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Familial Polyposis of the Entire Gastrointestinal Tract

Familial Polyposis of the Entire Gastrointestinal Tract Abstract WITHIN the past two years, three similar cases of familial polyposis diffusely involving the entire gastrointestinal tract have been encountered. In all cases, innumerable polyposis were demonstrated in the stomach, small bowel, and colorectum by x-ray film studies. Because of the extreme rarity of this condition it is difficult to determine whether it represents a variant of the usual "familial polyposis of the colon." The literature was reviewed, three additional cases presented, and a thorough presentation of the subject follows. Report of Cases Case 1.—A 9-year-old girl was first seen at the City of Hope Medical Center on March 27, 1966, with a history of frequent stools (three to four a day) and vague abdominal pain. She had passed mucus and blood through the rectum one week prior to admission. A barium enema showed extensive polyposis of the entire colon.The family history revealed that her father died at age References 1. Morson, B.C.: Precancerous Lesions of the Colon and Rectum , JAMA 179:316-321 ( (Feb 3) ) 1962.Crossref 2. Ravitch, M.M.: Polypoid Adenomatosis of the Entire Gastrointestinal Tract , Ann Surg 128:283-298 ( (July) ) 1948.Crossref 3. Veale, A.M.O.: Intestinal Polyposis , London: Cambridge University Press, 1965. 4. McKusick, V.A.: Genetic Factors in Intestinal Polyposis , JAMA 182:271-277 ( (Oct 20) ) 1962.Crossref 5. Peutz, J.L.A.: Over enn zeer merkwaardige, gecombineerde familiaire polyposis van de slijmvliezen van den tractus intestinalis met die van de neuskeelholte en gepaard met eigenaardige pigmentaties van huid en slijmvliezen , Nederl Maandschr Geneesk Leiden 10:134-146, 1921. 6. Jeghers, H.; McKusick, V.A.; and Katz, K.H.: Generalized Intestinal Polyposis and Melanin Spots of the Oral Mucosa, Lips and Digits: A Syndrome of Diagnostic Significance , New Eng J Med 241:1031-1036 ( (Dec 29) ) 1949.Crossref 7. Gardner, E.J.: Genetic and Clinical Study of Intestinal Polyposis, Predisposing Factor for Carcinoma and Rectum , Amer J Hum Genet 3:167-176 ( (March) ) 1951. 8. Turcot, J.; Despres, M.P.; and St. Pierre, F.: Malignant Tumors of Central Nervous System Associated With Familial Polyposis of Colon , Dis Colon Rectum 2:465-468 ( (Sept) -Oct) 1959.Crossref 9. Cronkhite, L.W., Jr., and Canada, W.J.: Generalized Gastrointestinal Polyposis: An Unusual Syndrome of Polyposis, Pigmentation, Alopecia, and Onychotrophia , New Eng J Med 252:1011-1015 ( (June 16) ) 1955.Crossref 10. Manousos, O., and Webster, C.U.: Diffuse Gastrointestinal Polyposis With Ectodermal Changes , Gut 7:375-379 ( (Aug) ) 1966.Crossref 11. Zollinger, R.M.: Endocrine Adenomas and Peptic Ulcer With Special Reference to Pancreatic Adenomas , Gastroenterology 39:541-543 ( (Nov) ) 1960. 12. Woolf, C.M.; Richards, R.C.; and Gardner, E.J.: Occasional Discrete Polyps of the Colon and Rectum Showing an Inherited Tendency in Kindred , Cancer 8:403-408 ( (March) -April) 1955.Crossref 13. Helwig, E.B.: The Evolution of Adenomas of the Large Intestine and Their Relation to Carcinoma , Surg Gynec Obstet 84:36-49 ( (Jan) ) 1947. 14. Bacon, H.E.: Anus, Rectum and Sigmoid Colon: Diagnosis and Treatment , ed 3, Philadelphia: J. B. Lippincott Co., 1949. 15. Andren, L., and Frieberg, S.: Frequency of Polyps of Rectum and Colon, According to Age, and Relation to Cancer , Gastroenterology 36:631-632 ( (May) ) 1959. 