2BD38zgslKgtQ%3D%3D Occurrence Handle12049105

    CAS  PubMed  Google Scholar 

  41. CM Coffin AL Pappin (1997) ArticleTitlePolyps and neoplasms of the gastrointestinal tract in childhood and adolescence Perspect. Pediatr. Pathol. 20 127–171

    Google Scholar 

  42. HJ Bussey AM Veale BC Morson (1978) ArticleTitleGenetics of gastrointestinal polyposis Gastroenterology 74 1325–1330 Occurrence Handle1:STN:280:CSeC28botlM%3D Occurrence Handle348556

    CAS  PubMed  Google Scholar 

  43. RC Haggitt BJ Reid (1986) ArticleTitleHereditary gastrointestinal polyposis syndromes Am. J. Surg. Pathol. 10 871–887 Occurrence Handle1:STN:280:BiiD2szptFY%3D Occurrence Handle3024515

    CAS  PubMed  Google Scholar 

  44. ZD Goodman JH Yardley FD Milligan (1979) ArticleTitlePathogenesis of colonic polyps in multiple juvenile polyposis: report of a case associated with gastric polyps and carcinoma of the rectum Cancer 43 1906–1913 Occurrence Handle1:STN:280:CSaC1cbjtlM%3D Occurrence Handle445378

    CAS  PubMed  Google Scholar 

  45. JR Jass CB Williams HJ Bussey BC Morson (1988) ArticleTitleJuvenile polyposis—a precancerous condition Histopathology 13 619–630 Occurrence Handle1:STN:280:BiaC2s3osFQ%3D Occurrence Handle2853131

    CAS  PubMed  Google Scholar 

  46. HW Grotsky RR Rickert WD Smith JF Newsome (1982) ArticleTitleFamilial juvenile polyposis coli. A clinical and pathologic study of a large kindred Gastroenterology 82 494–501 Occurrence Handle1:STN:280:Bi2D1MnhtlY%3D Occurrence Handle7054044

    CAS  PubMed  Google Scholar 

  47. C Subramony CE Scott-Conner D Skelton TJ Hall (1994) ArticleTitleFamilial juvenile polyposis. Study of a kindred: evolution of polyps and relationship to gastrointestinal carcinoma Am. J. Clin. Pathol. 102 91–97 Occurrence Handle1:STN:280:ByuA3M7islY%3D Occurrence Handle8037173

    CAS  PubMed  Google Scholar 

  48. SE Mills RE Fechner (1982) ArticleTitleUnusual adenomatous polyps in juvenile polyposis coli Am. J. Surg. Pathol. 6 177–183 Occurrence Handle1:STN:280:Bi2B2Mvis1I%3D Occurrence Handle7102894

    CAS  PubMed  Google Scholar 

  49. H Jarvinen KO Franssila (1984) ArticleTitleFamilial juvenile polyposis coli; increased risk of colorectal cancer Gut 25 792–800 Occurrence Handle1:STN:280:BiuB3sjmvFM%3D Occurrence Handle6735258

    CAS  PubMed  Google Scholar 

  50. K Vaiphei BR Thapa (1997) ArticleTitleJuvenile polyposis (coli)—high incidence of dysplastic epithelium J. Pediatr. Surg. 32 1287–1290 Occurrence Handle1:STN:280:ByiH2MjptVQ%3D Occurrence Handle9314244

    CAS  PubMed  Google Scholar 

  51. U Poddar BR Thapa K Vaiphei KL Rao SK Mitra K Singh (1998) ArticleTitleJuvenile polyposis in a tropical country Arch. Dis. Child. 78 264–266 Occurrence Handle1:STN:280:DyaK1c3ns1ejug%3D%3D Occurrence Handle9613360

    CAS  PubMed  Google Scholar 

  52. G Mendelsohn MP Diamond (1984) ArticleTitleFamilial ganglioneuromatous polyposis of the large bowel. Report of a family with associated juvenile polyposis Am. J. Surg. Pathol. 8 515–520 Occurrence Handle1:STN:280:BiuB2MvjsVM%3D Occurrence Handle6742313

    CAS  PubMed  Google Scholar 

  53. WH Donnelly WK Sieber EJ Yunis (1969) ArticleTitlePolypoid ganglioneurofibromatosis of the large bowel Arch. Pathol. 87 537–541 Occurrence Handle1:STN:280:CCaC2Mzgt1w%3D Occurrence Handle4975812

    CAS  PubMed  Google Scholar 

  54. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {175100} {5/21/2002}: World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  55. J Groden A Thliveris W Samowitz et al. (1991) ArticleTitleIdentification and characterization of the familial adenomatous polyposis coli gene Cell 66 589–600 Occurrence Handle1651174

    PubMed  Google Scholar 

  56. M Enomoto M Konishi T Iwama J Utsunomiya KI Sugihara M Miyaki (2000) ArticleTitleThe relationship between frequencies of extracolonic manifestations and the position of APC germline mutation in patients with familial adenomatous polyposis Jpn. J. Clin. Oncol. 30 82–88 Occurrence Handle10.1093/jjco/hyd017 Occurrence Handle1:STN:280:DC%2BD3c3ivFemug%3D%3D Occurrence Handle10768871

    Article  CAS  PubMed  Google Scholar 

  57. KH Goss J Groden (2000) ArticleTitleBiology of the adenomatous polyposis coli tumor suppressor J. Clin. Oncol. 18 1967–1979 Occurrence Handle1:STN:280:DC%2BD3c3ksleltg%3D%3D Occurrence Handle10784639

