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      Intussusception Caused by Peutz-Jeghers Syndrome

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          Abstract

          A 20-year-old female patient with a family history significant for Peutz-Jeghers syndrome presented to the hospital multiple times with complaints of abdominal pain. On the initial visit to the hospital, the patient underwent small bowel resection for small bowel obstruction secondary to intussusception, following which she visited the hospital again one year later for similar complaints and underwent reduction of multiple points of intussusception of the small bowel without any resection of the same. Eventually, the patient underwent resection of the small bowel for the second time, along with tumor resections. The importance of follow-up in patients with Peutz-Jeghers is particularly essential, in part, because it is vital to monitor the tumors, their size, and number to prevent surgical intestinal complications, anemia, and also to eventually monitor for carcinomatous changes. 

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          Very high risk of cancer in familial Peutz-Jeghers syndrome.

          F Giardiello, J Brensinger, A Tersmette … (2000)
          The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. We performed an individual patient meta-analysis to determine the relative risk (RR) of cancer in patients with PJS compared with the general population based on 210 individuals described in 6 publications. For patients with PJS, the RR for all cancers was 15.2 (95% confidence limits [CL], 2, 19). A statistically significant increase of RR was noted for esophagus (57; CL, 2.5, 557), stomach (213; CL, 96, 368), small intestine (520; CL, 220, 1306), colon (84; CL, 47, 137), pancreas (132; CL, 44, 261), lung (17.0; CL, 5.4, 39), breast (15.2; CL, 7.6, 27), uterus (16.0; CL, 1.9, 56), ovary (27; CL, 7.3, 68), but not testicular or cervical malignancies. Cumulative risk for all cancer was 93% from age 15 to 64 years old. Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers.
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            High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations.

            Ewout W. Steyerberg, Digna van Schaik, Anthony M Waas … (2010)
            Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder associated with increased cancer risk. Surveillance and patient management are, however, hampered by a wide range in cancer risk estimates. We therefore performed a systematic review to assess cancer risks in PJS patients and used these data to develop a surveillance recommendation. A systematic PubMed search was performed up to February 2009, and all original articles dealing with PJS patients with confirmed cancer diagnoses were included. Data involving cancer frequencies, mean ages at cancer diagnosis, relative risks (RRs), and cumulative risks were collected. Twenty-one original articles, 20 cohort studies, and one meta-analysis fulfilled the inclusion criteria. The cohort studies showed some overlap in the patient population and included a total of 1,644 patients; 349 of them developed 384 malignancies at an average age of 42 years. The most common malignancy was colorectal cancer, followed by breast, small bowel, gastric, and pancreatic cancers. The reported lifetime risk for any cancer varied between 37 and 93%, with RRs ranging from 9.9 to 18 in comparison with the general population. Age-related cumulative risks were given for any cancer and gastrointestinal, gynecological, colorectal, pancreatic, and lung cancers. PJS patients are markedly at risk for several malignancies, in particular gastrointestinal cancers and breast cancer. On the basis of these elevated risks, a surveillance recommendation is developed to detect malignancies in an early phase and to remove polyps that may be premalignant and may cause complications, so as to improve the outcome.
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              High cancer risk and increased mortality in patients with Peutz-Jeghers syndrome.

              Karen A. Westerman, Johannes van Beek, E Mathus-Vliegen … (2011)
              Peutz-Jeghers syndrome (PJS) is associated with an increased cancer risk. As the determination of optimal surveillance strategies is hampered by wide ranges in cancer risk estimates and lack of data on cancer-related mortality, we assessed cancer risks and mortality in a large cohort of patients with PJS. Dutch PJS patients were included in this cohort study. Patients were followed prospectively between January 1995 and July 2009, and clinical data from the period before 1995 were collected retrospectively. Data were obtained by interview and chart review. Cumulative cancer risks were calculated by Kaplan-Meier analysis and relative cancer and mortality risks by Poisson regression analysis. We included 133 PJS patients (48% males) from 54 families, contributing 5004 person-years of follow-up. 49 cancers were diagnosed in 42 patients (32%), including 25 gastrointestinal (GI) cancers. The median age at first cancer diagnosis was 45 years. The cumulative cancer risk was 20% at age 40 (GI cancer 12%), increasing to 76% at age 70 (GI cancer 51%). Cumulative cancer risks were higher for females than for males (p=0.005). The relative cancer risk was higher in PJS patients than in the general population (HR 8.96; 95% CI 6.46 to 12.42), and higher among female (HR 20.40; 95% CI 13.43 to 30.99) than among male patients (HR 4.76; 95% CI 2.82 to 8.04). 42 patients had died at a median age of 45 years, including 28 cancer-related deaths (67%). Mortality was increased in our cohort compared to the general population (HR 3.50; 95% CI 2.57 to 4.75). PJS patients carry high cancer risks, leading to increased mortality. The malignancies occur particularly in the GI tract and develop at young age. These results justify surveillance in order to detect malignancies in an early phase to improve outcome.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 3 April 2022
                Publication date (Electronic, collection): April 2022
                Volume: 14
                Issue: 4
                Electronic Location Identifier: e23792
                Affiliations
                [1 ] Internal Medicine, Mercy Catholic Medical Center, Darby, USA
                [2 ] Hematology/Oncology, Drexel University College of Medicine, Darby, USA
                [3 ] Physical Medicine and Rehabilitation, Mercy Catholic Medical Center, Darby, USA
                [4 ] Hematology/Oncology, Mercy Catholic Medical Center, Darby, USA
                Author notes
                Article
                DOI: 10.7759/cureus.23792
                PMC ID: 9067348
                PubMed ID: 35530863
                SO-VID: 6826b065-5b51-45e0-bd86-7ca35f28e031
                Copyright © Copyright © 2022, Sreemantula et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date accepted : 3 April 2022
                Categories
                Subject: Internal Medicine
                Subject: General Surgery
                Subject: Oncology

                Keywords: anemia,endoscopic screening,small bowel obstruction,intestinal polyps,small bowel resection,peutz jeghers,recurrent intussusception
                Data availability:
                Keywords: anemia, endoscopic screening, small bowel obstruction, intestinal polyps, small bowel resection, peutz jeghers, recurrent intussusception

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