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Peutz-Jeghers syndrome is rare and studies typically include only a small number of patients.
Follow-up care should be supervised by a physician familiar with Peutz-Jeghers syndrome.
These are rare tumours that may be sporadic or associated with Peutz-Jeghers syndrome.
These tumours may be seen in the context of Peutz-Jeghers syndrome or be sporadic.
Probably due to this limited evidence base, cancer risk estimates for Peutz-Jeghers syndrome vary from study to study.
The condition is unrelated to true melanoses, such as Peutz-Jeghers syndrome and smoker's melanosis.
STK11: Mutations produce Peutz-Jeghers syndrome.
Peutz-Jeghers syndrome - an autosomal dominant disorder characterized by hyperpigmented macules on the lips and oral mucosa and gastrointestinal polyps.
Peutz-Jeghers syndrome: STK11/LKB1 gene.
Giardiello FM, Trimbath JD: Peutz-Jeghers syndrome and management recommendations.
Other conditions consisting of multiple hamartomatous polyps of the digestive tract include Peutz-Jeghers syndrome, juvenile polyposis, and Cowden disease.
He has additionally identified genes implicated in the development of skin, testis, colorectal and thyroid cancers, Wilms tumour and Peutz-Jeghers syndrome.
McGrath DR, Spigelman AD: Preventive measures in Peutz-Jeghers syndrome.
The disease belongs to a family of hamartomatous polyposis syndromes, which also includes Peutz-Jeghers syndrome, juvenile polyposis and Cowden syndrome.
STK11: Serine/threonine kinase 11 (Peutz-Jeghers syndrome)
Peutz-Jeghers syndrome is associated with polyps of the GI tract and also increased pigmentation around the lips, genitalia, buccal mucosa feet and hands.
Spigelman AD, Murday V, Phillips RK: Cancer and the Peutz-Jeghers syndrome.
It also, however, may include individuals with family histories of Li-Fraumeni Syndrome, ataxia-telangiectasia, Cowden syndrome, or Peutz-Jeghers syndrome.
(Refer to the Peutz-Jeghers syndrome section in the PDQ summary on the Genetics of Breast and Ovarian Cancer for more information.)
Hearle N, Schumacher V, Menko FH, et al.: Frequency and spectrum of cancers in the Peutz-Jeghers syndrome.
Clinical Practice Guidelines for the Diagnosis of Colon Cancer in Peutz-Jeghers Syndrome (PJS)
Hemminki A, Markie D, Tomlinson I, et al.: A serine/threonine kinase gene defective in Peutz-Jeghers syndrome.
Jenne DE, Reimann H, Nezu J, et al.: Peutz-Jeghers syndrome is caused by mutations in a novel serine threonine kinase.
Gruber SB, Entius MM, Petersen GM, et al.: Pathogenesis of adenocarcinoma in Peutz-Jeghers syndrome.