    CAS  PubMed  Google Scholar 

  58. G Li K Tamura Y Yamamoto et al. (1999) ArticleTitleMolecular and clinical study of familial adenomatous polyposis for genetic testing and management J. Exp. Clin. Cancer Res. 18 519–529 Occurrence Handle1:CAS:528:DC%2BD3cXhsF2lu78%3D Occurrence Handle10746979

    CAS  PubMed  Google Scholar 

  59. Winter, H (1996) “Intestinal polyps” In: Walker, W, Durie, P, Hamilton, J, Walker-Smith, J, Watkins, J (editors), Pediatric Gastrointestinal Disease, Mosby, St. Louis, pp 891–907

  60. Talbot, IC, Burt, R, Jarvinen, H, Thomas, G (2000) “Familial adenomatous polyposis” In: Hamilton, SR, Aaltonen, LA (editors), Pathology and Genetics of Tumours of the Digestive System, International Agency for Research on Cancer, Lyon, France, pp 120

  61. JW Nash T Niemann WL Marsh WL Frankel (2002) ArticleTitleTo step or not to step: an approach to clinically diagnosed polyps with no initial pathologic finding Am. J. Clin. Pathol. 117 419–423 Occurrence Handle11888080

    PubMed  Google Scholar 

  62. ML Wu SM Dry CR Lassman (2002) ArticleTitleDeeper examination of negative colorectal biopsies Am. J. Clin. Pathol. 117 424–428 Occurrence Handle11888081

    PubMed  Google Scholar 

  63. AK Rustgi (1994) ArticleTitleHereditary gastrointestinal polyposis and nonpolyposis syndromes N. Engl. J. Med. 331 1694–1702 Occurrence Handle10.1056/NEJM199412223312507 Occurrence Handle1:STN:280:ByqD2MbjtlQ%3D Occurrence Handle7969362

    Article  CAS  PubMed  Google Scholar 

  64. CA Griffin S Lazar SR Hamilton et al. (1993) ArticleTitleCytogenetic analysis of intestinal polyps in polyposis syndromes: comparison with sporadic colorectal adenomas Cancer Genet. Cytogenet. 67 14–20 Occurrence Handle10.1016/0165-4608(93)90038-N Occurrence Handle1:STN:280:ByyB2sfmsVI%3D Occurrence Handle8389239

    Article  CAS  PubMed  Google Scholar 

  65. S Distante S Nasioulas GR Somers et al. (1996) ArticleTitleFamilial adenomatous polyposis in a 5 year old child: a clinical, pathological, and molecular genetic study J. Med. Genet. 33 157–160 Occurrence Handle1:STN:280:ByiD1czjvVU%3D Occurrence Handle8929955

    CAS  PubMed  Google Scholar 

  66. P Domizio IC Talbot AD Spigelman CB Williams RK Phillips (1990) ArticleTitleUpper gastrointestinal pathology in familial adenomatous polyposis: results from a prospective study of 102 patients J. Clin. Pathol. 43 738–743 Occurrence Handle1:STN:280:By6D3cbptFE%3D Occurrence Handle2170464

    CAS  PubMed  Google Scholar 

  67. TA Goedde MA Rodriguez-Bigas L Herrera NJ Petrelli (1992) ArticleTitleGastroduodenal polyps in familial adenomatous polyposis Surg. Oncol. 1 357–361 Occurrence Handle1:STN:280:ByuD2szks1w%3D Occurrence Handle1341271

    CAS  PubMed  Google Scholar 

  68. F Seow-Choen JM Ho J Wong HS Goh (1992) ArticleTitleGross and histological abnormalities of the foregut in familial adenomatous polyposis: a study from a South East Asian Registry Int. J. Colorectal Dis. 7 177–183 Occurrence Handle1:STN:280:ByyC1cjks1I%3D Occurrence Handle1338090

    CAS  PubMed  Google Scholar 

  69. H Watanabe M Enjoji T Yao K Ohsato (1978) ArticleTitleGastric lesions in familial adenomatosis coli: their incidence and histologic analysis Hum. Pathol. 9 269–283 Occurrence Handle1:STN:280:CSeC1M3ot1Q%3D Occurrence Handle26633

    CAS  PubMed  Google Scholar 

  70. S Bulow (1987) ArticleTitleFamilial polyposis coli Dan. Med. Bull. 34 1–15 Occurrence Handle1:STN:280:BiiC28%2FpslU%3D

    CAS  Google Scholar 

  71. DG Jagelman (1987) ArticleTitleExtracolonic manifestations of familial polyposis coli Semin. Surg. Oncol. 3 88–91 Occurrence Handle1:STN:280:BiiB3szlvVY%3D Occurrence Handle3035694

    CAS  PubMed  Google Scholar 

  72. R Caspari S Olschwang W Friedl et al. (1995) ArticleTitleFamilial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444 Hum. Mol. Genet. 4 337–340 Occurrence Handle1:CAS:528:DyaK2MXkvVeju7w%3D Occurrence Handle7795585

    CAS  PubMed  Google Scholar 

  73. EJ Gardner (1951) ArticleTitleA genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum Am. J. Hum. Genet. 3 167–176

    Google Scholar 

  74. EJ Gardner RC Richards (1953) ArticleTitleMultiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis Am. J. Hum. Genet. 5 139–147 Occurrence Handle13065261

    PubMed  Google Scholar 

  75. EJ Gardner (1960) ArticleTitleFollow-up study of a family group exhibiting dominant inheritance for a syndrome including intestinal polyps, osteomas, fibromas, and epidermal cysts Am. J. Hum. Genet. 14 376–390