16. Rider, J.A., et al: Polyps of Colon and Rectum: A Four-Year to Nine-Year Follow-up Study of 537 Patients , JAMA 170:633-638 ( (June 6) ) 1959.Crossref 17. Luschka, quoted by Mayo, C.W., and Wakefield, E.G.: Disseminated Polyposis of the Colon , JAMA 107:342-348 ( (Aug) ) 1936.Crossref 18. Lebert, quoted by Hewitt, J.H., and Howard, W.T.: Chronic Ulcerative Colitis With Polyps , Arch Intern Med 15:714-723 ( (May) ) 1915. 19. Cripps, H.: Two Cases of Disseminated Polypus of Rectum , Trans Path Soc London 33:165, 1882. 20. Dukes, C.E.: Hereditary Factor in Polyposis Intestini or Multiple Adenomata , Cancer Rev 5:241-256 ( (April) ) 1930. 21. LeFevre, H.W., and Jacques, T.F.: Multiple Polyposis in an Infant of Four Months , Amer J Surg 81:90-91 ( (Jan) ) 1951.Crossref 22. Dukes, C.E.: Familial Intestinal Polyposis , Ann Eugen London 17:1-29 ( (Aug) ) 1952.Crossref 23. Gross, R.E.: The Surgery of Infancy and Childhood , Philadelphia: W. B. Saunders Co., 1953. 24. Everson, T.C., and Allen, M.J.: Subtotal Colectomy With Ileosigmoidostomy and Fulguration of Polyps in Retained Colon , Arch Surg 69:806-817 ( (Nov) ) 1954.Crossref 25. Ravitch, M.M., and Sabiston, D.C., Jr.: Anal Ileostomy With Preservation of the Sphincter , Surg Gynec Obstet 84:1095-1099 ( (June) ) 1947. 26. Hubbard, T.B., Jr.: Familial Polyposis of the Colon: The Fate of the Retained Rectum After Colectomy in Children , Amer Surg 23:577-586 ( (June) ) 1957. 27. Cole, J.W., and Holden, W.D.: Postcolectomy Regression of Adenomatous Polyps of Rectum , Arch Surg 79:385-392 ( (Sept) ) 1959.Crossref 28. Dunphy, J.E.; Patterson, W.B.; and Legg, M.A.: Etiologic Factors in Polyposis and Carcinoma of Colon , Ann Surg 150:488-498 ( (Sept) ) 1959.Crossref 29. Neel, J.V.: Problems in the Estimation of the Frequency of Uncommon Inherited Traits , Amer J Hum Genet 6:51-60 ( (March) ) 1954. 30. Reed, T.E., and Neel, J.V.: A Genetic Study of Multiple Polyposis of the Colon (With an Appendix Deriving a Method of Estimating Relative Fitness) , Amer J Hum Genet 7:236-263 ( (Sept) ) 1955. 31. Pollack, J.L., and Swinton, N.W.: Congenital Polyposis of the Colon With Extension to the Small Intestine and Stomach , Lahey Clin Found Bull 9:174-179 ( (Oct) ) 1955. 32. Gumpel, R.C., and Carballo, J.D.: A New Concept of Familial Adenomatosis , Ann Intern Med 45:1045-1058 ( (Dec) ) 1956.Crossref 33. Lazar, H.P., and Bendix, R.M.: Familial Polyposis With External Manifestations , Gastroenterology 36:841-847 ( (June) ) 1959. 34. Boley, S.J.; McKinnon, W.M.P.; and Marzulli, V.F.: The Management of Familial Gastrointestinal Polyposis Involving Stomach and Colon , Surgery 50:691-696 ( (Oct) ) 1961. 35. Hullsiek, H.E.: Multiple Polyposis of Colon , Surg Gynec Obstet 47:346-356 ( (Sept) ) 1928. 36. Turner, F.P.: Familial Polyposis of the Large Intestine , Ann Surg 146:131-135 ( (July) ) 1957.Crossref 37. Pack, G.T., and Ariel, I.M.: Treatment of Cancer and Allied Diseases, Tumors of Gastro-Intestinal Tract, Pancreas, Biliary System , and Liver, ed 2, New York: Paul B. Hoeber, Inc., Medical Bk. Dept. of Harper & Row, 1962, vol 5. 38. Struthers, J.E.: Multiple Polyposis of the Intestinal Tract , Ann Surg 72:649-664 ( (Dec) ) 1920.Crossref 39. Gatersleben, H.: Beitrag zur Polyposis des Dünndarms , Deutsch Z Chir 245:628-640 (No. (9) -10) 1935.Crossref 40. Cabot Case: Multiple Polyposis of Duodenum, Jejunum, Ileum, and Large Bowel , New Eng J Med 212:263-267 ( (Feb 7) ) 1935.Crossref 41. Glass, F.A.: Multiple Polyposis of Gastro-Intestinal Tract , J Okla Med Assoc 33:1-3 ( (Oct) ) 1940. 42. Brachetto-Brian, D., and Lascano, E.F.: Polyposis Generalizada Gastro-Intestinal , Rev Asoc Med Argent 56:385-388 ( (July) ) 1942. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Familial Polyposis of the Entire Gastrointestinal Tract

