Desmoid Tumor Overview
Learn About Desmoid Tumor
A desmoid tumor is an abnormal growth that arises from connective tissue, which is the tissue that provides strength and flexibility to structures such as bones, ligaments, and muscles. Typically, a single tumor develops, although some people have multiple tumors. The tumors can occur anywhere in the body. Tumors that form in the abdominal wall are called abdominal desmoid tumors; those that arise from the tissue that connects the abdominal organs are called intra-abdominal desmoid tumors; and tumors found in other regions of the body are called extra-abdominal desmoid tumors. Extra-abdominal tumors occur most often in the shoulders, upper arms, and upper legs.
Mutations in the CTNNB1 gene or the APC gene cause desmoid tumors. CTNNB1 gene mutations account for around 85 percent of sporadic desmoid tumors. APC gene mutations cause desmoid tumors associated with familial adenomatous polyposis as well as 10 to 15 percent of sporadic desmoid tumors. Both genes are involved in an important cell signaling pathway that controls the growth and division (proliferation) of cells and the process by which cells mature to carry out specific functions (differentiation).
Desmoid tumors are rare, affecting an estimated 1 to 2 per 500,000 people worldwide. In the United States, 900 to 1,500 new cases are diagnosed per year. Sporadic desmoid tumors are more common than those associated with familial adenomatous polyposis.
Most desmoid tumors are sporadic and are not inherited. Sporadic tumors result from gene mutations that occur during a person's lifetime, called somatic mutations. A somatic mutation in one copy of the gene is sufficient to cause the disorder. Somatic mutations in either the CTNNB1 or the APC gene can cause sporadic desmoid tumors.
Stony Brook Orthopaedic Associates, University Faculty Practice Corpor
Fazel Khan is an Orthopedics provider in East Setauket, New York. Dr. Khan and is rated as a Distinguished provider by MediFind in the treatment of Desmoid Tumor. His top areas of expertise are Bone Tumor, Congenital Generalized Fibromatosis, Infantile Myofibromatosis, Hip Replacement, and Osteotomy.
John T Mather Memorial Hospital
Mohammad Bilal is a Radiologist and a Radiation Oncologist in Port Jefferson, New York. Dr. Bilal and is rated as an Experienced provider by MediFind in the treatment of Desmoid Tumor. His top areas of expertise are Ascites, Thyroid Hormone Plasma Membrane Transport Defect, Familial Hyperthyroidism due to Mutations in TSH Receptor, Vertebroplasty, and Bone Graft.
North Shore Hematology Oncology Associates PC
Giuseppe Condemi is an Oncologist and a Pain Medicine provider in Port Jefferson Station, New York. Dr. Condemi and is rated as an Experienced provider by MediFind in the treatment of Desmoid Tumor. His top areas of expertise are Familial Colorectal Cancer, Colorectal Cancer, Paget Disease of the Breast, and Breast Cancer.
Summary: This is a Phase 2 study using talimogene laherparepvec, nivolumab, and trabectedin as first, second or third line therapy for advanced sarcoma, including desmoid tumor and chordoma.
Summary: This study gathers health information for the Project: Every Child for younger patients with cancer. Gathering health information over time from younger patients with cancer may help doctors find better methods of treatment and on-going care.
Published Date: March 01, 2013
Published By: National Institutes of Health