    Google Scholar 

  76. EJ Gardner FE Stephens (1950) ArticleTitleCancer of the lower digestive tract in one family group Am. J. Hum. Genet. 2 41–48

    Google Scholar 

  77. AH Diwan ED Graves JA King MG Horenstein (2000) ArticleTitleNuchal-type fibroma in two related patients with Gardner’s syndrome Am. J. Surg. Pathol. 24 1563–1567 Occurrence Handle1:STN:280:DC%2BD3M%2FjtlGrtg%3D%3D Occurrence Handle11075861

    CAS  PubMed  Google Scholar 

  78. BM Wehrli SW Weiss CM Coffin (2001) ArticleTitleGardner syndrome Am. J. Surg. Pathol. 25 694–696 Occurrence Handle10.1097/00000478-200105000-00022 Occurrence Handle1:STN:280:DC%2BD3M3ovVKksw%3D%3D

    Article  CAS  Google Scholar 

  79. BM Wehrli SW Weiss S Yandow CM Coffin (2001) ArticleTitleGardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis Am. J. Surg. Pathol. 25 645–651 Occurrence Handle10.1097/00000478-200105000-00012 Occurrence Handle1:STN:280:DC%2BD3M3ktV2rtw%3D%3D Occurrence Handle11342777

    Article  CAS  PubMed  Google Scholar 

  80. MC Oldfield (1954) ArticleTitleThe association of familial polyposis of the colon with multiple sebaceous cysts Br. J. Surg. 41 534–541 Occurrence Handle13149823

    PubMed  Google Scholar 

  81. EW Naylor EJ Gardner RC Richards (1979) ArticleTitleDesmoid tumors and mesenteric fibromatosis in Gardner’s syndrome: report of kindred 109 Arch. Surg. 114 1181–1185 Occurrence Handle1:STN:280:Bi%2BD3MbmvVA%3D Occurrence Handle158353

    CAS  PubMed  Google Scholar 

  82. SK Clark RK Phillips (1996) ArticleTitleDesmoids in familial adenomatous polyposis Br. J. Surg. 83 1494–1504 Occurrence Handle1:STN:280:ByiC28npslY%3D Occurrence Handle9014661

    CAS  PubMed  Google Scholar 

  83. SK Clark K Pack J Pritchard SV Hodgson (1997) ArticleTitleFamilial adenomatous polyposis presenting with childhood desmoids Lancet 349 471–472 Occurrence Handle10.1016/S0140-6736(97)24007-9 Occurrence Handle1:STN:280:ByiC1M3pvVM%3D Occurrence Handle9040579

    Article  CAS  PubMed  Google Scholar 

  84. SK Clark TG Smith DE Katz RH Reznek RK Phillips (1998) ArticleTitleIdentification and progression of a desmoid precursor lesion in patients with familial adenomatous polyposis Br. J. Surg. 85 970–973 Occurrence Handle10.1046/j.1365-2168.1998.00773.x Occurrence Handle1:STN:280:DyaK1czlsFejsQ%3D%3D Occurrence Handle9692575

    Article  CAS  PubMed  Google Scholar 

  85. DM Eccles PW Lunt Y Wallis et al. (1997) ArticleTitleAn unusually severe phenotype for familial adenomatous polyposis Arch. Dis. Child. 77 431–435 Occurrence Handle1:STN:280:DyaK1c7kvV2nug%3D%3D Occurrence Handle9487968

    CAS  PubMed  Google Scholar 

  86. AK Gurbuz FM Giardiello GM Petersen et al. (1994) ArticleTitleDesmoid tumours in familial adenomatous polyposis Gut 35 377–381 Occurrence Handle1:STN:280:ByuB3c%2Fms1M%3D Occurrence Handle8150351

    CAS  PubMed  Google Scholar 

  87. MS Halata J Miller RK Stone (1989) ArticleTitleGardner syndrome. Early presentation with a desmoid tumor. Discovery of multiple colonic polyps Clin. Pediatr. (Phila.) 28 538–540 Occurrence Handle1:STN:280:By%2BD3s7ivFA%3D

    CAS  Google Scholar 

  88. CP Karakousis RA Berjian R Lopez U Rao (1978) ArticleTitleMesenteric fibromatosis in Gardner’s syndrome Arch. Surg. 113 998–1000 Occurrence Handle1:STN:280:CSeB2Mnit1Y%3D Occurrence Handle687099

    CAS  PubMed  Google Scholar 

  89. SC Abraham TT Wu (2001) ArticleTitleNasopharyngeal angiofibroma Hum. Pathol. 32 455 Occurrence Handle10.1053/hupa.2001.24307 Occurrence Handle1:STN:280:DC%2BD3Mzis1SntQ%3D%3D Occurrence Handle11331964

    Article  CAS  PubMed  Google Scholar 

  90. SC Abraham EA Montgomery FM Giardiello TT Wu (2001) ArticleTitleFrequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas Am. J. Pathol. 158 1073–1078 Occurrence Handle1:CAS:528:DC%2BD3MXitlOmsrg%3D Occurrence Handle11238055

    CAS  PubMed  Google Scholar 

  91. FM Giardiello SR Hamilton AJ Krush JA Offerhaus SV Booker GM Petersen (1993) ArticleTitleNasopharyngeal angiofibroma in patients with familial adenomatous polyposis Gastroenterology 105 1550–1552

    Google Scholar 

  92. B Guertl A Beham R Zechner H Stammberger G Hoefler (2000) ArticleTitleNasopharyngeal angiofibroma: an APC-gene-associated tumor? Hum. Pathol. 31 1411–1413 Occurrence Handle10.1053/hupa.2000.19462 Occurrence Handle1:CAS:528:DC%2BD3cXovVektb4%3D Occurrence Handle11112217

