A case of Gardner's syndrome

Gardner's syndrome is a hereditary disease characterized by the triad of soft tissue tumor, hard tumor (osteoma) and multiple intestinal polyposis and is also accompanied by a variety of dental abnormalities.It has been reported that early diagnosis is essential as intestinal polyposis in parti...

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Published inJapanese Journal of Oral and Maxillofacial Surgery Vol. 33; no. 1; pp. 179 - 183
Main Authors SATOH, Kohji, ABE, Asako, OKUI, Mie, TSUJIKAWA, Takaaki, HIBI, Goroh, OKA, Tohru
Format Journal Article
LanguageEnglish
Published Japanese Society of Oral and Maxillofacial Surgeons 1987
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Abstract Gardner's syndrome is a hereditary disease characterized by the triad of soft tissue tumor, hard tumor (osteoma) and multiple intestinal polyposis and is also accompanied by a variety of dental abnormalities.It has been reported that early diagnosis is essential as intestinal polyposis in particular, has a high malignant pontential.In this paper, a case study is reported on a patient with complete Gardner's syndrome discovered on presentation for examination at our Department of Oral Surgery and a medical documentation is also included. The patient was a thirty-five years old male who presented for initial diagnosis with a hard tumor in both mandibular angles and a bean-sized atheroma-like tumor in theepithelium of the right oral angle. X-ray findings revealed osteoma-like radiopaque in the maxilla and mandibular as well as several impacted teeth.In addition, the patient had a history of tumor resection in the dorsal, right orbital epithelium. Based on the above findings, Gardner's syndrome was suspected and gastrointestinal surgery was subsequently conducted.Surgical findings revealed multiple polyps occurring in the region from the stomach through the small intestine to the large intestine and complete Gardner's syndrome was diagnosed.The largest polyp found in resected intestine was 5 cm in diameter, and although it was found to be pathohistologically adenoma, malignant findings were not observed.
AbstractList Gardner's syndrome is a hereditary disease characterized by the triad of soft tissue tumor, hard tumor (osteoma) and multiple intestinal polyposis and is also accompanied by a variety of dental abnormalities.It has been reported that early diagnosis is essential as intestinal polyposis in particular, has a high malignant pontential.In this paper, a case study is reported on a patient with complete Gardner's syndrome discovered on presentation for examination at our Department of Oral Surgery and a medical documentation is also included. The patient was a thirty-five years old male who presented for initial diagnosis with a hard tumor in both mandibular angles and a bean-sized atheroma-like tumor in theepithelium of the right oral angle. X-ray findings revealed osteoma-like radiopaque in the maxilla and mandibular as well as several impacted teeth.In addition, the patient had a history of tumor resection in the dorsal, right orbital epithelium. Based on the above findings, Gardner's syndrome was suspected and gastrointestinal surgery was subsequently conducted.Surgical findings revealed multiple polyps occurring in the region from the stomach through the small intestine to the large intestine and complete Gardner's syndrome was diagnosed.The largest polyp found in resected intestine was 5 cm in diameter, and although it was found to be pathohistologically adenoma, malignant findings were not observed.
Author OKUI, Mie
TSUJIKAWA, Takaaki
HIBI, Goroh
SATOH, Kohji
ABE, Asako
OKA, Tohru
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  fullname: OKA, Tohru
  organization: Department of Oral Surgery, School of Medicine, Fujita-Gakuen Health University
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References 16) Leppard, B. and Bussey, H. J. R.: Epidermoid cysts, polyposis coli and Gardner's syndrome. Brit J Surg 62: 387-393 1975.
2) 大塚寿, 藤田浄秀, 他: Gardner症候群にみられた歯牙および口腔の異常について. 日口外誌20: 20-27 1974.
14) Parks, T. G., Bussey, H. J. R., et al.: Familial polyposis coli associated with extracolonic abnormalities.Gut 11: 323-329 1970.
1) 豊嶋昭治, 池潤, 他: Gardner'syndrome を思わせる2症例. 日口外誌16: 54-59 1970.
9) 宇都宮譲二: 遺伝性消化管ポリポーシス. 日本医事新報No.3044: 126 1982.
4) Gardner, E. J. and Plenk, H. P.: Hereditary pattern for multiple osteomas in a family group. Am J Human Genet 4: 31-36 1952.
15) Gardner, E. J.: Follow-up study of a family group exhibiting dominant inheritance for a syndrome including intestinal polyps, osteomas, fibromas and edpidermal cysts. Am J Human Genet 14: 376-390 1962.
12) Fader, M., Kline, S. N., et al.: Gardner's syndrome and a new dental discovery. Oral Surg 15: 153-172 1962.
3) Gardner, E. J.: A genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum. Am J Human Genet 3: 167-176 1951.
6) 宇都宮譲二, 鈴木宏文, 他: Gardner症候群癌の臨床18: 79-100 1972.
10) Utsunomiya, J., Iwama, T., et al.: Clinical and population genetics of the hereditary gastrointestinal polyposes. In the genetics and heterogeneity of common gastrointestinal disorders. Academic Press: 395-415 1980.
11) 大塚寿, 上石弘, 他: Gardner症候群の1例. 形成外科16: 240-250 1973.
8) Utsunomiya, J. and Nakamura, T.: The occult osteomatous changes in the mandible in patients with familial polyposis coli. Br J Surg 62: 45-51 1975.
13) 大槻寿朗, 香月武, 他: 家族性大腸腺腫症に伴う歯牙顎骨の異常所見. 癌の症例27: 1617-1621 1981.
5) Gardner, E. J. and Richards, R. C.: Multiple cutaneous and subcutaneous lesions occuring simultaneously with hereditary polyposis and osteomatosis. Am J Human Genet 5: 139-147 1953.
7) 宇都宮譲二, 岩間毅夫, 他: 大腸ポリポージスの分類. 外科診療17: 235-246 1975.
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Snippet Gardner's syndrome is a hereditary disease characterized by the triad of soft tissue tumor, hard tumor (osteoma) and multiple intestinal polyposis and is also...
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SubjectTerms familial polyposis coli
Gardner's syndrome
osteoma
Title A case of Gardner's syndrome
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