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January 1969

Peutz-Jeghers Syndrome With Metastases to an Abdominal Incision

Author Affiliations

Madison, Wis
From the Division of Clinical Oncology and the Department of Surgery (Dr. Mackman), the Health Service and the Department of Medicine (Dr. Perna), and the Department of Pathology (Dr. Gossett), University of Wisconsin Medical Center, Madison.

Arch Surg. 1969;98(1):99-102. doi:10.1001/archsurg.1969.01340070117026

Peutz-Jeghers syndrome consisting of the triad-gastrointestinal-polyps, mucocutaneous pigmentation, and familial history is a well recognized clinical entity.1,2 The polyps which are generally in the small intestine but which may be found throughout the gastrointestinal tract, are peculiar in that they contain all types of epithelial cells found in the gut arranged in the usual manner on a branching muscular stroma, and are thus felt to represent hamartomatas.3,4 Because of this unusual histological appearance, they have often been misinterpreted as invading muscularis and are thus considered by some as being malignant in 15% to 20% of cases.1 In reality, only six of the more than 300 cases reported have the polyps shown definite malignancy with metastases.5 The following case records the unusual occurrence of a metastases to the abdominal wall in a patient with Peutz-Jeghers syndrome.

Report of a Case  This 22-year-old white man was first seen

Berkowitz, S.B.; Pearl, M.J.; and Shapiro, N.H.:  Syndrome of Intestinal Polyposis with Melanosis of the Lips and Buccal Mucosa: A Study of the Incidence and Location of Malignancy , Ann Surg 141:129-133 ( (Jan) ) 1955.Article
Warren, K.W.; Kune, G.A.; and Poulantzas, J.K.:  Peutz-Jeghers Syndrome with Carcinoma of the Duodenum , Lahey Clin Found Bull 14:97-102, 1965.
Bartholomew, L.G.; Dahlin, D.C.; and Waugh, J.M.:  Intestinal Polyposis Associated with Mucocutaneous Melanin Pigmentation (Peutz-Jeghers Syndrome) , Gastroenterology 32:434-451 ( (March) ) 1957.
Wenzl, J.E., et al:  Gastrointestinal Polyposis with Mucocutaneous Pigmentation in Children (Peutz-Jeghers Syndrome) , Pediatrics 28:655-661 ( (Oct) ) 1961.
Reid, J.D.:  Duodenal Carcinoma in the Peutz-Jeghers Syndrome , Cancer 18:970-977 ( (Aug) ) 1965.Article
Berkowitz, S.B.; Pearl, M.J.; and Shapiro, N.H.:  Syndrome of Intestinal Polyposis , Ann Surg 141:129-133 ( (Jan) ) 1955.Article
Burdick, D.; Prior, J.T.; and Scanlon, G.T.:  Peutz-Jeghers Syndrome: A Clinical-Pathological Study of a Large Family with a 10-year Follow-up , Cancer 16:854-867 ( (July) ) 1963.Article
Humphries, A.L.; Shepherd, M.A.; and Peters, H.J.:  Peutz-Jeghers Syndrome With Colonic Adenocarcinoma and Ovarian Tumor , JAMA 197:296-298 ( (July 25) ) 1966.Article
McKusick, V.A.:  Genetic Factors in Intestinal Polyposis , JAMA 182:271-277 ( (Oct 20) ) 1962.Article
Warren, K.W.; Kune, G.A.; and Poulantzas, J.K.:  Peutz-Jeghers Syndrome With Carcinoma of the Duodenum and Jejunum , Lahey Clin Found Bull 14:97-102, 1965.
Williams, J.P., and Knudsen, A.:  Peutz-Jeghers Syndrome With Metastasizing Duodenal Carcinoma , GUT 6:179-184 ( (April) ) 1965.Article