    Article  CAS  PubMed  Google Scholar 

  93. SJ Armstrong AW Duncan MG Mott (1991) ArticleTitleRhabdomyosarcoma associated with familial adenomatous polyposis Pediatr. Radiol. 21 445–446 Occurrence Handle1:STN:280:By2D1cnkt1U%3D Occurrence Handle1661001

    CAS  PubMed  Google Scholar 

  94. S Bulow JO Sondergaard I Witt E Larsen G Tetens (1984) ArticleTitleMandibular osteomas in familial polyposis coli Dis. Colon Rectum 27 105–108 Occurrence Handle1:STN:280:BiuC3s7ksFU%3D Occurrence Handle6697825

    CAS  PubMed  Google Scholar 

  95. CH Chang ED Piatt KE Thomas AL Watne (1968) ArticleTitleBone abnormalities in Gardner’s syndrome Am. J. Roentgenol. Radium Ther. Nucl. Med. 103 645–652 Occurrence Handle1:STN:280:CCeA3Mfis1I%3D Occurrence Handle5301730

    CAS  PubMed  Google Scholar 

  96. F Katou K Motegi S Baba (1989) ArticleTitleMandibular lesions in patients with adenomatosis coli J. Craniomaxillofac. Surg. 17 354–358 Occurrence Handle1:STN:280:By%2BD2sfmslY%3D Occurrence Handle2592576

    CAS  PubMed  Google Scholar 

  97. K Kubo H Miyatani Y Takenoshita et al. (1989) ArticleTitleWidespread radiopacity of jaw bones in familial adenomatosis coli J. Craniomaxillofac. Surg. 17 350–353 Occurrence Handle1:STN:280:By%2BD2sfmslU%3D Occurrence Handle2592575

    CAS  PubMed  Google Scholar 

  98. JE Garber FP Li JE Kingston et al. (1988) ArticleTitleHepatoblastoma and familial adenomatous polyposis J. Natl. Cancer Inst. 80 1626–1628 Occurrence Handle1:STN:280:BiaD2Mrot1I%3D Occurrence Handle2848134

    CAS  PubMed  Google Scholar 

  99. FM Giardiello GM Petersen JD Brensinger et al. (1996) ArticleTitleHepatoblastoma and APC gene mutation in familial adenomatous polyposis Gut 39 867–869 Occurrence Handle1:STN:280:ByiC1M%2FntFY%3D Occurrence Handle9038672

    CAS  PubMed  Google Scholar 

  100. LJ Hughes VV Michels (1992) ArticleTitleRisk of hepatoblastoma in familial adenomatous polyposis Am. J. Med. Genet. 43 1023–1025 Occurrence Handle1:STN:280:ByyD3srks1A%3D Occurrence Handle1329510

    CAS  PubMed  Google Scholar 

  101. T Iwama Y Mishima (1994) ArticleTitleMortality in young first-degree relatives of patients with familial adenomatous polyposis Cancer 73 2065–2068 Occurrence Handle1:STN:280:ByuB3cjps1A%3D Occurrence Handle8156512

    CAS  PubMed  Google Scholar 

  102. JE Kingston A Herbert GJ Draper JR Mann (1983) ArticleTitleAssociation between hepatoblastoma and polyposis coli Arch. Dis. Child. 58 959–962 Occurrence Handle1:STN:280:BiuC3czitlc%3D Occurrence Handle6318669

    CAS  PubMed  Google Scholar 

  103. I Uzoaru FJ Podbielski P Chou JG Raffensperger F Gonzalez-Crussi (1993) ArticleTitleFamilial adenomatous polyposis coli and clear cell sarcoma of the kidney Pediatr. Pathol. 13 133–141 Occurrence Handle1:STN:280:ByyB3MbitVQ%3D Occurrence Handle8385323

    CAS  PubMed  Google Scholar 

  104. C Bulow S Bulow (1997) ArticleTitleIs screening for thyroid carcinoma indicated in familial adenomatous polyposis? The Leeds Castle Polyposis Group Int. J. Colorectal Dis. 12 240–242 Occurrence Handle10.1007/s003840050097 Occurrence Handle1:STN:280:ByiH3MfkvVU%3D Occurrence Handle9272455

    Article  CAS  PubMed  Google Scholar 

  105. F Cetta P Toti M Petracci et al. (1997) ArticleTitleThyroid carcinoma associated with familial adenomatous polyposis Histopathology 31 231–236 Occurrence Handle1:STN:280:DyaK1c%2Fhs1GisQ%3D%3D Occurrence Handle9354893

    CAS  PubMed  Google Scholar 

  106. P Marchesa VW Fazio JM Church E McGannon (1997) ArticleTitleAdrenal masses in patients with familial adenomatous polyposis Dis. Colon Rectum 40 1023–1028 Occurrence Handle1:STN:280:ByiH3sfhsFA%3D Occurrence Handle9293929

    CAS  PubMed  Google Scholar 

  107. JT Perkins MO Blackstone RH Riddell (1985) ArticleTitleAdenomatous polyposis coli and multiple endocrine neoplasia type 2b. A pathogenetic relationship Cancer 55 375–381 Occurrence Handle1:STN:280:BiqD2svgtFY%3D Occurrence Handle2856897

    CAS  PubMed  Google Scholar 

  108. WK Cavenee (2000) ArticleTitleHigh-grade gliomas with chromosome 1p loss J. Neurosurg. 92 1080–1081 Occurrence Handle1:STN:280:DC%2BD3c3ps1SqtA%3D%3D Occurrence Handle10839286

