Peutz-Jeghers syndrome in children: Report of two cases and review of the literature

Tovar, Juan A.; I, Eizaguirre; Albert, Alexander; J, Jiménez Alonso

doi:10.1016/s0022-3468(83)80262-0

Cited by 78 publications

(37 citation statements)

References 36 publications

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“…Tover et al 2 reported a series of 70 pediatric patients who presented with intestinal obstruction in 71.4%, melena in 18.5%, and prolapse of rectal polyps in 7.1%. Intestinal obstruction is often caused by intussusception because of the presence of intestinal polyps, as described here in our patient.…”

Section: Discussionmentioning

confidence: 99%

Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome

Wada¹,

Asoh²,

Imamura³

et al. 1998

Journal of Gastroenterology

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A 16-year-old man who had been diagnosed with the Peutz-Jeghers syndrome at the age of 8 years presented with crampy abdominal pain. Thorough examinations revealed a large jejunal polyp causing intussusception, as well as multiple polyps in the small and large intestines. Preoperative proctoscopy demonstrated the coexistence of a submucosal tumor in the rectum. Proctoscopic mucosal resection was performed and histological and immunohistochemical examinations led to a diagnosis of carcinoid tumor. Additional transanal resection of the rectal wall showed no residual tumor and the patient has been well for 2 years to date. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the English-language and Japanese literature.

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Section: Discussionmentioning

confidence: 99%

Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome

Wada¹,

Asoh²,

Imamura³

et al. 1998

Journal of Gastroenterology

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“…13 Abdominal symptoms tend to occur early in life, with more than 50% symptomatic patients before the age of 20. 9,14 Although it has been found that there is a high cancer risk in PJS patients according to John Hopkin's hospital polyposis registry 6,no cancer was found in ourpatient's polyps.…”

mentioning

confidence: 66%

PeutzJeghers syndrome: A case report and literature review

Lakhey

Shakya

2014

J Pathol Nep

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Peutz-Jeghers Syndrome is an autosomal dominant inheritedhamartomatous polyp. We present a case of a 5-year-old young boywith a history of per rectal bleeding and mass protruding out of the anus. Physical examination revealed presence of mucocutaneous pigmented lesions over the tongue, and few hamartomatous polyps protruding out of the rectum suggesting Peutz-Jeghers syndrome. The presence in early infancy of small, well-demarcated and dark-brown to blue-black lentigines on the lips,buccal mucosa and perioral skin, should alert the clinician to Peutz-Jeghers Syndrome.

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“…Since then, many other reports of this disorder have appeared (2,3). Patients with the P-J syndrome are at increased risk for the development of intestinal and extraintestinal malignancies (4,(7)(8)(9). Intestinal carcinoma of the P-J syndrome develop from hamartomatous polyps through adenomatous transformations (10).…”

Section: Discussionmentioning

confidence: 99%

“…The majority of cancers did not occur in the gastointestinal lumen but in organs in which hamartomatous polyps were not found. Previous reports have described carcinomas of the tonsils (ll), lung (4, 9), bile duct (ll), gallbladder (12, 13), pancreas (4, 7, 9, 14, 15), breast (8,(15)(16)(17)(18), ovary (4) and cervix (19-24) in patients with P-J syndrome, suggesting that there is a generalized endodermal epithelial growth disturbance in the patients with P-J syndrome, who appear to be predisposed to develop malignancies.…”

Section: Discussionmentioning

confidence: 99%

Thyroid Papillary Adenocarcinoma in a Woman with Peutz-Jeghers Syndrome.

et al. 1992

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We present a rare case of Peutz-Jeghers(P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be the first precise report of a P-J syndrome associated with thyroid carcinoma. This case adds an example of predisposition of P-J syndrome to extra intestinal malignancy and indicates the importance of long-term observation of malignancy.

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Peutz-Jeghers syndrome in children: Report of two cases and review of the literature

Cited by 78 publications

References 36 publications

Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome

Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome

PeutzJeghers syndrome: A case report and literature review

Thyroid Papillary Adenocarcinoma in a Woman with Peutz-Jeghers Syndrome.

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