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The Peutz-Jeghers Syndrom – A Case Review


Authors: M. Škrovina 1;  S. Czudek 1;  J. Bartoš 1;  I. Ferák 2;  L. Adamčík 1;  E. Bezunková 3;  R. Vaňko 1
Authors place of work: Chirurgické oddělení, Onkocentrum J. G. Mendela a Nemocnice Nový Jičín primář: MUDr. S. Czudek, CSc. 1;  Patologie, P & R LAB s. r. o., Onkocentrum J. G. Mendela Nový Jičín, primář: MUDr. I. Ferák, Ph. D. 2;  Radiodiagnostické oddělení, Onkocentrum J. G. Mendela a Nemocnice Nový Jičín primář: MUDr. M. Velkoborský 3
Published in the journal: Rozhl. Chir., 2007, roč. 86, č. 1, s. 24-26.
Category: Monothematic special - Original

Summary

Introduction:
The authors present a case review of a localized Peutz-Jeghers syndrome of the caecum, emulating a carcinoma.

Methods:
The patient who presented with insignificant family history with a diagnosed stenosing tumor of his appendix and histological findings of highly suspected adenocarcinoma, was indicated for surgical revision and right-sided hemicolectomy.

Results:
The patient underwent laparoscopically assissted right-sided hemicolectomy and his postoperative course was adequate. The final histological finding diagnosed solitary hamartoma of the Peutz-Jeghers polyp.

Conclusion:
Differential diagnostic reasoning in patients with negative family history and unclear case history with findings of polypous tumorous lesions of the GIT, should also consider the Peutz-Jeghers syndrome. The syndrome is related to a high risk of malignancies in the elderly.

Key words:
Peutz-Jeghers syndrome – caecum – laparoscopic resection


Zdroje

1. Peutz, J. L. A. Over een 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. Ned. Maandschr. Geneeskd., 1921; 10: s. 134–146.

2. Jeghers, H, McKusick, K. V., Katz, K. H. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits; a syndróme of diagnostic signifícance. N. Engl. J. Med., 1949; 241: 1031–1036.

3. Jenne, D. E., Reimann, H., Nezu, J., Friedel, W., Loff, S., Jeschke, R., Muller, O., Back, W., Zimmer, M. Peutz-Jeghers syndróme is caused by mutations in a novel serine threonine. Nat. Genet., 1998; 18 (1): s. 38–43.

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10. Jones, D. V. Jr., Skibber, J., Levin, B. Adenocarcinoma and Other Small Intestinal Neoplasms, Including Benign Tumors. In: Feldman, M., Scharschmidt, B. F., Sleisenger, M. H. Gastrointestinal and Liver Disease. 6th Edition, Volume 2, W. B. Saunders Company, Philadelphia, 1998, s. 1894–1895, ISBN 0-7216-6293-5.

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12. Giardiello, F. M., Welsh, S. B., Hamilton, S. R., Offerhaus, G. J., Gittelsohn, A. M., Booker, S.V., et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N. Engl. J. Med., 1987; 316 (24): s. 1511–1514.

13. De Facq, L., DeSutter, J., et al. A case of Peutz-Jeghers syndrome with nasal polyposis, extréme iron deficiency anémia and hamartoma-adenomy deficiency anemia and hamartoma-adenoma transformation: management by combined surgical and endoscopical approach. Am. J. Gastroenterol., 1995, 90(8): s. 1330–1332.

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16. Barussaud, M., Regenet, N., Brienon, X., de Kerviler, B., Pessaux, P., Kohneh-Sharhi, N., Lehur, P.A., Hamy, A., Leborgne, J., le Neel, J.C, Mirallie, E. Clinical spectrum and surgical approach of adult intussusceptions: a multicentric study. Int. J. Colorectal Dis., 2005, 20(6).

17. Pedersen, I. R., Hartvigsen, A., Hansen, B., et al. Management of Peutz-Jeghers syndrome: Experience with patients from the Danish Polyposis Registry. Int. J. Colorectal Dis., 1994, 9: s. 177.

Štítky
Surgery Orthopaedics Trauma surgery

Článok vyšiel v časopise

Perspectives in Surgery

Číslo 1

2007 Číslo 1
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