    CAS  PubMed  Google Scholar 

  109. F Paraf S Jothy EG Van Meir (1997) ArticleTitleBrain tumor-polyposis syndrome: two genetic diseases? J. Clin. Oncol. 15 2744–2758 Occurrence Handle1:STN:280:ByiA2MfnsFc%3D Occurrence Handle9215849

    CAS  PubMed  Google Scholar 

  110. EG Van Meir (1998) ArticleTitle“Turcot’s syndrome”: phenotype of brain tumors, survival and mode of inheritance Int. J. Cancer 75 162–164 Occurrence Handle10.1002/(SICI)1097-0215(19980105)75:1<162::AID-IJC26>3.3.CO;2-5 Occurrence Handle1:STN:280:DyaK1c%2FovVWisQ%3D%3D Occurrence Handle9426707

    Article  CAS  PubMed  Google Scholar 

  111. EW Naylor E Lebenthal (1980) ArticleTitleGardner’s syndrome. Recent developments in research and management Dig. Dis. Sci. 25 945–959 Occurrence Handle1:STN:280:Bi6D1cboslc%3D Occurrence Handle7004810

    CAS  PubMed  Google Scholar 

  112. JW Milsom KA Ludwig JM Church A Garcia-Ruiz (1997) ArticleTitleLaparoscopic total abdominal colectomy with ileorectal anastomosis for familial adenomatous polyposis Dis. Colon Rectum 40 675–678 Occurrence Handle1:STN:280:ByiA3sjns1M%3D Occurrence Handle9194461

    CAS  PubMed  Google Scholar 

  113. MS Dunker WA Bemelman JF Slors P van Duijvendijk DJ Gouma (2001) ArticleTitleFunctional outcome, quality of life, body image, and cosmesis in patients after laparoscopic-assisted and conventional restorative proctocolectomy: a comparative study Dis. Colon Rectum 44 1800–1807 Occurrence Handle1:STN:280:DC%2BD3MjgtV2jug%3D%3D Occurrence Handle11742165

    CAS  PubMed  Google Scholar 

  114. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {175200} {8/20/2002}: World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  115. J Howell K Pringle B Kirschner JD Burrington (1981) ArticleTitlePeutz-Jeghers polyps causing colo-colic intussusception in infancy J. Pediatr. Surg. 16 82–84 Occurrence Handle1:STN:280:Bi6C1MvgsVc%3D Occurrence Handle7229853

    CAS  PubMed  Google Scholar 

  116. MJ Fernandez Seara MI Martinez Soto JR Fernandez Lorenzo S Trabazo E Gamborino J Forteza Vila (1995) ArticleTitlePeutz-Jeghers syndrome in a neonate J. Pediatr. 126 965–967 Occurrence Handle1:STN:280:ByqB1MvntFM%3D Occurrence Handle7776109

    CAS  PubMed  Google Scholar 

  117. DA Corley TM Uyeki JP Cello (1997) ArticleTitleGastrointestinal bleeding and gastric outlet obstruction from Peutz-Jeghers polyposis. Diagnosis and treatment West J. Med. 166 350–352 Occurrence Handle1:STN:280:ByiA28%2FktlI%3D Occurrence Handle9217445

    CAS  PubMed  Google Scholar 

  118. FM Giardiello JD Brensinger AC Tersmette et al. (2000) ArticleTitleVery high risk of cancer in familial Peutz-Jeghers syndrome Gastroenterology 119 1447–1453 Occurrence Handle1:STN:280:DC%2BD3M%2Fnt1ersw%3D%3D Occurrence Handle11113065

    CAS  PubMed  Google Scholar 

  119. SB Gruber MM Entius GM Petersen et al. (1998) ArticleTitlePathogenesis of adenocarcinoma in Peutz-Jeghers syndrome Cancer Res. 58 5267–5270 Occurrence Handle1:CAS:528:DyaK1cXotVSksbo%3D Occurrence Handle9850045

    CAS  PubMed  Google Scholar 

  120. E Fulcheri P Baracchini A Pagani G Lapertosa G Bussolati (1991) ArticleTitleSignificance of the smooth muscle cell component in Peutz-Jeghers and juvenile polyps Hum. Pathol. 22 1136–1140 Occurrence Handle1:STN:280:By2D2sjmsFc%3D Occurrence Handle1743697

    CAS  PubMed  Google Scholar 

  121. NA Shepherd HJ Bussey JR Jass (1987) ArticleTitleEpithelial misplacement in Peutz-Jeghers polyps. A diagnostic pitfall Am. J. Surg. Pathol. 11 743–749 Occurrence Handle1:STN:280:BieD387kvVA%3D Occurrence Handle3661821

    CAS  PubMed  Google Scholar 

  122. KH Perzin MF Bridge (1982) ArticleTitleAdenomatous and carcinomatous changes in hamartomatous polyps of the small intestine (Peutz-Jeghers syndrome): report of a case and review of the literature Cancer 49 971–983 Occurrence Handle1:STN:280:Bi2C3snmtFQ%3D Occurrence Handle7059931

    CAS  PubMed  Google Scholar 

  123. T Narita T Eto T Ito (1987) ArticleTitlePeutz-Jeghers syndrome with adenomas and adenocarcinomas in colonic polyps Am. J. Surg. Pathol. 11 76–81 Occurrence Handle1:STN:280:BiiD2szpt1A%3D Occurrence Handle3789261

