Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature

Cai, Huai-Jie; Wang, Han; Cao, Nan; Wang, Wei; Sun, Xiaopeng; Huang, Bin

doi:10.12998/wjcc.v8.i3.577

Cited by 5 publications

(4 citation statements)

References 37 publications

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“…Peutz-Jeghers syndrome was first reported by Peutz in 1921 and described in 1949 by Jeghers. [4] The etiology of Peutz-Jeghers syndrome remains unknown. The clinical symptoms are non-specific such as anemia, nausea, abdominal pain, and intestinal intussusception.…”

Section: Discussionmentioning

confidence: 99%

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A rare case of a juvenile polyp of patient with Peutz-Jeghers syndrome, complicated with intussusception of the small intestine

Rycyk¹,

Kasztelan-Szczerbińska²,

Cichoż‐Lach³

2022

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We report a rare case of Peutz-Jeghers syndrome (PJS) in a 35-year-old female. The patient was diagnosed with PJS when she was 11 years old. She has remained under observation since then. We strongly believe that PJS is a very rare diagnosis. However, it can have serious complications such as the intussusception we observed in our patient. Her condition (recurrent abdominal pain and vomiting) in childhood required further diagnostic procedures. Although the diagnosis of PJS was made, among many resected polyps, one of them appeared to be a juvenile polyp. The diagnosis was confirmed in the histopathology report, which was incredibly unique. Genetic testing revealed LKB1/STK11 gene mutation. Clinicians should be aware of the malignant potential in the course of PJS. Hence, these patients require tailor-made management, long-term follow-up, and our particular attention.

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Section: Discussionmentioning

confidence: 99%

“…Next, after 7 months, 30 polyps were found in the stomach, one of which was removed. During the next years, 23,18,12,11,14,4, and 4 gastrointestinal polyps were removed in iterative colonoscopy procedures, consecutively.…”

Section: Intussusception Of Small Intestinementioning

confidence: 99%

A rare case of a juvenile polyp of patient with Peutz-Jeghers syndrome, complicated with intussusception of the small intestine

Rycyk¹,

Kasztelan-Szczerbińska²,

Cichoż‐Lach³

2022

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“…Peutz-Jeghers syndrome (PJS). 30 It is a rare autosomal dominant genetic disease characterized by mucocutaneous pigmentation and multiple polyps in the gastrointestinal tract. PJS can be classed as one of various hereditary intestinal polyposis syndromes and one of various hamartomatous polyposis syndromes.…”

Section: Continued On Next Pagementioning

confidence: 99%

Eponyms in digestive system pathology

Malki¹,

Solami²,

Aboud³

et al. 2020

PJMS

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“…Peutz-Jeghers syndrome (PJS) is an uncommon autosomal dominant genetic disease, included in the familiar syndromes of polyps, such as Cowden and juvenile polyposis [ 1 ]. It is a rare disease (1/20,000), described first in 1921 (Peutz) and 1949 (Jeghers) [ 2 , 3 ]. The majority of the cases are diagnosed between 12 and 30 years, appearing mostly in males [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Minimally Invasive Elective Surgery as a Treatment of Bowel Invagination in a Peutz-Jeghers Syndrome Case

Picazo¹,

Torre²,

Vivas³

et al. 2021

Case Rep Gastroenterol

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We present a case of a 24-year-old woman with Peutz-Jeghers syndrome, recurrent colic abdominal pain, and lower gastrointestinal bleed for the last 5 years. Colonoscopy showed hamartomas without any dysplasia. In the enteral magnetic resonance imaging, a distal jejunum and ileum invagination, secondary to hamartomas was detected. The patient was referred to the Surgery Department and despite few symptoms, elective surgery was proposed. By laparoscopic surgery approach, the entire bowel was carefully revised, 3 intussusceptions and bowel volvulus were found, 2 in jejunum and 1 in ileum, causing incomplete obstruction and intestinal dilatation, with a diameter of 6 cm. These intussusception areas were marked with a silk filament, after achieving devolvulation and disinvagination. A 5-cm laparotomy was done, to externalize the entire bowel, to explore it manually, to verify the absence of other lesions, and locate silk points. By longitudinal enterotomies on the antimesenteric intestinal border where silk filaments were located, the polyps were removed through their stalk, and the enterotomies were transversely closed. Postoperative evolution was favorable, starting oral tolerance on the fourth day and being discharged from the hospital on the seventh day. Eight months later, the patient was asymptomatic with a better quality of life.

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Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature

Cited by 5 publications

References 37 publications

A rare case of a juvenile polyp of patient with Peutz-Jeghers syndrome, complicated with intussusception of the small intestine

A rare case of a juvenile polyp of patient with Peutz-Jeghers syndrome, complicated with intussusception of the small intestine

Eponyms in digestive system pathology

Minimally Invasive Elective Surgery as a Treatment of Bowel Invagination in a Peutz-Jeghers Syndrome Case

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