Unusually Early Presentation of Small-Bowel Adenocarcinoma in a Patient With Peutz-Jeghers Syndrome

Wangler, Michael F.; Chavan, Rishikesh; Hicks, M. John; Nuchtern, Jed G.; Hegde, Madhuri; Plon, Sharon E.; Thompson, Patrick A.

doi:10.1097/mph.0b013e318282db11

Cited by 15 publications

(8 citation statements)

References 19 publications

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“…The risk of developing GI cancers at ages 30, 40, 50 and 60 years is 1%, 9%, 15% and 33%, respectively 2. Paediatric patients with PJS present with earlier onset of GI-related complications like a prolapsed polyp or intussusception and rarely with small bowel carcinoma 5–9. Small bowel adenocarcinoma in children as such is a very rare disease 10.…”

Section: Discussionmentioning

confidence: 99%

Peutz-Jeghers syndrome: need for early screening

Khanna¹,

Khanna

Bhatnagar

2018

BMJ Case Rep

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Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.

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Section: Discussionmentioning

confidence: 99%

Peutz-Jeghers syndrome: need for early screening

Khanna¹,

Khanna

Bhatnagar

2018

BMJ Case Rep

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“…There is also an increased lifetime risk of malignancy in the breast (54 %), ovaries (21 %), uterus (9 %), and lung (15 %). Small intestine adenocarcinoma has been detected in a child as young as 13 years of age [7], with patients in the third decade having a cumulative cancer risk of 5 % which rises to 85 % by the age of 70 years.…”

Section: Discussionmentioning

confidence: 99%

Peutz-Jeghers Syndrome with Synchronous Adenocarcinoma Arising from Ileal Polyps

2014

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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 PeutzJeghers 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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“…The incidence of tumors in patients with Peutz-Jeghers syndrome patients is 15 times higher than that observed in normal individuals, and the incidence of malig nant tumors may be as high as 20% (8). Malignancy in Peutz-Jeghers syndrome is considered to arise in adulthood, and occurs only rarely in children (9). In addition to the hamartomas, malignant tumors of other organs outside the gastroin testinal tract also evolve into adenomas and carcinomas in a process occurring via de novo pathways (10).…”

Section: Discussionmentioning

confidence: 99%

Acute intussusception and polyp with malignant transformation in Peutz-Jeghers syndrome: A case report

Jiang

2015

Oncology Letters

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Abstract. Intussusception is one of the most frequent complications of Peutz-Jeghers syndrome, and has been well described in previous studies. More attention has been paid to malignancy, which is another complication of Peutz-Jeghers syndrome and which leads to increased mortality. Few cases of intussusception combined with malignant polyps in Peutz-Jeghers syndrome have been reported to date. In the present study, we report a case of intussusception and malignant polyps occurring in various parts of the small intestine in a 43-year-old male. In addition to repair of the intussusception and partial resection of the small intestine with malignant polyps, we also simultaneously performed polypectomy of as many polyps as possible without resection of the small intestine. Our aim is to make clinicians aware of intussusception and malignant polyps coexisting in Peutz-Jeghers syndrome when performing emergency surgery. Prophylaxis and polypectomy of the entire small bowel is an effective way to reduce the frequency of laparotomies in patients with this disease.

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Unusually Early Presentation of Small-Bowel Adenocarcinoma in a Patient With Peutz-Jeghers Syndrome

Cited by 15 publications

References 19 publications

Peutz-Jeghers syndrome: need for early screening

Peutz-Jeghers syndrome: need for early screening

Peutz-Jeghers Syndrome with Synchronous Adenocarcinoma Arising from Ileal Polyps

Acute intussusception and polyp with malignant transformation in Peutz-Jeghers syndrome: A case report

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