    CAS  PubMed  Google Scholar 

  124. J Utsunomiya H Gocho T Miyanaga E Hamaguchi A Kashimure (1975) ArticleTitlePeutz-Jeghers syndrome: its natural course and management Johns Hopkins Med. J. 136 71–82 Occurrence Handle1:STN:280:CSqC3MjivVE%3D Occurrence Handle1117595

    CAS  PubMed  Google Scholar 

  125. K Hizawa M Iida T Matsumoto et al. (1993) ArticleTitleCancer in Peutz-Jeghers syndrome Cancer 72 2777–2781 Occurrence Handle1:STN:280:ByuD3cnhtVQ%3D Occurrence Handle8402503

    CAS  PubMed  Google Scholar 

  126. A Hemminki D Markie I Tomlinson et al. (1998) ArticleTitleA serine/threonine kinase gene defective in Peutz-Jeghers syndrome Nature 391 184–187 Occurrence Handle10.1038/34432 Occurrence Handle1:CAS:528:DyaK1cXmt1Kjtw%3D%3D Occurrence Handle9428765

    Article  CAS  PubMed  Google Scholar 

  127. DE Jenne H Reimann J Nezu et al. (1998) ArticleTitlePeutz-Jeghers syndrome is caused by mutations in a novel serine threonine kinase Nat. Genet. 18 38–43 Occurrence Handle1:CAS:528:DyaK1cXivFelsw%3D%3D Occurrence Handle9425897

    CAS  PubMed  Google Scholar 

  128. ES d’Amore JC Manivel G Pettinato GA Niehans DC Snover (1991) ArticleTitleIntestinal ganglioneuromatosis: mucosal and transmural types. A clinicopathologic and immunohistochemical study of six cases Hum. Pathol. 22 276–286 Occurrence Handle1:STN:280:By6C28bkvVE%3D Occurrence Handle1706307

    CAS  PubMed  Google Scholar 

  129. CM Fenoglio-Preiser AE Noffsinger GN Stemmermann PE Lantz MB Listrom FO Rilke (1998) Gastrointestinal Pathology, an Atlas and Text 2nd edition, Lippincott-Raven Philadelphia 656, 664–666

    Google Scholar 

  130. JK Kelly JM Langevin LM Price NB Hershfield S Share P Blustein (1986) ArticleTitleGiant and symptomatic inflammatory polyps of the colon in idiopathic inflammatory bowel disease Am. J. Surg. Pathol. 10 420–428 Occurrence Handle1:STN:280:BimB3sbos1w%3D Occurrence Handle3717497

    CAS  PubMed  Google Scholar 

  131. C Torres D Antonioli RD Odze (1998) ArticleTitlePolypoid dysplasia and adenomas in inflammatory bowel disease: a clinical, pathologic, and follow-up study of 89 polyps from 59 patients Am. J. Surg. Pathol. 22 275–284 Occurrence Handle10.1097/00000478-199803000-00001 Occurrence Handle1:STN:280:DyaK1c7mtVCjtw%3D%3D Occurrence Handle9500769

    Article  CAS  PubMed  Google Scholar 

  132. CM Coffin KA Neilson S Ingels R Frank-Gerszberg LP Dehner (1995) ArticleTitleCongenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis Pediatr. Pathol. Lab. Med. 15 571–587 Occurrence Handle1:STN:280:BymC2s%2FjvF0%3D Occurrence Handle8597844

    CAS  PubMed  Google Scholar 

  133. AE Walts M Asch C Raj (1982) ArticleTitleSolitary lesion of congenital fibromatosis. A rare cause of neonatal intestinal obstruction Am. J. Surg. Pathol. 6 255–260 Occurrence Handle1:STN:280:Bi2B2Mvislw%3D Occurrence Handle7102903

    CAS  PubMed  Google Scholar 

  134. JR Srigley K Mancer (1984) ArticleTitleSolitary intestinal fibromatosis with perinatal bowel obstruction Pediatr. Pathol. 2 249–258 Occurrence Handle1:STN:280:BiqD1cfisFE%3D Occurrence Handle6542653

    CAS  PubMed  Google Scholar 

  135. IK Mecrow V Miller M Lendon CM Doig (1990) ArticleTitleMesenteric fibromatosis presenting with ascites in childhood J. Pediatr. Gastroenterol. Nutr. 11 118–122 Occurrence Handle1:STN:280:By%2BA2MvpsFI%3D Occurrence Handle2388122

    CAS  PubMed  Google Scholar 

  136. M Michel J Ninane D Claus S Gosseye FX Wese D Moulin (1990) ArticleTitleMajor malformations in a case of infantile myofibromatosis Eur. J. Pediatr. 149 251–252 Occurrence Handle1:STN:280:By%2BC2M7ns1Y%3D Occurrence Handle2303073

    CAS  PubMed  Google Scholar 

  137. EB Chung FM Enzinger (1981) ArticleTitleInfantile myofibromatosis Cancer 48 1807–1818 Occurrence Handle1:STN:280:Bi2D38rjtV0%3D Occurrence Handle7284977

    CAS  PubMed  Google Scholar 

  138. T Mentzel E Calonje AG Nascimento CD Fletcher (1994) ArticleTitleInfantile hemangiopericytoma versus infantile myofibromatosis. Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions Am. J. Surg. Pathol. 18 922–930 Occurrence Handle1:STN:280:ByuA2cnlsF0%3D Occurrence Handle8067513

    CAS  PubMed  Google Scholar 

  139. CM Coffin LP Dehner (1993) ArticleTitleVascular tumors in children and adolescents: a clinicopathologic study of 228 tumors in 222 patients Pathol. Annu. 28 IssueIDPt 1 97–120 Occurrence Handle8416140

