Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome

H. T. Lynch, Thomas Christopher Smyrk, S. J. Lanspa, J. X. Jenkins, P. M. Lynch, J. Cavalieri, J. F. Lynch

Research output: Contribution to journalArticle

43 Citations (Scopus)

Abstract

Background. The hereditary flat adenoma syndrome (HFAS) is characterized by an autosomal dominantly inherited predisposition to multiple colonic adenomas (usually less than 100) with proximal predominance and flat as opposed to polypoid growth. Patients with the syndrome experience colorectal cancers in excess, and the lesions are distributed randomly in the colon. The polyps occur at a later age (median, 55 years) compared with age at onset of polyps in patients with familial adenomatous polyposis (FAP) and patients with the Lynch syndromes. FAP and HFAS are linked to the same locus on chromosome 5q21-q22. Methods. Upper endoscopic examination was performed on 22 relatives from four families with HFAS. Results. Fifteen patients from three of these families had multiple fundic gland polyps; four had duodenal or gastric adenomas. Periampullary carcinoma was present in two members from separate families. Conclusions. The authors conclude that the upper gastrointestinal tract pathologic characteristics of HFAS are similar to those described in FAP. Thus, those at risk for HFAS require upper endoscopic screening in addition to meticulous attention to the colon.

Original languageEnglish (US)
Pages (from-to)2709-2714
Number of pages6
JournalCancer
Volume71
Issue number9
StatePublished - 1993
Externally publishedYes

Fingerprint

Adenoma
Adenomatous Polyposis Coli
Polyps
Colon
Hereditary Nonpolyposis Colorectal Neoplasms
Upper Gastrointestinal Tract
Age of Onset
Colorectal Neoplasms
Stomach
Chromosomes
Carcinoma
Growth

Keywords

  • colorectal cancer
  • flat adenomas
  • fundic gland polyps
  • genetics
  • hereditary flat adenoma syndrome

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Lynch, H. T., Smyrk, T. C., Lanspa, S. J., Jenkins, J. X., Lynch, P. M., Cavalieri, J., & Lynch, J. F. (1993). Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome. Cancer, 71(9), 2709-2714.

Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome. / Lynch, H. T.; Smyrk, Thomas Christopher; Lanspa, S. J.; Jenkins, J. X.; Lynch, P. M.; Cavalieri, J.; Lynch, J. F.

In: Cancer, Vol. 71, No. 9, 1993, p. 2709-2714.

Research output: Contribution to journalArticle

Lynch, HT, Smyrk, TC, Lanspa, SJ, Jenkins, JX, Lynch, PM, Cavalieri, J & Lynch, JF 1993, 'Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome', Cancer, vol. 71, no. 9, pp. 2709-2714.
Lynch HT, Smyrk TC, Lanspa SJ, Jenkins JX, Lynch PM, Cavalieri J et al. Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome. Cancer. 1993;71(9):2709-2714.
Lynch, H. T. ; Smyrk, Thomas Christopher ; Lanspa, S. J. ; Jenkins, J. X. ; Lynch, P. M. ; Cavalieri, J. ; Lynch, J. F. / Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome. In: Cancer. 1993 ; Vol. 71, No. 9. pp. 2709-2714.
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N2 - Background. The hereditary flat adenoma syndrome (HFAS) is characterized by an autosomal dominantly inherited predisposition to multiple colonic adenomas (usually less than 100) with proximal predominance and flat as opposed to polypoid growth. Patients with the syndrome experience colorectal cancers in excess, and the lesions are distributed randomly in the colon. The polyps occur at a later age (median, 55 years) compared with age at onset of polyps in patients with familial adenomatous polyposis (FAP) and patients with the Lynch syndromes. FAP and HFAS are linked to the same locus on chromosome 5q21-q22. Methods. Upper endoscopic examination was performed on 22 relatives from four families with HFAS. Results. Fifteen patients from three of these families had multiple fundic gland polyps; four had duodenal or gastric adenomas. Periampullary carcinoma was present in two members from separate families. Conclusions. The authors conclude that the upper gastrointestinal tract pathologic characteristics of HFAS are similar to those described in FAP. Thus, those at risk for HFAS require upper endoscopic screening in addition to meticulous attention to the colon.

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