Very high risk of cancer in familial Peutz-Jeghers syndrome
Autor: | Susan V. Booker, Steven N. Goodman, Jill D. Brensinger, Francis M. Giardiello, Johan Offerhaus, Marcia Cruz-Correa, Gloria M. Petersen, Anne C. Tersmette |
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Přispěvatelé: | Other departments |
Jazyk: | angličtina |
Rok vydání: | 2000 |
Předmět: |
Adult
Male medicine.medical_specialty Pathology Lung Neoplasms Adolescent Genital Neoplasms Female Peutz-Jeghers Syndrome Peutz–Jeghers syndrome Digestive System Neoplasms Gastroenterology Risk Factors Internal medicine Neoplasms medicine Carcinoma Humans Juvenile polyposis syndrome Esophagus Hepatology business.industry Stomach Absolute risk reduction Cancer Middle Aged medicine.disease medicine.anatomical_structure Relative risk Female business |
Zdroj: | Gastroenterology, 119(6), 1447-1453. W.B. Saunders Ltd |
ISSN: | 0016-5085 |
Popis: | Background & Aims: The Peutz–Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. Methods: 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. Results: 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. Conclusions: Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers. GASTROENTEROLOGY 2000;119:1447-1453 |
Databáze: | OpenAIRE |
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