Cowden syndrome natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Overview

Cowden syndrome follows an autosomal dominant pattern of inheritance. If left untreated, patients with cowden syndrome may prone to develop manu cancers.Common complications of cowden syndrome include breast, colon cancer and endometrial cancer. Prognosis is generally ranges from excellent to poor.

The symptoms of cowden syndrome usually tend to develop in second decade of life but varies and develops in ranging from 4 to 75 years.^[1]^[2]
If left untreated, patients with cowden syndrome may prone to develop many cancers.^[3]

Prognosis is generally ranges from excellent to poor, and the mortality rate of patients with cowden syndrome is based on malignant tumors associated with it.
The presence of benign tumours is associated with a particularly poor prognosis among patients with cowden syndrome.

↑ Hildenbrand C, Burgdorf WH, Lautenschlager S (2001). “Cowden syndrome-diagnostic skin signs”. Dermatology (Basel). 202 (4): 362–6. doi:10.1159/000051684. PMID 11455162.
↑ Starink TM, van der Veen JP, Arwert F, de Waal LP, de Lange GG, Gille JJ, Eriksson AW (March 1986). “The Cowden syndrome: a clinical and genetic study in 21 patients”. Clin. Genet. 29 (3): 222–33. PMID 3698331.
↑ Baker WD, Soisson AP, Dodson MK (April 2013). “Endometrial cancer in a 14-year-old girl with Cowden syndrome: a case report”. J. Obstet. Gynaecol. Res. 39 (4): 876–8. doi:10.1111/j.1447-0756.2012.02052.x. PMID 23279635.
↑ FitzGerald MG, Marsh DJ, Wahrer D, Bell D, Caron S, Shannon KE, Ishioka C, Isselbacher KJ, Garber JE, Eng C, Haber DA (August 1998). “Germline mutations in PTEN are an infrequent cause of genetic predisposition to breast cancer”. Oncogene. 17 (6): 727–31. doi:10.1038/sj.onc.1201984. PMID 9715274.
↑ Pilarski R, Burt R, Kohlman W, Pho L, Shannon KM, Swisher E (November 2013). “Cowden syndrome and the PTEN hamartoma tumor syndrome: systematic review and revised diagnostic criteria”. J. Natl. Cancer Inst. 105 (21): 1607–16. doi:10.1093/jnci/djt277. PMID 24136893.
↑ Baker WD, Soisson AP, Dodson MK (April 2013). “Endometrial cancer in a 14-year-old girl with Cowden syndrome: a case report”. J. Obstet. Gynaecol. Res. 39 (4): 876–8. doi:10.1111/j.1447-0756.2012.02052.x. PMID 23279635.