Bannayan-Zonana syndrome

WikiDoc Resources for Bannayan-Zonana syndrome
Articles
Most recent articles on Bannayan-Zonana syndrome Most cited articles on Bannayan-Zonana syndrome Review articles on Bannayan-Zonana syndrome Articles on Bannayan-Zonana syndrome in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Bannayan-Zonana syndrome Images of Bannayan-Zonana syndrome Photos of Bannayan-Zonana syndrome Podcasts & MP3s on Bannayan-Zonana syndrome Videos on Bannayan-Zonana syndrome
Evidence Based Medicine
Cochrane Collaboration on Bannayan-Zonana syndrome Bandolier on Bannayan-Zonana syndrome TRIP on Bannayan-Zonana syndrome
Clinical Trials
Ongoing Trials on Bannayan-Zonana syndrome at Clinical Trials.gov Trial results on Bannayan-Zonana syndrome Clinical Trials on Bannayan-Zonana syndrome at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Bannayan-Zonana syndrome NICE Guidance on Bannayan-Zonana syndrome NHS PRODIGY Guidance FDA on Bannayan-Zonana syndrome CDC on Bannayan-Zonana syndrome
Books
Books on Bannayan-Zonana syndrome
News
Bannayan-Zonana syndrome in the news Be alerted to news on Bannayan-Zonana syndrome News trends on Bannayan-Zonana syndrome
Commentary
Blogs on Bannayan-Zonana syndrome
Definitions
Definitions of Bannayan-Zonana syndrome
Patient Resources / Community
Patient resources on Bannayan-Zonana syndrome Discussion groups on Bannayan-Zonana syndrome Patient Handouts on Bannayan-Zonana syndrome Directions to Hospitals Treating Bannayan-Zonana syndrome Risk calculators and risk factors for Bannayan-Zonana syndrome
Healthcare Provider Resources
Symptoms of Bannayan-Zonana syndrome Causes & Risk Factors for Bannayan-Zonana syndrome Diagnostic studies for Bannayan-Zonana syndrome Treatment of Bannayan-Zonana syndrome
Continuing Medical Education (CME)
CME Programs on Bannayan-Zonana syndrome
International
Bannayan-Zonana syndrome en Espanol Bannayan-Zonana syndrome en Francais
Business
Bannayan-Zonana syndrome in the Marketplace Patents on Bannayan-Zonana syndrome
Experimental / Informatics
List of terms related to Bannayan-Zonana syndrome

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

Synonyms and keywords: Ruvalcaba-Myhre syndrome, Ruvalcaba-Myhre-Smith syndrome, Riley-Smith syndrome, Bannayan syndrome, or Bannayan-Zonana syndrome

Overview

Bannayan-Zonana syndrome is a rare hamartomatous disorder with occurrence of multiple subcutaneous lipomas, macrocephaly and hemangiomas.

Pathophysiology

The disease is inherited in an autosomal dominant form, but sporadic cases have been reported. The disease belongs to a family of hamartomatous polyposis syndromes, which also includes Peutz-Jeghers syndrome, juvenile polyposis and Cowden syndrome.

• Bannayan-Riley-Ruvalcaba syndrome (BRRS) is transmitted in autosomal dominant pattern.
• It is understood that Bannayan-Riley-Ruvalcaba syndrome (BRRS) is the result caused by the following gene mutations:
  • Germline phosphatase and tensin homolog (PTEN) mutations.

Natural History, Complications, and Prognosis

Most lesions of BZS are slowly growing and easily resectable. Visceral as well as intracranial involvement may occur in rare cases, and can cause bleeding and symptomatic mechanical compression, especially of the spinal cord or spinal nerve roots. This may require surgical resection.

History and Symptoms

Physical Examination

The macroencephaly of BZS is symmetrical, and does not cause widening of the ventricles or raised ICP (intracerebral pressure).