ArticleFrontiers in oncology2022
High risk and early onset of cancer in Chinese patients with Peutz-Jeghers syndrome.
Article in Frontiers in oncology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Closing the Diagnostic Gap in Early-Onset Colorectal Cancer: Red-Flag Symptoms, Screening, and Inherited Risk.Cancers · 2026Review
- Peutz-Jeghers Syndrome and lung cancer: does the risk meet the threshold for lung cancer screening?Familial cancer · 2026Review
- Neurofibromatosis type 1 with concomitant Peutz-Jeghers syndrome in a child: a case report.BMC pediatrics · 2026Article
- Natural History of Rare Tumor Risk Syndromes: A Scoping Review to Inform Cost-Effectiveness Models.Journal of health economics and outcomes research · 2026Article
- Risk Assessment and Fertility Counseling for Hereditary Gynecological Cancer Syndromes.Cancer medicine · 2025Review
- Rare large sigmoid hamartomatous polyp in an elderly patient with atypical Peutz-Jeghers syndrome: A case report.World journal of gastrointestinal surgery · 2025Article
- Diagnosis of Peutz-Jeghers syndrome with pulmonary chondromatous hamartoma in a 21-year-old man: a case report.Gastroenterology report · 2025Article
- MRI grading for informed clinical decision-making in Peutz-Jeghers syndrome patients with cervical lesions.Scientific reports · 2024Article
- Poorly differentiated adenocarcinoma of the jejunum in a patient with Peutz-Jeghers syndrome: A case report.International journal of surgery case reports · 2024Article
- Cancer Risk of Peutz-Jeghers Syndrome and Treatment Experience: A Chinese Medical Center.Clinics in colon and rectal surgery · 2023Review
- The Progress of Colorectal Polyposis Syndrome in Chinese Population.Clinics in colon and rectal surgery · 2023Review
- Clinical features, diagnosis, and treatment of Peutz-Jeghers syndrome: Experience with 566 Chinese cases.World journal of gastroenterology · 2023Article
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9 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder associated with a predisposition to a variety of cancers. Previous studies that have evaluated the cancer spectrum and risk of this rare disease have primarily been based on small data sets or heterogeneous cohorts from different countries. Here, we report the results of a large homogeneous cohort of Chinese PJS patients who were followed prospectively from 2006 to July 2021, and clinical data before 2006 were retrospectively collected. A total of 412 PJS patients (56.55% males) from 208 families were enrolled, contributing 12,798 person-years of follow-up. A total of 113 cancers were diagnosed in 109 patients (26.46%). The median age at the first cancer diagnosis was 40 years. In particular, patients born after the 1980s were diagnosed with cancer at an earlier median age of 30.5 years. The cumulative cancer risk was sharply increased to 30.9% at age 40 years; this high cancer risk age was 10 years earlier than that reported in previous Western studies, and increased to 76.2% at an age of 60 years. The most common cancer was gastrointestinal (GI) cancer (64.6%), in which colorectal cancer constituted a significantly larger proportional distribution (32.74%), when compared with previous investigations (11.1%-20.83%). There was some evidence that overrepresentation point variants in domain XI of
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