[Gardner syndrome. A report of 3 cases in members of the same family].
The aim of the present report is to describe the overall mandibular and extramandibular lesions correlated to Gardner's syndrome which were found in the same family (mother and two daughters). In two cases (mother and one daughter) mandibular lesions consisted of osteoma. In the latter, these were associated with composite odontoma. In the other daughter the lesions consisted of desmoid fibroma. The two daughters revealed single colonic polyposis whereas the mother was affected by multiple colonic polyposis and subcutaneous lipoma. Such a constellation of lesions may be attributed to Gardner's syndrome, a Mendelian hereditary syndrome which is often incomplete. In fact, intestinal polyposis can only be associated with one of the various extraintestinal manifestations of the syndrome. The recognition of the syndrome is important given the high percentage of intestinal polyp-adenocarcinoma associations.
Bertoni
F
F
Servizio di Anatomia ed Isotologia Patologica, Ospedale Provinciale Specializzato M. Malpighi, Bologna.
Bacchini
P
P
Marchetti
C
C
Fagioli
S
S
Campobassi
A
A
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D003113:Colonoscopy; D005260:Female; D005350:Fibroma / Q000175:diagnosis / Q000235:genetics; D005736:Gardner Syndrome / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D008334:Mandible / Q000000981:diagnostic imaging / Q000473:pathology; D008339:Mandibular Neoplasms / Q000175:diagnosis / Q000235:genetics; D008875:Middle Aged; D009810:Odontoma / Q000175:diagnosis / Q000235:genetics; D010016:Osteoma / Q000175:diagnosis / Q000235:genetics; D011862:Radiography, Panoramic
Sindrome di Gardner. Descrizione di tre casi componenti una stessa famiglia.