    PubMed  Google Scholar 

  140. SM Hammoudi JJ Corkery (1985) ArticleTitleCongenital hemangiopericytoma of duodenum J. Pediatr. Surg. 20 559–560 Occurrence Handle1:STN:280:BimD3snntF0%3D Occurrence Handle4057028

    CAS  PubMed  Google Scholar 

  141. FM Quinn S Brown D O’Hara (1991) ArticleTitleHemangiopericytoma of the stomach in a neonate J. Pediatr. Surg. 26 101–102 Occurrence Handle1:STN:280:By6C2s7gvFQ%3D Occurrence Handle2005513

    CAS  PubMed  Google Scholar 

  142. MR Poplausky JO Haller LS Gerson (1992) ArticleTitleCongenital hemangiopericytoma of the duodenum Pediatr. Radiol. 22 344–345 Occurrence Handle1:STN:280:ByyD38nnvFE%3D Occurrence Handle1408441

    CAS  PubMed  Google Scholar 

  143. CM Coffin LP Dehner (1991) ArticleTitleFibroblastic-myofibroblastic tumors in children and adolescents: a clinicopathologic study of 108 examples in 103 patients Pediatr. Pathol. 11 569–588 Occurrence Handle1:STN:280:By2D2M7msVE%3D Occurrence Handle1946077

    CAS  PubMed  Google Scholar 

  144. PW Allen (1977) ArticleTitleThe fibromatoses: a clinicopathologic classification based on 140 cases Am. J. Surg. Pathol. 1 255–270 Occurrence Handle1:STN:280:CSeD3sfhsFw%3D Occurrence Handle920873

    CAS  PubMed  Google Scholar 

  145. JA Bar-Maor U Shabshin (1993) ArticleTitleMesenteric fibromatosis J. Pediatr. Surg. 28 1618–1619 Occurrence Handle1:STN:280:ByuC3szgs1I%3D Occurrence Handle8301512

    CAS  PubMed  Google Scholar 

  146. CM Coffin J Watterson JR Priest LP Dehner (1995) ArticleTitleExtrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases Am. J. Surg. Pathol. 19 859–872 Occurrence Handle1:STN:280:ByqA3srpsVM%3D Occurrence Handle7611533

    CAS  PubMed  Google Scholar 

  147. TT Tang AD Segura HW Oechler et al. (1990) ArticleTitleInflammatory myofibrohistiocytic proliferation simulating sarcoma in children Cancer 65 1626–1634 Occurrence Handle1:STN:280:By%2BC1c3ls1Q%3D Occurrence Handle2311072

    CAS  PubMed  Google Scholar 

  148. CK Maves JF Johnson K Bove RL Malott (1989) ArticleTitleGastric inflammatory pseudotumor in children Radiology 173 381–383 Occurrence Handle1:STN:280:By%2BD3MfjvVE%3D Occurrence Handle2678252

    CAS  PubMed  Google Scholar 

  149. K Kojimahara M Mukai K Yamazaki et al. (1993) ArticleTitleInflammatory pseudotumor of the stomach: report of a highly infiltrative case with electron microscopic and immunohistochemical studies Acta. Pathol. Jpn. 43 65–70 Occurrence Handle1:STN:280:ByyB38%2FkvVc%3D Occurrence Handle8465658

    CAS  PubMed  Google Scholar 

  150. DL Day S Sane LP Dehner (1986) ArticleTitleInflammatory pseudotumor of the mesentery and small intestine Pediatr. Radiol. 16 210–215 Occurrence Handle1:STN:280:BimB3crgslU%3D Occurrence Handle3703596

    CAS  PubMed  Google Scholar 

  151. G Pettinato JC Manivel N De Rosa LP Dehner (1990) ArticleTitleInflammatory myofibroblastic tumor (plasma cell granuloma). Clinicopathologic study of 20 cases with immunohistochemical and ultrastructural observations Am. J. Clin. Pathol. 94 538–546 Occurrence Handle1:STN:280:By6D2M7jvVE%3D Occurrence Handle2239820

    CAS  PubMed  Google Scholar 

  152. JM Meis FM Enzinger (1991) ArticleTitleInflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor Am. J. Surg. Pathol. 15 1146–1156 Occurrence Handle1:STN:280:By2D1c7pt1Q%3D Occurrence Handle1746682

    CAS  PubMed  Google Scholar 

  153. SW Weiss (1994) Histologic Typing of Soft Tissue Tumors 2nd edition, Springer-Verlag New York

    Google Scholar 

  154. F Gonzalez-Crussi DE de Mello C Sotelo-Avila (1983) ArticleTitleOmental-mesenteric myxoid hamartomas. Infantile lesions simulating malignant tumors Am. J. Surg. Pathol. 7 567–578 Occurrence Handle1:STN:280:BiuD387lt1U%3D Occurrence Handle6625050

    CAS  PubMed  Google Scholar 

  155. GR Shimer EB Helwig (1984) ArticleTitleInflammatory fibroid polyps of the intestine Am. J. Clin. Pathol. 81 708–714 Occurrence Handle1:STN:280:BiuB38fjsVY%3D Occurrence Handle6731350

    CAS  PubMed  Google Scholar 

  156. PP Anthony DS Morris KD Vowles (1984) ArticleTitleMultiple and recurrent inflammatory fibroid polyps in three generations of a Devon family: a new syndrome Gut 25 854–862 Occurrence Handle1:STN:280:BiuB283lvFU%3D Occurrence Handle6745724

