DICER1 Syndrome
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Learn About DICER1 Syndrome

What is the definition of DICER1 Syndrome?

DICER1 syndrome is an inherited disorder that increases the risk of a variety of cancerous and noncancerous (benign) tumors, most commonly certain types of tumors that occur in the lungs, kidneys, ovaries, and thyroid (a butterfly-shaped gland in the lower neck). Affected individuals can develop one or more types of tumors, and members of the same family can have different types. However, the risk of tumor formation in individuals with DICER1 syndrome is only moderately increased compared with tumor risk in the general population; most individuals with genetic changes associated with this condition never develop tumors.

What are the causes of DICER1 Syndrome?

DICER1 syndrome is caused by mutations in the DICER1 gene. This gene provides instructions for making a protein that is involved in the production of molecules called microRNA (miRNA). MicroRNA is a type of RNA, a chemical cousin of DNA, that attaches to a protein's blueprint (a molecule called messenger RNA) and blocks the production of proteins from it. Through this role in regulating the activity (expression) of genes, the Dicer protein is involved in many processes, including cell growth and division (proliferation) and the maturation of cells to take on specialized functions (differentiation).

How prevalent is DICER1 Syndrome?

DICER1 syndrome is a rare condition; its prevalence is unknown.

Is DICER1 Syndrome an inherited disorder?

DICER1 syndrome is inherited in an autosomal dominant pattern, which means one copy of the altered gene is sufficient to cause the disorder. It is important to note that people inherit an increased risk of tumors; many people who have mutations in the DICER1 gene do not develop abnormal growths.

Who are the top DICER1 Syndrome Local Doctors?
Radha G. Iyengar
Advanced in DICER1 Syndrome
Surgical Oncology | General Surgery
Advanced in DICER1 Syndrome
Surgical Oncology | General Surgery

Texas Health Breast Specialists

1105 Central Expy N, Ste 2100, 
Allen, TX 
Languages Spoken:
English

Dr. Radha Iyengar is the medical director of the Breast Center at Texas Health Presbyterian Hospital Allen. She obtained her undergraduate and medical degrees from the University of Florida and went on to complete a general surgery residency at the University of Texas Southwestern Medical School in Dallas. She then completed a Society of Surgical Oncology accredited breast fellowship at Emory University in Atlanta before returning to Texas.Dr. Iyengar is board-certified by the American Board of Surgery and is a Fellow of the American College of Surgeons. She is a member of the American College of Surgeons, Dallas Medical Society, American Society of Breast Surgeons, American Society of Breast Disease and American Society of Clinical Oncology. Dr. Iyengar is rated as an Advanced provider by MediFind in the treatment of DICER1 Syndrome. Her top areas of expertise are Breast Cancer, Paget Disease of the Breast, Inflammatory Breast Cancer, Intraductal Papilloma, and Lymphadenectomy.

Elite in DICER1 Syndrome
Elite in DICER1 Syndrome
Ottawa, ON, CA 

Leanne De Kock practices in Ottawa, Canada. Ms. De Kock is rated as an Elite expert by MediFind in the treatment of DICER1 Syndrome. Her top areas of expertise are Sertoli-Leydig Cell Tumor, DICER1 Syndrome, Leydig Cell Testicular Tumor, and Pleuropulmonary Blastoma.

 
 
 
 
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Advanced in DICER1 Syndrome
General Surgery
Advanced in DICER1 Syndrome
General Surgery

ETSU Physicians & Associates- Surgery

325 N State Of Franklin Rd, 
Johnson City, TN 
Languages Spoken:
English

. Dr. Scott is rated as an Advanced provider by MediFind in the treatment of DICER1 Syndrome. Her top areas of expertise are Breast Cancer, Paget Disease of the Breast, Giant Mammary Hamartoma, Lymphadenectomy, and Hernia Surgery.

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Who are the sources who wrote this article ?

Published Date: May 01, 2016
Published By: National Institutes of Health