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Prévalence et suivi endoscopique des lésions digestives extra-coliques de la polyadénomatose familiale

Prevalence and endoscopic surveillance of extra-colic digestive lesions in familial adenomatous polyposis

Résumé

Nous avons étudié 120 malades colectomisés pour une polypose adénomateuse familiale (PAF) ayant bénéficié d’au moins une endoscopie digestive haute pour rechercher des adénomes gastriques, duodénaux et papillaires. Une iléoscopie par voie basse a également été réalisée chez 42 de ces patients. Le typage génétique de la mutation responsable a été réalisé chez 84 de ces malades.

Globalement un ou plusieurs adénomes du tube digestif ont été observés chez 54 patients (44,6 %). Il s’agissait d’adénomes de la papille duodénale dans 39 cas, duodénaux à distance de la papille dans 43 cas, d’adénomes gastriques dans 7 cas. Quatre cancers de la papille ont été observés. Des adénomes de l’iléon étaient présents chez 20 patients (48 %). Il s’agissait d’adénomes tubuleux dans 15 cas et d’adénomes villeux dans 5 cas. Le risque de dégénérescence de ces adénomes iléaux est inconnu. Leur grande fréquence contraste avec la relative rareté des cancers iléaux observés au cours des PAF.

Aucune relation n’a été mise en évidence entre l’importance des lésions digestives extra-coliques ou des tumeurs desmoïdes et le site de la mutation du gène APC.

Il semble donc légitime de proposer à ces malades une surveillance endoscopique haute et basse dont le rythme sera adapté à leurs lésions.

Summary

We studied 120 patients after colectomy for familial adenomatous polyposis (FAP), who underwent at least one upper digestive endoscopy to look for gastric, duodenal and papillary adenomas. A lower ileoscopy was also performed in 42 of these patients. Genotyping of the involved mutation was performed in 84 of these patients.

Globally, one or several digestive adenomas were observed in 54 patients (4.46 %). They were papillary duodenal adenomas in 39 cases, non-papillary duodenal adenomas in 43 cases, and gastric adenomas in 7 cases. Four papillary cancers were observed. Ileal adenomas were present in 20 patients (48 %). They consisted of tubulous adenomas in 15 cases and villous adenomas in 5 cases. The risk that these ileal adenomas degenerate is unknown. Their high frequency is in contrast with the relatively low frequency of ileal cancers in FAP.

No relationship was found between the importance of extra-colic digestive lesions or desmoid tumors and the location of the APC gene mutation.

It seems therefore legitimate to propose to these patients an upper and lower endoscopic surveillance, which frequency will be adapted to their lesions.

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Correspondence to N. Meary.

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Meary, N., Florent, C. Prévalence et suivi endoscopique des lésions digestives extra-coliques de la polyadénomatose familiale. Acta Endosc 27, 467–477 (1997). https://doi.org/10.1007/BF02963630

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Mots clés

  • lésions digestives extra-coliques
  • polyadénomatose familiale
  • suivi

Key-words

  • adenomatous polyposis
  • extra-colic digestive lesions
  • surveillance