    CAS  PubMed  Google Scholar 

  157. RO Allibone JK Nanson PP Anthony (1992) ArticleTitleMultiple and recurrent inflammatory fibroid polyps in a Devon family (‘Devon polyposis syndrome’): an update Gut 33 1004–1005 Occurrence Handle1:STN:280:By2A2MvpsVE%3D Occurrence Handle1644320

    CAS  PubMed  Google Scholar 

  158. JS Hyams JE Haswell MA Gerber MM Berman (1985) ArticleTitleColonic ulceration in histiocytosis X J. Pediatr. Gastroenterol. Nutr. 4 286–290 Occurrence Handle1:STN:280:BiqC1crgtlQ%3D Occurrence Handle3872932

    CAS  PubMed  Google Scholar 

  159. M Iwafuchi H Watanabe M Shiratsuka (1990) ArticleTitlePrimary benign histiocytosis X of the stomach. A report of a case showing spontaneous remission after 5 1/2 years Am. J. Surg. Pathol. 14 489–496 Occurrence Handle1:STN:280:By%2BB38rit1Q%3D Occurrence Handle2183638

    CAS  PubMed  Google Scholar 

  160. RG Lee RM Braziel P Stenzel (1990) ArticleTitleGastrointestinal involvement in Langerhans cell histiocytosis (histiocytosis X): diagnosis by rectal biopsy Mod. Pathol. 3 154–157 Occurrence Handle1:STN:280:By%2BB383htVc%3D Occurrence Handle2183212

    CAS  PubMed  Google Scholar 

  161. LA Boccon-Gibod HA Krichen LM Carlier-Mercier JF Salaun JL Fontaine GR Leverger (1992) ArticleTitleDigestive tract involvement with exudative enteropathy in Langerhans cell histiocytosis Pediatr. Pathol. 12 515–524 Occurrence Handle1:STN:280:ByyD38josFE%3D Occurrence Handle1409150

    CAS  PubMed  Google Scholar 

  162. R Wada S Yagihashi R Konta T Ueda T Izumiyama (1992) ArticleTitleGastric polyposis caused by multifocal histiocytosis X Gut 33 994–996 Occurrence Handle1:STN:280:By2A2MvpslE%3D Occurrence Handle1644344

    CAS  PubMed  Google Scholar 

  163. GM Groisman JR Rosh N Harpaz (1994) ArticleTitleLangerhans cell histiocytosis of the stomach. A cause of granulomatous gastritis and gastric polyposis Arch. Pathol. Lab. Med. 118 1232–1235 Occurrence Handle1:STN:280:ByqD2s%2FntFE%3D Occurrence Handle7979922

    CAS  PubMed  Google Scholar 

  164. Online Mendelian Inheritance in Man, OMIM (TM). John Hopkins University, Baltimore, MD. MIM Number: {109400} {5/21/1997}: World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/

  165. RA Schwartz (1978) ArticleTitleBasal-cell-nevus syndrome and gastrointestinal polyposis N. Engl. J. Med. 299 49 Occurrence Handle1:STN:280:CSeC1Mbmt1I%3D

    CAS  Google Scholar 

  166. H Shimizu Y Shimomura I Kobayashi S Kobayashi (1987) ArticleTitle[A case of basal cell nevus syndrome with gastrointestinal polyposis accompanying the various endocrinological abnormalities] Nippon Naika Gakkai Zasshi 76 844–848 Occurrence Handle1:STN:280:BieD3cbkt1U%3D Occurrence Handle3655495

    CAS  PubMed  Google Scholar 

  167. JD Atwell D Burge D Wright (1985) ArticleTitleNodular lymphoid hyperplasia of the intestinal tract in infancy and childhood J. Pediatr. Surg. 20 25–29 Occurrence Handle1:STN:280:BiqC3svit1c%3D Occurrence Handle3973808

    CAS  PubMed  Google Scholar 

  168. MA Capitanio JA Kirkpatrick (1970) ArticleTitleRoentgenographic evaluation of intestinal obstruction in the newborn infant Pediatr. Clin. North Am. 17 983–1001 Occurrence Handle1:STN:280:CS6D3sfisF0%3D Occurrence Handle5529801

    CAS  PubMed  Google Scholar 

  169. M Ranchod KJ Lewin RF Dorfman (1978) ArticleTitleLymphoid hyperplasia of the gastrointestinal tract. A study of 26 cases and review of the literature Am. J. Surg. Pathol. 2 383–400 Occurrence Handle1:STN:280:CSaC3c7lvFc%3D Occurrence Handle736212

    CAS  PubMed  Google Scholar 

  170. JJ Brooks HT Enterline (1983) ArticleTitlePrimary gastric lymphomas. A clinicopathologic study of 58 cases with long-term follow-up and literature review Cancer 51 701–711 Occurrence Handle1:STN:280:BiyC3c3nslU%3D Occurrence Handle6336982

    CAS  PubMed  Google Scholar 

  171. CM Coffin AL Pappin (1997) Polyps and neoplasms of the gastrointestinal tract in childhood and adolescents BB Dahms S Qualman (Eds) Perspectives in Pediatric Pathology, Volume 20 S. Karger AG Basel, Switzerland 127–171

    Google Scholar 

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ACKNOWLEDGMENTS

Portions of the text, tables, and figures in this article are an updated version of a previous article (from Coffin and Pappin [171], with permission of S. Karger AG, Basel). We thank Samantha Farr and Krista Spilker for manuscript preparation.

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Lowichik, A., Jackson, W. & Coffin, C. Gastrointestinal Polyposis in Childhood: Clinicopathologic and Genetic Features . Pediatr. Dev. Pathol. 6, 371–391 (2003). https://doi.org/10.1007/s10024-002-0701-x

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