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Publisher
American Medical Association
Copyright
Copyright © 1969 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1969.01340160007003
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Abstract

Abstract WITHIN the past two years, three similar cases of familial polyposis diffusely involving the entire gastrointestinal tract have been encountered. In all cases, innumerable polyposis were demonstrated in the stomach, small bowel, and colorectum by x-ray film studies. Because of the extreme rarity of this condition it is difficult to determine whether it represents a variant of the usual "familial polyposis of the colon." The literature was reviewed, three additional cases presented, and a thorough presentation of the subject follows. Report of Cases Case 1.—A 9-year-old girl was first seen at the City of Hope Medical Center on March 27, 1966, with a history of frequent stools (three to four a day) and vague abdominal pain. She had passed mucus and blood through the rectum one week prior to admission. A barium enema showed extensive polyposis of the entire colon.The family history revealed that her father died at age References 1. Morson, B.C.: Precancerous Lesions of the Colon and Rectum , JAMA 179:316-321 ( (Feb 3) ) 1962.Crossref 2. Ravitch, M.M.: Polypoid Adenomatosis of the Entire Gastrointestinal Tract , Ann Surg 128:283-298 ( (July) ) 1948.Crossref 3. Veale, A.M.O.: Intestinal Polyposis , London: Cambridge University Press, 1965. 4. McKusick, V.A.: Genetic Factors in Intestinal Polyposis , JAMA 182:271-277 ( (Oct 20) ) 1962.Crossref 5. Peutz, J.L.A.: Over enn zeer merkwaardige, gecombineerde familiaire polyposis van de slijmvliezen van den tractus intestinalis met die van de neuskeelholte en gepaard met eigenaardige pigmentaties van huid en slijmvliezen , Nederl Maandschr Geneesk Leiden 10:134-146, 1921. 6. Jeghers, H.; McKusick, V.A.; and Katz, K.H.: Generalized Intestinal Polyposis and Melanin Spots of the Oral Mucosa, Lips and Digits: A Syndrome of Diagnostic Significance , New Eng J Med 241:1031-1036 ( (Dec 29) ) 1949.Crossref 7. Gardner, E.J.: Genetic and Clinical Study of Intestinal Polyposis, Predisposing Factor for Carcinoma and Rectum , Amer J Hum Genet 3:167-176 ( (March) ) 1951. 8. Turcot, J.; Despres, M.P.; and St. Pierre, F.: Malignant Tumors of Central Nervous System Associated With Familial Polyposis of Colon , Dis Colon Rectum 2:465-468 ( (Sept) -Oct) 1959.Crossref 9. Cronkhite, L.W., Jr., and Canada, W.J.: Generalized Gastrointestinal Polyposis: An Unusual Syndrome of Polyposis, Pigmentation, Alopecia, and Onychotrophia , New Eng J Med 252:1011-1015 ( (June 16) ) 1955.Crossref 10. Manousos, O., and Webster, C.U.: Diffuse Gastrointestinal Polyposis With Ectodermal Changes , Gut 7:375-379 ( (Aug) ) 1966.Crossref 11. Zollinger, R.M.: Endocrine Adenomas and Peptic Ulcer With Special Reference to Pancreatic Adenomas , Gastroenterology 39:541-543 ( (Nov) ) 1960. 12. Woolf, C.M.; Richards, R.C.; and Gardner, E.J.: Occasional Discrete Polyps of the Colon and Rectum Showing an Inherited Tendency in Kindred , Cancer 8:403-408 ( (March) -April) 1955.Crossref 13. Helwig, E.B.: The Evolution of Adenomas of the Large Intestine and Their Relation to Carcinoma , Surg Gynec Obstet 84:36-49 ( (Jan) ) 1947. 14. Bacon, H.E.: Anus, Rectum and Sigmoid Colon: Diagnosis and Treatment , ed 3, Philadelphia: J. B. Lippincott Co., 1949. 15. Andren, L., and Frieberg, S.: Frequency of Polyps of Rectum and Colon, According to Age, and Relation to Cancer , Gastroenterology 36:631-632 ( (May) ) 1959. 