Prognosis

The major life-threatening complications to individuals with PJS are cancer and small bowel obstruction. Two small studies showed that the age of death is earlier in patients with PJS than their unaffected family members, and was attributable to obstruction or cancer.[47][48]

It is clear that small bowel obstruction is one of the leading causes of surgery and can be minimised by clearance of polyps within the small bowel.[35][36][37]

Patients with PJS are reported to have a significantly increased lifetime risk of developing intestinal and extra-intestinal malignancies compared with the general population; the cumulative lifetime risk has been reported at 93%.[1] The median age of onset of cancer is reported to be in the fourth and fifth decade of life. Lifetime risks for specific cancers include colorectal (39%), gastric (29%), small bowel (13%), breast (40% to 85%), pancreas (11% to 36%), ovary (21%), lung (7% to 17%), cervical (10% to 23%), and uterus (9%).[14] Male children and adolescents, and, less commonly, younger adults are also at risk for testicular cancer.[16] However, the evidence to support the data on cancer incidence, and, in particular, mortality, is extremely limited, being derived from a small number of observed individuals and families.[16] It is unknown whether implementation of surveillance for the early detection or prevention of cancer is effective, although it is recommended.[14][19][20][33]​​

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