Bannayan–Riley–Ruvalcaba syndrome (BRRS) is a rare overgrowth syndrome and  hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomas. The disease is inherited in an autosomal dominant manner. The disease belongs to a family of hamartomatous polyposis syndromes, which also includes Peutz–Jeghers syndrome, juvenile polyposis and Cowden syndrome. Mutation of the PTEN gene underlies this syndrome, as well as Cowden syndrome, Proteus syndrome, and Proteus-like syndrome, these four syndromes are referred to as PTEN Hamartoma-Tumor Syndromes. Signs and symptoms
Bannayan–Riley–Ruvalcaba syndrome is associated with enlarged head and benign mesodermal hamartomas (multiple hemangiomas, and intestinal polyps). Dysmorphy as well as delayed neuropsychomotor development can also be present. The head enlargement does not cause widening of the ventricles or raised intracranial pressure; these individuals have a higher risk of developing tumors, as the gene involved in BRRs is phosphatase and tensin homologue.Some individuals have thyroid issues consistent with multinodular goiter, thyroid adenoma, differentiated non-medullary thyroid cancer,
most lesions are slowly growing. Visceral as well as intracranial involvement may occur in some cases, and can cause bleeding and symptomatic mechanical compression

Genetics
The genetics of the Bannayan–Riley–Ruvalcaba syndrome is determined, in the majority of cases, via the PTEN gene which presents about 30 mutations in this condition. This gene which regulates cell growth, when not working properly can lead to hamartomas. PTEN chromosomal location is 10q23.31, while the molecular location is 87,863,438 to 87,971,930  There are many syndromes that are linked to PTEN aside from Bannayan–Riley–Ruvalcaba Syndrome.The syndrome combines Bannayan–Zonana syndrome, Riley–Smith syndrome, and Ruvalcaba–Myhre–Smith syndrome.
Aminocaproic acid (also known as ε-aminocaproic acid, ε-Ahx, or 6-aminohexanoic acid) is a derivative and analogue of the amino acid lysine, which makes it an effective inhibitor for enzymes that bind that particular residue. Such enzymes include proteolytic enzymes like plasmin, the enzyme responsible for fibrinolysis. For this reason it is effective in treatment of certain bleeding disorders, and it is sold under the brand name Amicar. Aminocaproic acid is also an intermediate in the polymerization of Nylon-6, where it is formed by ring-opening hydrolysis of caprolactam. The crystal structure determination showed that the 6-aminohexanoic acid is present as a salt, at least in the solid state. Medical use
Aminocaproic acid (Amicar) is FDA-approved for use in the treatment of acute bleeding due to elevated fibrinolytic activity. It also carries an orphan drug designation from the FDA for the prevention of recurrent hemorrhage in patients with traumatic hyphema. In clinical practice, aminocaproic acid is frequently used off-label for control of bleeding in patients with severe thrombocytopenia, control of oral bleeding in patients with congenital and acquired coagulation disorders, control of perioperative bleeding associated with cardiac surgery, prevention of excessive bleeding in patients on anticoagulation therapy undergoing invasive dental procedures, and reduction of the risk of catastrophic hemorrhage in patients with acute promyelocytic leukemia. References
Further reading
Alkjaersig N, Fletcher AP, Sherry S (April 1959). "xi-Aminocaproic acid: an inhibitor of plasminogen activation". The Journal of Biological Chemistry. 234 (4): 832–7. doi:10.1016/S0021-9258(18)70185-3. PMID 13654273. Kang Y, Lewis JH, Navalgund A, Russell MW, Bontempo FA, Niren LS, Starzl TE (June 1987).