Abstract
Peutz-Jeghers syndrome is a rare inherited autosomal disease characterized by mucocutaneous pigmentation and multiple polyps in the gastrointestinal tract. The clinical picture is characterized by repeated episodes of polyp-induced intestinal obstruction, abdominal pain, and bleeding per rectum. Predisposition to both gastrointestinal and nongastrointestinal malignancies is increased in a patient with Peutz-Jeghers syndrome. This is a case report of a 29-year-old male with Peutz-Jeghers syndrome who presented with a complaint of recurrent abdominal pain. CT scan revealed a single obstructing ileal polyp. However, preoperatively, another large ileal polyp with multiple small jejunal polyps was seen. Histopathology of both ileal polyps was identified as a mucinous adenocarcinoma infiltrating up to the serosa. The follow-up endoscopies showed small multiple polyps in the stomach, duodenum, and colon. Histopathology of all endoscopically removed polyps was consistent with the diagnosis of hamartomatous polyps. Very few cases of intestinal intussusception combined with synchronous malignant small intestine polyps have been reported until to date.
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McGarrity TJ, Amos C (2006) Peutz-Jeghers syndrome: clinicopathology and molecular altercation. Cell Mol Life Sci 63:2135–44
Niimi K, Tomoda H, Furusawa M et al (1991) Peutz-Jeghers syndrome associated with adenocarcinoma of the caecum and focal carcinoma in hamartomatous polyps of the colon: a case report. Jpn J Surg 21:220–3
Gill SS, Heuman DM, Mihas AA (2001) Small intestinal neoplasms. J Clin Gastroenterol 33:267–82
Lindor NM, Greene MH (1998) The concise handbook of family cancer syndromes Mayo Familial Cancer Program. J Natl Cancer Inst 90:1039–71
Giardiello FM, Trimbath JD (2006) Peutz-Jeghers syndrome and management recommendations. Clin Gastroenterol Hepatol 4:408–15
Giardiello FM, Brensinger JD, Tersmette AC, Goodman SN, Petersen GM, Booker SV (2000) Very high risk of cancer on familial Peutz-Jeghers syndrome. Gastroenterology 119:1447–53
Wangler MF, Chavan R, Hicks MJ et al (2013) Unusually early presentation of small-bowel adenocarcinoma in a patient with Peutz-Jeghers syndrome. J Pediatr Hematol Oncol May 35(4):323–8
Eren T, Bayraktar B, Celik Y, Boluk S, Adali G (2012) Acute malignant intestinal obstruction accompanied by synchronous multifocal intestinal cancer in Peutz-Jeghers syndrome: report of a case. Surg Today 42(11):1125–9
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Sharma, M., Singh, R. & Grover, A.S. Peutz-Jeghers Syndrome with Synchronous Adenocarcinoma Arising from Ileal Polyps. Indian J Surg 77 (Suppl 1), 100–102 (2015). https://doi.org/10.1007/s12262-014-1178-3
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DOI: https://doi.org/10.1007/s12262-014-1178-3