Bannayan-Riley-Ruvalcaba syndrome

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by hamartomatous intestinal polyposis, lipomas, macrocephaly and genital lentiginosis.

Intestinal polyposis

The presence of multiple polyps in the intestine.


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PMID (PMCID)
29168369
 MALE Child
Polyposis deserves a perfect physical examination for final diagnosis: Bannayan-Riley-Ruvalcaba syndrome.
Hzarcoglu-Gulsen H, Klc E, Dominguez-Garrido E, Aydemir Y, Utine GE, Saltk-Temizel N.
Turk J Pediatr. 2017;59(1):80-83.
Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare autosomal dominant inherited polyposis syndrome characterized by macrocephaly, lipomatosis, hemangiomatosis, intestinal polyposis and pigmented macules on penis.
21856113
 FEMALE Middle Aged
Recurrent and extensive vascular malformations in a patient with Bannayan--Riley--Ruvalcaba syndrome.
Litzendorf M, Hoang K, Vaccaro P.
Ann Vasc Surg. 2011;25(8):1138.e15-9.
Bannayan--Riley--Ruvalcaba syndrome (BRRS) is a rare, usually autosomal dominant, disease associated with the PTEN hamartoma tumor syndrome, and it is clinically diagnosed in the presence of the triad of macrocephaly, genital lentiginosis, and intestinal polyposis.
16198785
 MALE Child
Clinical and histopathological findings in Bannayan-Riley-Ruvalcaba syndrome.
Erkek E, Hizel S, Sanly C, Erkek AB, Tombakoglu M, Bozdogan O, Ulkatan S, Akarsu C.
J Am Acad Dermatol. 2005;53(4):639-43.
Bannayan-Riley-Ruvalcaba syndrome is a rare autosomal dominant genodermatosis with the classical triad of macrocephaly, genital lentiginosis, and intestinal polyposis.