16. Rider, J.A., et al: Polyps of Colon and Rectum: A Four-Year to Nine-Year Follow-up Study of 537 Patients , JAMA 170:633-638 ( (June 6) ) 1959.Crossref 17. Luschka, quoted by Mayo, C.W., and Wakefield, E.G.: Disseminated Polyposis of the Colon , JAMA 107:342-348 ( (Aug) ) 1936.Crossref 18. Lebert, quoted by Hewitt, J.H., and Howard, W.T.: Chronic Ulcerative Colitis With Polyps , Arch Intern Med 15:714-723 ( (May) ) 1915. 19. Cripps, H.: Two Cases of Disseminated Polypus of Rectum , Trans Path Soc London 33:165, 1882. 20. Dukes, C.E.: Hereditary Factor in Polyposis Intestini or Multiple Adenomata , Cancer Rev 5:241-256 ( (April) ) 1930. 21. LeFevre, H.W., and Jacques, T.F.: Multiple Polyposis in an Infant of Four Months , Amer J Surg 81:90-91 ( (Jan) ) 1951.Crossref 22. Dukes, C.E.: Familial Intestinal Polyposis , Ann Eugen London 17:1-29 ( (Aug) ) 1952.Crossref 23. Gross, R.E.: The Surgery of Infancy and Childhood , Philadelphia: W. B. Saunders Co., 1953. 24. Everson, T.C., and Allen, M.J.: Subtotal Colectomy With Ileosigmoidostomy and Fulguration of Polyps in Retained Colon , Arch Surg 69:806-817 ( (Nov) ) 1954.Crossref 25. Ravitch, M.M., and Sabiston, D.C., Jr.: Anal Ileostomy With Preservation of the Sphincter , Surg Gynec Obstet 84:1095-1099 ( (June) ) 1947. 26. Hubbard, T.B., Jr.: Familial Polyposis of the Colon: The Fate of the Retained Rectum After Colectomy in Children , Amer Surg 23:577-586 ( (June) ) 1957. 27. Cole, J.W., and Holden, W.D.: Postcolectomy Regression of Adenomatous Polyps of Rectum , Arch Surg 79:385-392 ( (Sept) ) 1959.Crossref 28. Dunphy, J.E.; Patterson, W.B.; and Legg, M.A.: Etiologic Factors in Polyposis and Carcinoma of Colon , Ann Surg 150:488-498 ( (Sept) ) 1959.Crossref 29. Neel, J.V.: Problems in the Estimation of the Frequency of Uncommon Inherited Traits , Amer J Hum Genet 6:51-60 ( (March) ) 1954. 30. Reed, T.E., and Neel, J.V.: A Genetic Study of Multiple Polyposis of the Colon (With an Appendix Deriving a Method of Estimating Relative Fitness) , Amer J Hum Genet 7:236-263 ( (Sept) ) 1955. 31. Pollack, J.L., and Swinton, N.W.: Congenital Polyposis of the Colon With Extension to the Small Intestine and Stomach , Lahey Clin Found Bull 9:174-179 ( (Oct) ) 1955. 32. Gumpel, R.C., and Carballo, J.D.: A New Concept of Familial Adenomatosis , Ann Intern Med 45:1045-1058 ( (Dec) ) 1956.Crossref 33. Lazar, H.P., and Bendix, R.M.: Familial Polyposis With External Manifestations , Gastroenterology 36:841-847 ( (June) ) 1959. 34. Boley, S.J.; McKinnon, W.M.P.; and Marzulli, V.F.: The Management of Familial Gastrointestinal Polyposis Involving Stomach and Colon , Surgery 50:691-696 ( (Oct) ) 1961. 35. Hullsiek, H.E.: Multiple Polyposis of Colon , Surg Gynec Obstet 47:346-356 ( (Sept) ) 1928. 36. Turner, F.P.: Familial Polyposis of the Large Intestine , Ann Surg 146:131-135 ( (July) ) 1957.Crossref 37. Pack, G.T., and Ariel, I.M.: Treatment of Cancer and Allied Diseases, Tumors of Gastro-Intestinal Tract, Pancreas, Biliary System , and Liver, ed 2, New York: Paul B. Hoeber, Inc., Medical Bk. Dept. of Harper & Row, 1962, vol 5. 38. Struthers, J.E.: Multiple Polyposis of the Intestinal Tract , Ann Surg 72:649-664 ( (Dec) ) 1920.Crossref 39. Gatersleben, H.: Beitrag zur Polyposis des Dünndarms , Deutsch Z Chir 245:628-640 (No. (9) -10) 1935.Crossref 40. Cabot Case: Multiple Polyposis of Duodenum, Jejunum, Ileum, and Large Bowel , New Eng J Med 212:263-267 ( (Feb 7) ) 1935.Crossref 41. Glass, F.A.: Multiple Polyposis of Gastro-Intestinal Tract , J Okla Med Assoc 33:1-3 ( (Oct) ) 1940. 42. Brachetto-Brian, D., and Lascano, E.F.: Polyposis Generalizada Gastro-Intestinal , Rev Asoc Med Argent 56:385-388 ( (July) ) 1942.

Journal

Archives of SurgeryAmerican Medical Association

Published: Oct 1, 1969

References